# Question MCQ A 1 Pick the correctly paired keratin with its structure: 1. K1K1! " #asal cells $. K%K1$ " esopha&us %. K'K1% " cornea '. K(K1' " supra#asal cells (. K1)K) " pal*s and soles + $ ,hich of the followin& state*ents a#out the direct i**uno-uorescence pattern in lichen planus is correct. 1. /he 012 is ne&ati3e in the 3ast *a4ority of cases $. 0eposition of 1&5 is within cytoid #odies in the super6cial der*is %. /he 0+7 deposition is &ranular '. 0eposition of 6#rino&en is within cytoid #odies in the deep der*is (. /here is pro*inent deposition of 1&M within the spinous layer of the epider*is C % Krause end #ul#s are: 1. Adaptin& *echanoreceptors found on wei&ht"#earin& sites that respond to 3i#rational sti*ul $. 2ound on the 3er*illion #order of the lips %. 8ocated in the der*al papillae of di&ital skin '. 8ocated in the deep der*is and within the su#cutis in wei&ht"#earin& sites of the #ody (. 2ound at the ori6ce of the hair follicle and particularly sensiti3e to cold B ' 9n electron *icroscopy: which cell de*onstrates cytoplas*ic pro4ections and secretory &ranules. 1. 8an&erhans cell $. Keratinocyte %. Mast cell '. Melanocyte (. Macropha&e C ( ,hen do *elanocytes #e&in to synthesi;e *elanin. 1. $nd *onth of &estation $. %rd *onth of &estation %. 'th *onth of &estation '. (th *onth of &estation (. )th *onth of &estation B ) +pider*olysis #ullosa si*ple< =+BS>: ,e#er Cockayne type: is caused #y what defect. 1. Colla&en ?11 $. Alpha")"#eta"' inte&rin %. Keratins 1 @ 1! '. Keratins ( (. Plectin 0 A Mutations in which of the followin& proteins results in epider*olysis #ullosa si*ple< associated with *uscular dystrophy: 1. Bncein $. Plectin %. a)#' inte&rin '. 8a*inin ( (. Colla&en type 1? B C S1!! stainin& would #e ne&ati3e in. 1. lan&erhans cells $. eccrine cells %. schwann cells '. adipocytes (. keratinocytes + D 0irect i**uno-uorescence is of no 3alue in the dia&nosis of: 1. Eeonatal 8+ $. 8ichen planus %. Mi<ed connecti3e tissue disease '. S8+ (. +rythe*a *ultifor*e A 1! /he *a4or co*ponent of the anchorin& 6la*ents is: 1. Bncein $. Plectin %. a)#' inte&rin 0 1 '. 8a*inin ( (. Colla&en type 1? 11 Fair &rows at: 1. !.!!' **day $. !.!' **day %. !.' **day '. ' **day (. 1! **day C 1$ 2ra&*entation andor loss of elastic 6#ers in not seen in: 1. Cutis la<a $. MarfanGs syndro*e %. Anetoder*a '. Psuedo<antho*a elasticu* (. Buschke"9llendorf Syndro*e + 1% Se#aceous &lands secrete se#u* throu&h which of the followin& secretory *echanis*s. 1. Folocrine $. Merocrine %. Apocrine '. Folocrine and Merocrine (. Folocrine and Apocrine A 1' ,hich of the followin& is true re&ardin& the de3elop*ent of hair follicles. 1. /he 6rst pri*ordial hair follicles for* at 1( weeks &estation $. /he 6rst hair follicles for* on the scalp and eyelashes %. 2ollicles de3elop in a cephalad to caudal direction '. Eew follicles de3elop durin& the 6rst % *onths postpartu* (. /he eye#rows de3elop late in &estation C 1( /he proteins that *ake up the corni6ed cell en3elope are synthesi;ed in the: 1. Stratu* corneu* $. Stratu* #asale %. Melanocytes '. Stratu* &ranulosu* (. 8an&erhans cells 0 1) Keratin 6la*ents in #asal cells insert into: 1. 0es*oso*es $. Adherens 4unctions %. Conne<ins '. 8a*ellar &ranules (. 9dland #odies A 1A ,hich of the followin& state*ents is true a#out eccrine &lands. 1. Post&an&lionic sy*pathetic 6#ers with acetylcholine as the principal neurotrans*itter $. Post&an&lionic sy*pathetic 6#ers with norepinephrine as the principal neurotrans*itter %. Post&an&lionic parasy*pathetic 6#ers with acetylcholine as the principal neurotrans*itter '. Post&an&lionic parasy*pathetic 6#ers with norepinephrine as the principal neurotrans*itter (. Post&an&lionic sy*pathetic 6#ers with #oth norepinephrine and acetylcholine as the principal neurotrans*itters A 1C Hed or #londe hair pi&*entation pri*arily results fro*: 1. /he presence of eu*elanin $. /he a#sence of *elanin %. /he presence of pheo*elanin '. /he reduced acti3ity of tyrosinase (. /he reduced acti3ity of 09PA dehydro<ylase C 1D +ach of the followin& is true a#out *elanoso*es e<cept: 1. Most characteristic or&anelle of the *elanocyte $. /yrosinase acti3ity decreases as *elanoso*oes *ature %. Are transferred to keratinocytes 3ia pha&ocytosis '. Are sin&ly dispersed in the #asal layer in white skin 0 $ (. Are lar&er in si;e in #lack skin co*pared to white skin $! /he *icro-ora of pilose#aceous unit consist of which of the followin&: 1. Pityrosporu* o3ale $. Staphylococcus aureus %. +scherichia coli '. Pseudo*onas aeru&inosa (. Coryne#acteriu* diphtheriae A $1 ,hich protein is the lar&est co*ponent of the corni6ed cell en3elope. 1. Keratin $. 1n3olucrin %. Pro6la&&rin '. 8oricrin (. Cera*ide 0 $$ Apocrine &lands are found in all of the followin& areas of the #ody e<cept: 1. A<illae $. Breasts %. +yelid '. Pal*s (. Perineu* 0 $% ,hich type of colla&en in *utated in osteo&enesis i*perfecta. 1. Colla&en 1 $. Colla&en 11 %. Colla&en 111 '. Colla&en 1? (. Colla&en ?11 A $' /he 6ndin& on 012 that re-ects #indin& of the Ho and 8a anti&ens in su#acute cutaneous lupus erythe*atosus is: 1. 5ranular -uorescence throu&hout the cytoplas* and nucleus of #asal keratinocytes $. Cytoid #odies %. 1**une deposits alon& the 0+ 4unction '. 5ranular deposits alon& the #ase*ent *e*#rane (. A Ichicken"wireI pattern within the epider*is A $( Cutaneous warts in renal transplant recipients show increased e<pression of which keratin. 1. K$ $. K( %. KD '. K1% (. K1) 0 $) /he #est su#strate for i**uno-uoresence in the der*atolo&ic condition trans*itted #y the #lack -y is: 1. Hat #ladder $. Monkey esopha&us %. 5uinea pi& esopha&us '. Ar*adillo foot pads (. ,hite *ouse tail C $A ,hich of the followin& options is characteristic of inte&rins. 1. 0efects in the alpha") do*ain of inte&rin result in epider*olysis #ullosa si*ple< with *uscular dystrophy $. Alpha")"Beta"' inte&rin is found at sites where des*o&leins attach %. +<pression is seen in all layers of the epider*is '. /he e<tracellular alpha) do*ain #inds colla&en A (. /hese proteins coordinate linka&e #etween inter*ediate 6la*ents and e<tracellular *atri< of the #ase*ent *e*#rane + $C ,hich of the followin& a*ino acids are typically found in elastic 6#ers. 1. 0es*osine $. 1soleucine %. Proline '. Fydro<yproline (. 5lycine A $D /he cutaneous i**uno-uorescence pattern in patients with Senear"Bsher syndro*e is: 1. 1ntercellular 1&5 and C% $. 8inear 1&5 and C% alon& the #ase*ent *e*#rane ;one %. 1ntercellular 1&5 on &uinea pi& esopha&us 0 % '. 1ntercellular 1&5 and C% and linear 1&5 and C% alon& the #ase*ent *e*#rane ;one (. 8inear 1&5 and C% alon& the #ase*ent *e*#rane ;one and intercellular 1&5 on &uinea pi& esopha&us %! ,hich of the followin& state*ents a#out &lo*us cells is correct. 1. /u*ors of &lo*us cells are *ost co**only found on the ton&ue $. Are of neural ori&in %. Allow rapid shuntin& of #lood fro* the arterioles to 3enules: #ypassin& capillaries '. /u*ors co*posed of &lo*us cells are asy*pto*atic (. /u*ors co*posed of &lo*us cells are often *ali&nant C %1 Keratinocytes are deri3ed fro* which of the followin&: 1. +ndoder* $. Mesoder* %. +ctoder* '. Eeural Crest (. Bone *arrow precursors C %$ ,hich *echanoreceptor found in hair #earin& areas sense deep touch and 3i#ration. 1. Merkel cell $. Meissner corpuscle %. ?ater"Pacini corpuscle '. Krase end"#ul# (. 2ree ner3e endin& C %% ,hich of the followin& *edications is concentrated in the eccrine &lands. 1. Cyclophospha*ide $. Cytara#ine %. Cipro-o<acin '. Cephale<in (. All of the answers are correct + %' 0er*al dendrocytes: 1. Are responsi#le for i**ediate"type hypersensiti3ity reactions. $. Acti3ely synthesi;e and release 1&5. %. Present anti&en to naJ3e / cells in the ly*ph node. '. Are the pri*ary cell found in a &lo*an&io*a. (. Are the pri*ary cell found in an an&iosarco*a. C %( ,hich ele*ent is necessary for function of *atri< *etalloproteinases. 1. 1ron $. Eitro&en %. Man&anese '. Ma&nesiu* (. Kinc + %) ,hich of the followin& diseases has decreased or a#sent la*ellar &ranules. 1. 2le&elGs $. Psoriasis %. 8a*ellar ichthyosis '. +pider*olytic hyperkeratosis (. Pe*phi&us 3ul&aris A %A ,hich of the followin& cells are reLuired for wound healin&. 1. Eeutrophil $. Macropha&e %. +osinophil '. 8an&erhans cell (. 8y*phocyte B %C 5lo*us cells are: 1. Modi6ed 6#ro#lasts $. Modi6ed skeletal *uscle cells %. ?ascular s*ooth *uscle cells '. Monocytes (. Eeuronal cells C %D Apocrine &lands: 1. 0e*onstrate holocrine secretion $. 0e*onstrate decapitation secretion %. Are fully functional at #irth B ' '. Are diMusely distri#uted on the #ody (. Are ther*ore&ulatory '! /he &reatest density of *ast cells is found in the: 1. Stratu* spinosu* $. Stratu* #asale %. Papillary der*is '. Heticular der*is (. Su#cutaneous fat C '1 ,hich si&nalin& *olecule *ediates the transition of hair cyclin& fro* telo&en to ana&en phase. 1. 2o<n1 $. 2&f( %. Sonic hed&eho& '. 0ihydotestosterone (. (a"reductase C '$ ,hich one of the followin& is responsi#le for *aintainin& a #arrier to water loss in the stratu* corneu*. 1. 1n3olucrin $. 2ila&&rin %. 8oricrin '. /rans&luta*inase (. 9dland #odies + '% /yrosinase is the en;y*e that cataly;es the con3ersion of tyrosine to 09PA and 09PA to 09PALuinone. /he en;y*e contains which of the followin& ions. 1. Kinc $. Copper %. Seleniu* '. 1ron (. Ma&nesiu* B '' /he *a4or protein co*ponent of the corni6ed en3elope is: 1. +n3oplakin $. 0es*oplakin %. Plectin '. 8oricrin (. /rans&luta*inase 0 '( /he e*#ryonic perider* #eco*es part of the 1. 3erni< caseosa $. stratu* corneu* %. stratu* #asale '. der*is (. hair follicle A ') /he stren&th of a scar: 1. 1s (N at 1 week $. 1s $!N at % weeks %. 1s A!N at 1 year '. All of these options are correct (. Eone of these options are correct 0 'A Fo*ocystinuria has a#nor*al crosslinkin& of colla&en #ecause of a *utation in: 1. Cystathione synthase $. /ype 1 colla&en E"peptidase &ene %. 8ysyl hydro<ylase '. /enascin O (. 8ysyl o<idase A 'C ,hich of the followin& protein plays a *a4or role in wound healin&. 1. Bncein $. 2i#ronectin %. Eido&en '. +ntactin (. Band") protein B 'D 1n the epider*is: the cell *ost responsi#le for anti&en detection and processin& is the: 1. Keratinocytes $. Merkel Cell %. Melanocyte '. 8an&erhans cell (. C0'P / cell 0 (! Fow soon does epitheliali;ation #e&in after a skin wound occurs. 1. Minutes $. Fours %. $ days '. ' days (. ) days B (1 0es*osine and isodes*osine are typical a*ino acids found in: 1. Colla&en 6#ers $. Anchorin& 6#ril C ( %. +lastic 6#ers '. Feparan sulfate (. Anchorin& plaLues ($ Hetinoids upre&ulate transcription of which types of colla&en. 1. 1 and % $. 1 and ' %. 1 and A '. % and A (. ' and A C (% Mutations in which of the followin& &enes will produce red hair. 1. MC1"H $. A&outi %. Fairless '. c"kit (. /yrosinase A (' Keratinocytes ha3e #een shown to secrete all of the followin& cytokines e<cept: 1. 18"1 $. 18") %. 18"C '. /E2"alpha (. 18"$ + (( 0irect i**uno-uorescence stainin& of intercellular spaces and the #ase*ent *e*#rane ;one: in co*#ination: is seen in: 1. Paraneoplastic pe*phi&us $. Anti"epili&rin pe*phi&oid %. Pe*phi&us 3e&etans '. Pe*phi&us foliaceous (. 1&A pe*phi&us A () /he des*oso*al connections of the epider*is are dependent on which of the followin& ions. 1. 1ron $. Kinc %. Seleniu* '. Calciu* (. Sodiu* 0 (A ,hich of the followin& is the *ost co**on &enetic alteration seen in *ucosal *elano*as. 1. 5EAQ $. Cyclin 0ependant Kinase ') %. BHA2 '. K1/ (. F0M$ 0 (C People with darker skin show: 1. S*aller: *ore concentrated *elanoso*es $. A *ore rapid de&radation of *elanoso*es %. A lessened production of *elanoso*es within *elanocytes '. A hi&her de&ree of dispersion of *elanoso*es in keratinocytes (. A &roupin& of *elanoso*es with a low de&ree of *elani;ation 0 (D A salt split skin 012 is perfor*ed on a #iopsy taken ad4acent to the skin lesions shown. ,here would you e<pect stainin& to #e seen. 1. +pider*al side $. 0er*al side %. +pider*al and 0er*al sides eLually '. 1n the la*ina densa (. 1n the anchorin& plaLues A )! ,hich epony* descri#es 3esti&ial lines of pi&*entary de*arcation. 1. 2uchter lines $. ,allaceGs lines %. 8an&erGs lines '. 8ines of Blaschko (. 0er*ato*e A )1 +ach of the followin& is true a#out the #ase*ent *e*#rane ;one e<cept: 1. Anchorin& 6la*ents attach the #asal cell *e*#rane to the la*in lucida $. Can #e 3isuali;ed on li&ht *icroscopy with PAS stainin& %. 8a*ina densa is co*posed of type 1? colla&en '. Contains la*inin 1 and la*inin ( (. Anchorin& 6#rils are co*posed of type ?11 colla&en A ) )$ 0urin& hair follicle de3elop*ent: the ,E/ si&nalin& pathway is one of the earliest *olecular pathways in3ol3ed in hair follicle initiation. ,hat is the downstrea* *ediator of ,E/ si&nalin&. 1. S*oothened $. Beta"catenin %. Keratin 1) '. p(% (. F8A"B$A B )% /ype ?11 colla&en in found in anchorin& 6#rils and also in: 1. 2etal skin $. Bone %. A*nion '. Aorta (. Blood 3essels C )' 1tch is *ost co**only trans*itted #y: 1. C"poly*odal nociceptor class ner3es $. A"delta class ner3es %. A"#eta class ner3es '. Parasy*pathetic post&an&lionic 6#ers (. A"#eta and A"delta 6#ers A )( ,hat co*ponent is the *a4or #arrier in the stratu* corneu*. 1. Se#u* $. SLualene %. Colla&en '. Cera*ide (. /ri&lycerides 0 )) Sneddon ,ilkinson disease is caused #y a defect in what anti&en. 1. 0es*ocollin 1 $. 0es*o&lein 1 %. 0es*o&elin % '. BPA&1 (. BPA&$ A )A ,hich of the followin& state*ents a#out elastic 6#ers is true. 1. +lastic 6#ers for* %(N of the dry wei&ht of the skin $. +lastic 6#ers are D!N elastin wrapped in 6#rillin %. Colla&en 1 is *utated in Marfan syndro*e '. 9<ytalan 6#ers run parallel within the super6cial papillary der*is (. +launin 6#ers run perpendicular in thin #ands within the reticular der*is. B )C ,here are &lo*us cells deri3ed fro*. 1. Mesenchy*e $. Bone *arrow %. SusLuet"Foyer canal '. Skeletal *uscle (. 0er*is C )D ,hich of the followin& cytokines are secreted fro* keratinocytes. 1. 18"$ $. 18"% %. 18"1A '. 18"$$ (. 18"$% + A! 0es*o&lein 1 is the anti&en in which of the followin& autoi**une diseases of the skin: 1. Pe*phi&us foliaceus $. Bullous i*peti&o %. 0er*atitis herpetifor*is '. Bullous pe*phi&oid (. Pe*phi&oid &estationis A A1 ,hich cytokeratin would you e<pect to #e preferentially e<pressed in the keratinocytes of a psoriatic plaLue: 1. K1 $. K( %. K1$ '. K1( (. K1) + A$ +launin 6#ers: 1. Hun parallel in #ands within the super6cial papillary der*is $. Hun perpendicular fro* the der*o"epider*al 4unction within the super6cial papillary der*is %. Hun parallel in #ands within the reticular C A der*is '. Hun perpendicular in #ands within the deep der*is (. Hun perpendicular in #ands within the reticular der*is A% He&ardin& der*al"epider*al 4unction: which of the followin& state*ents is true: 1. /here are no anchorin& 6la*ents in la*ina lucida $. 8a*ina 6#roreticularis lies a#o3e la*ina densa %. 8a*ina 6#roreticularis co*prises of anchorin& 6#rils and the elastic *icro6#rils '. Blood 3essels cross the der*al"epider*al 4unction to reach the epider*is (. 8a*ina lucida is an electron"dense layer C A' ,hich of the followin& en;y*es does not reLuire copper for functionin&. 1. 8ysyl o<idase $. A/PAa %. /yrosinase '. Cystathione #eta"synthase (. 2errochelatase + A( ,hat structure delineates the anato*ic re&ion #etween the nail #ed and the distal &roo3e: where the nail plate detaches of the distal portion of the di&it. 1. Eail *atri< $. Pro<i*al nail fold %. 8unula '. +ponychiu* (. Fyponychiu* + A) ,hich keratins are e<pressed in the stratu* &er*inati3u* and are present #ut not *ade de no3o in the stratu* spinosu*. 1. K': 1% $. K1:1! %. K$e: 1! '. K%:1$ (. K(:1' + AA ,hich of the followin& der*al cells always e<press C011c and C0). 1. Mononuclear pha&ocytic cells $. 2i#ro#lasts %. Mast cells '. +rythrocytes (. 5lo*us cells A AC ,hich of the followin& state*ents a#out darkly pi&*ented races 3ersus li&hter pi&*ented races is correct. 1. /he nu*#er of *elanoso*es in *elanocytes are the sa*e $. /he indi3idual *elanoso*es ha3e the sa*e de&ree of *elani;ation %. /he *elanoso*es are eLual in si;e *elanoso*es '. /here are eLual nu*#ers of *elanocytes (. /here is a faster rate of *elanoso*e de&radation 0 AD ,hich of the followin& do*ains is tar&eted #y the autoanti#odies in #ullous pe*phi&oid. 1. EC1)A of BP1C! $. 8a*inin ( %. Plectin '. alpha") inte&rin (. EC1)A of BP$%! A C! Acid keratins are e<pressed on which of the followin& chro*oso*es. 1. 1$ $. 1A %. D '. 1) (. % B C1 ,hich of the followin& cytokines is secreted #y adipocytes. 1. 18"$ $. 1l"' %. 18") '. 18"1! (. 18"1$ C C$ /issue contraction #e&ins: 1. At %rd day of wound healin& $. 0urin& the $nd week of wound healin& B C %. After the 6rst *onth of wound healin& '. After the %rd *onth of wound healin& (. After the Dth *onth of wound healin& C% /he hair follicle is the site of production or con3ersion of which of the followin& hor*ones. 1. All the options are correct $. dihydrotestosterone =0F/> %. prolactin '. adrenocorticotropin hor*one =AC/F> (. alpha"*elanocyte sti*ulatin& hor*one =MSF> A C' ,hat is the *a4or function of urocanic acid. 1. Bacteriocidal acid produced #y stratu* corneu* $. Pri*arily a B?B 6lter %. Pri*arily a B?A 6lter '. Felps de&rade free fatty acids (. Aids in protectin& the skin fro* der*atophytes C C( A speci6c *arker of Merkel cells is: 1. Cytokeratin 1! $. Cytokeratin 1( %. Cytokeratin $! '. 8oricrin (. +n3oplakin C C) ,hich keratins are upre&ulated in hyperproliferati3e disease such as psoriasis. 1. Keratins 1 and 1! $. Keratins $e and 1! %. Keratins ( and 1' '. Keratins ) and 1) (. Keratins C and 1C 0 CA A AC year"old *an is dia&nosed with cicatricial pe*phi&oid. ,hich of the followin& anti&ens #ein& i*plicated in his disease should tri&&er a work"up for *ali&nancy. 1. BPA&$ $. 8a*inin ( %. 8a*inin ) '. Beta"' inte&rin (. /ype ?11 colla&en B CC /he *ain per*ea#ility #arrier in the la*ina densa is: 1. heparan sulfate proteo&lycan $. colla&en 1? %. la*inin ( '. nido&en (. alpha")"#eta"' inte&rin A CD Mo3in& fro* internally to e<ternally choose the correct description of the hair follicle: 1. 1nner root sheath cuticle " Fu<leyGs layer " FenleGs layer " *edulla " corte< " hair shaft cuticle $. Fair shaft cuticle " corte< " *edulla " FenleGs layer " Fu<leyGs layer Q inner root sheath cuticle %. FenleGs layer " Fu<leyGs layer " inner root sheath cuticle " hair shaft cuticle " corte< " *edulla '. Medulla Q corte< Q hair shaft cuticle Q inner root sheath cuticle Q Fu<leyGs layer Q FenleGs layer (. 1nner root sheath cuticle Q outer root sheath cuticle Q hair shaft cuticle Q corte< Q Fu<leyGs layer Q FenleGs layer 0 D! Platelets release which of the followin& factors to pro*ote new tissue &rowth. 1. Eeutrophil che*otactic factor $. 18"1 %. A0P '. /52"alpha (. 252 0 D1 ,hich co*ponent of hair is positi3e for citrulline. 1. 9uter root sheath $. 1nner root sheath %. Corte< '. 5lassy 3itreous layer (. Medulla B D D$ ,hat percenta&e of the dry wei&ht of skin in elastin. 1. $ $. ' %. ) '. C (. 1! B D% Apocrine &lands: 1. Are coiled &lands $. Fa3e a two se&*ent ducts that e*pties onto the skin %. Are present e3erywhere on the skin e<cept on the pal*s and soles '. 2unction fro* #irth (. Secretions are initially odorless + D' All *ononuclear pha&ocytic cells in the der*is e<press: 1. C0% $. C0) %. C0%' '. C0)C (. C0$! B D( /he epider*is is co*prised of what type of cells. 1. Keratinocytes: Melanocytes: Merkel cells: 8an&erhan cells $. Keratinocytes: +ndothelial cells: Merkel cells: 8an&erhan cells %. Keratinocytes: Melanocytes: Eeutrophils: 8an&erhan cells '. Keratinocytes: Melanocytes: Merkel cells: 5o#let cells (. Keratinocytes: +ndothelial cells: Merkel cells: 5o#let A D) 0efects in what kind of structural protein lead to pyloric atresia associated with 4unctional epider*olysis #ullosa: 1. Colla&en $. +lastin %. 8oricrin '. 1nte&rin (. Plectin 0 DA ,hat is the *ost i*portant cell for wound healin&. 1. 2i#ro#lasts $. Eeutrophils %. Macropha&es '. 8y*phocytes (. Mast cells C DC All of the followin& are true re&ardin& the for*ation of hair e<cept: 1. /he inner root sheath keratini;es #y *eans of trichohyalin &ranules $. FenleJs layer is outside of Fu<leyJs layer %. /he outer root sheath is a downward e<tension of the epider*is '. Fu<leyJs layer contains *elanin (. /he hair *atri< #eco*es the hair and the inner root sheath 0 DD At what esti*ated &estational a&e are all layers of the keratini;ed epider*is identi6a#le. 1. C weeks $. 1$ weeks %. 1) weeks '. $! weeks (. $' weeks + 1!! ,hich cadherin is responsi#le for adhesion of 8an&erhan cells to the epider*is. 1. +"cadherin $. P"cadherin %. E"cadherin '. 0es*o&lein (. 0es*ocollin A 1!1 Fo*ocystinuria is an autoso*al recessi3e condition with 6ndin&s includin& a *arfanoid ha#itus: downward dislocation of the lens: cardio3ascular disease and *ental 1. A#nor*al crosslinkin& of colla&en $. A#nor*al de3elop*ent of elastin 6#ers %. Melanocyte death '. Pi&*entation of cartila&e (. Black urine A 1! retardation. 1t is caused #y a *utation in cystathionine #eta" synthetase. ,hat does this *utation in cystathionine #eta" synthetase cause other than an accu*ulation of ho*ocystine. 1!$ Fair follicle de3elop*ent in the hu*an e*#ryo #e&ins durin&: 1. 1st tri*ester $. $nd tri*ester %. %rd tri*ester '. At the #lastocoele sta&e (. ,ithin $ weeks of fertii;ation A 1!% Bpon presentation of an anti&en in the skin surface: a hapten for*s. /he 6rst cell to take up the hapten is: 1. B cells $. 8an&erhans cells %. keratinocytes '. / cells (. Mast cell B 1!' ,hich of the followin& keratins would *ost likely #e e<pressed in the nail #ed. 1. K)a1) $. K)#1A %. K1D '. K$e1! (. K'1% B 1!( Keratohyalin &ranules contain: 1. 0es*oplakin $. +n3oplakin and Keratin ) %. Pro6la&&rin and loricrin '. Eu*erous 5ol&i apparati (. 1n3olucrin C 1!) +lastic 6#ers contain the speci6c a*ino acids: 1. 8ysine and proline $. 8eucine and isoleucine %. Alanine and phenylalanine '. 0es*osine and isodes*osine (. 5lycine and proline 0 1!A ,hich of the followin& polypeptides is found in the la*ina lucida. 1. plako&lo#in $. des*oplakin %. keratocal*in '. de*oyokin (. la*inin ( + 1!C ,hich of the followin& is not a speciali;ed type of se#aceous &land. 1. MollGs &land $. Mei#o*ian &land %. 5land of Keis '. Mont&o*eryGs tu#ercle (. 2ordyce spot A 1!D /he anti#ody tar&et in ocular cicatricial pe*phi&oid is also *utated in: 1. 7unctional epider*olysis #ullosa: Ferlit; type $. Hecessi3e dystrophic epider*olysis #ullosa %. 7unctional epider*olysis #ullosa with *yotonic dystrophy '. 0o*inant dystrophic epider*olysis #ullosa (. 7unctional epider*olysis #ullosa with pyloric atresia + 11! 0urin& e*#ryo&enesis: perider* cells of the fetus contain which of the followin& su#stances. 1. Cera*ide $. 5lyco&en %. 2ree fatty acids '. Porphyrins (. Se#u* B 111 Anchorin& 6#rils are pri*arily co*posed of: 1. /ype 1 colla&en $. /ype 111 colla&en %. /ype 1? colla&en '. /ype ?11 colla&en (. /ype 11 colla&en 0 11$ ,hich of the followin& is true a#out *elanoso*es. 1. Spheroid *elanoso*es ha3e concentric la*ellae + 11 $. Spheroid *elanoso*es synthesi;e #rown" #lack eu*elanin %. +lliptical *elanoso*es ha3e *icro3esicular structure '. +lliptical *elanoso*es synthesi;e yellow or red pheo*elanin. (. /he *elanoso*es are positioned after the 5ol&i apparatus in the secretory pathway. 11% At any one ti*e: the appro<i*ate proportion of hair follicles in ana&en is: 1. '!N $. )!N %. C(N '. D(N (. 1(N C 11' /he 6rst pri*ordial hair follicles for* on the eye#rows: upper lip and chin at which &estational a&e. 1. A weeks $. D weeks %. 1$ weeks '. 1) weeks (. $! weeks B 11( Anchorin& 6la*ents ori&inate at the he*ides*oso*es and insert into the: 1. 0es*oso*e $. Su# #asal dense plate %. 8a*ina lucida '. BPA5 1C! (. 8a*ina densa + 11) 9dland #odies: 1. Contain keratins $. Are found intercellularly in the #asal cell layer %. Are e<clusi3ely intracellular '. Crosslink with keratins ( and 1' (. Are secretory &ranules with features of lysoso*es + 11A /elo&en eRu3iu*: 1. 1n3ol3es a diMuse alopecia aMectin& *ore than (!N of the scalp $. 9ften results fro* anti*eta#olites used durin& cancer che*otherapy %. 1s an in-a**atory alopecia de3oid of scarrin& '. 1s a patchy alopecia aMectin& less than (!N of the scalp (. Hesults fro* sudden illness or sur&ery + 11C ,hat is the *a4or co*ponent of the anchorin& 6#ril. 1. /ype 1 Colla&en $. /ype 111 Colla&en %. /ype 1? Colla&en '. /ype ?11 Colla&en (. 8a*inin ( 0 11D Ana&en eRu3iu* is #est descri#ed as: 1. An a#ru#t transition fro* ana&en to cata&en in rapidly di3idin& hair *atri< cells $. A cessation of *itotic acti3ity in rapidly di3idin& hair *atri< cells %. An a#rupt transition of telo&en to ana&en in restin& hair *atri< cells '. A cessation of *itotic acti3ity in restin& hair *atri< cells (. A scarrin& alopecia aMectin& only ana&en sta&e follicles B 1$! ,hat is the a3era&e duration of the telo&en cycle in ter*inal scalp hair. 1. $") years $. $"% weeks %. % *onths '. ) *onths (. D *onths C 1$1 He&ardin& se#aceous &lands: 1. /hese &lands are present at #irth at their 0 1$ adult si;e $. Si;e of the &land is proportional to the si;e of the associated hair follicle %. Are always associated with a hair follicle '. Are found e3erywhere on the skin e<cept pal*s and soles (. Are unilo#ular &lands 1$$ ,hich hor*one is ho*olo&ous to alpha"MSF =*elanocyte sti*ulatin& hor*one>. 1. 1nsulin $. Fu*an &rowth factor %. Prolactin '. /hyroid sti*ulatin& hor*one (. Adrenocorticotropic hor*one + 1$% Ana&en eRu3iu* is 1. Harely seen followin& ad*inistration of cancer che*otherapeutic a&ents $. Sti*ulus induces the a#rupt cessation of *iotic acti3ity in the rapidly di3idin& hair *atri< cells %. 9ccurs within $' to 'C hours of e<posure '. +ntirely inre3ersi#le (. Patients #ein& treated with nitrosurea a&ents are usually spared B 1$' +*#ryolo&ically: epider*al strati6cation occurs at appro<i*ately what esti*ated &estational a&e. 1. ' weeks $. C weeks %. 1$ weeks '. 1) weeks (. $! weeks B 1$( He&ardin& the stratu* &er*inati3u* =#asale>: 1. 1nter*ediate 6la*ents in #asal cells insert into only he*ides*oso*es $. Keratins 1 and 1! are e<pressed %. Eot all #asal cells ha3e the potential to di3ide '. Micro6la*ents assist in downward *o3e*ent of cells (. Plectins re&ulate adhesion and initiation of diMerentiation. C 1$) He&ardin& the stratu* spinosu*: which of the followin& is correct. 1. Eo keratin 11! is present $. Eew synthesis of K(1' occurs in this layer %. /he IspinesI seen on patholo&y are due to des*oso*al connections #etween keratinocytes '. /his layer contains *elanocytes (. /his layer contains the corni6ed cell *e*#rane C 1$A +lastic 6#ers are present in the der*is and are responsi#le for pro3idin& tissue resiliency. /hey are co*prised of elastin as well as *icro6#rillar proteins includin&: 1. 2i#rillins and 6#ulins $. Fyaluronic acid %. Colla&en 11 '. 8a*inin ( and BPA5$ (. 0es*oplakin and plako&lo#in A 1$C ,hat is the location of the un#ound corticosteroid receptor. 1. Cytoplas* $. Eucleus %. Mitochondria '. Plas*a *e*#rane (. &ol&i apparatus A 1$D 5ranulation tissue pri*arily contains: 1. Colla&en 1 $. Colla&en 111 %. Colla&en 1? '. 2i#rin (. Colla&en ?11 B 1%! Se#aceous &lands for* a lipid"rich su#stance called se#u* and are usually associated with a hair 1. ?acuolar e<ocytosis $. Passi3e diMusion %. 0ecapitation secretion + 1% follicle. /hey secrete se#u* #y what *echanis*. '. Autocrine secretion (. Folocrine secretion 1%1 1 *elanocyte has contact with: 1. ) keratinocytes $. 1$ keratinocytes %. 1C keratinocytes '. %! keratinocytes (. %) keratinocytes + 1%$ All of the followin& pertain to 9dland #odies +OC+P/: 1. Contain sLualene $. Are found intracellularly in upper le3el keratinocytes %. 0ischar&e their contents into the e<tracellular space at the 4unction of the &ranular and corni6ed layers '. +sta#lish a #arrier to water loss (. Mediate stratu* corneu* adhesion in con4unction with 6la&&rin. A 1%% ,hich of the followin& is a *e*#er of the ar*adillo fa*ily of linkin& proteins. 1. +"cadherin $. Periplakin %. +n3oplakin '. 0es*ocollin (. Plako&lo#in + 1%' Eu*erous neuro*ediators are in3ol3ed in cutaneous neuro#iolo&y and *any play a role in the de3elop*ent of in-a**ation in the skin. 9ne such *ediator can #e induced #y application of capsaicin to the skin. ,hich of the followin& is the correct neuro*ediator. 1. Eoradernaline $. Su#stance P %. Eeurokinin A '. Acetylcholine (. Pro"opio*elanocortin B 1%( /ype 1 colla&en is the *ost pre3alent colla&en in skin: accountin& for C!N or *ore of the total colla&en in the adult der*is. /he ne<t *ost predo*inant colla&en in adult hu*an der*is is: 1. /ype 11 colla&en $. /ype 111 colla&en %. /ype 1? colla&en '. /ype ?11 colla&en (. /ype O?11 colla&en B 1%) Melanocytes are deri3ed fro*: 1. Bone *arrow $. Eeural crest %. Mesoder*al precursors '. +ndoder*al precursors (. Solk sac deri3ed B 1%A ,hich of the followin& *ake up the *a4or protein of the corni6ed cell en3elope. 1. 8oricrin $. 1n3olucrin %. +n3oplakin '. 2ila&&rin (. 8a*inin ? A 1%C Mast cells are deri3ed fro* #one *arrow QQQQP cells. 1. C0% $. C0) %. C0$! '. C0%' (. C0)C 0 1%D ,hich of the followin& a#nor*alities would #e present in a patient with FarleLuin ichthyosis. 1. la*ellar &ranules are unifor*ly a#sent $. keratohyaline &ranules are nor*al in all types of harleLuin fetus %. de*oyokin *utation '. #and ) protein is a#sent (. plako&lo#in is a#nor*al A 1'! ,hich cell type is reLuired for wound healin&. 1. Macropha&e $. 8y*phocyte %. Mast cell '. 0er*al dendrocyte A 1' (. Eeutrophil 1'1 Acid keratins =K1!"$!> are e<pressed on which of the followin& chro*oso*es. 1. 1A $. 1C %. 1D '. 1! (. 1$ A 1'$ Apocrine chro*hidrosis results fro* which of the followin& contents of apocrine sweat. 1. 8ipofuschin $. SLualene %. Cholesterol '. 2atty acids (. A**onia A 1'% Mei#o*ian &lands are: 1. +ccrine &lands locali;ed to the 3er*illion #order of the lips $. Se#aceous &lands found on the areola of the #reast %. Se#aceous &lands found on the eyelids '. Apocrine &lands found in the ano&enital re&ions (. Apocrine &lands found on the eylelids C 1'' /he triple heli< of the colla&en *olecule is lar&ely *aintained due to its a*ino acid co*position. /he polypeptide chains of colla&en are repeatin& triplets of 5lycine"O"S. /he O and S positions can #e occupied #y *ultiple a*ino acids: #ut are *ost often: 1. Alanine and aspara&ine $. /yrosine and threonine %. Fistidine and ornithine '. 8eucine and isoleucine (. Proline and hydro<yproline + 1'( Fu*an se#u* is distin&uished fro* lipids of internal or&ans #y the presence of: 1. Cholestrol $. Cholestrol esters %. SLualene '. ,a< esters (. 5lycerides 0 1') /he 6rst cell type to *i&rate into a new wound in &reat nu*#ers is the: 1. Eeutrophil $. Monocyte %. Macropha&e '. 8y*phocyte (. Mast cell A 1'A 0ystrophic epider*olysis #ullosa results fro* *utations in: 1. Colla&en type 1 $. Colla&en type 11 %. Colla&en type 111 '. Colla&en type ? (. Colla&en type ?11 + 1'C ,hich of the followin& state*ents a#out 8a*inins is correct. 1. 8a*inins span fro* the plas*a *e*#rane of #asal keratinocytes to the la*ina lucida $. 8a*inins pro3ide little structural support in the #ase*ent *e*#rane %. 8a*inins pro3ide si&nalin& *olecules that interact with other proteins to trans*it *orpho&enetic infor*ation to the cellGs interior '. 8a*inin ( is also called plectin (. 8a*inin ( is the only la*inin found in the #ase*ent *e*#rane C 1'D ,hich of the followin& skin conditions in3ol3es elastin. 1. Hoth*und"/ho*pson $. +pider*olytic Fyperkeratosis %. Pseudo<antho*a elasticu* '. Pro&eria (. Classic type +hlers"0anlos syndro*e C 1(! ,hich of the followin& ele*ents is necessary for *elanin production. 1. Copper $. Seleniu* %. 1ron A 1( '. Kinc (. Calciu* 1(1 Keratinocytes in the #asal layer of the epider*is attach to the #ase*ent *e*#rane ;one at he*ides*oso*es 3ia what inter*ediate 6la*ent *olecules. 1. Keratins 1 and 1! $. Keratins ( and 1' %. 0es*o&leins 1 and % '. 0es*oplakin and 0es*o&lo#in (. BPA51 and BPA5$ B 1($ ,hich of the followin& stains would you e<pect to #e positi3e in a nor*al eccrine unit. 1. S"1!! $. Prussian #lue %. 5ie*sa '. ?erhoeM 3on 5ieson (. Steiner A 1(% ,hich of the followin& state*ents a#out plectin is correct. 1. 8inks 6lla&rin to the plas*a *e*#rane $. Crosslinks proteins in adherens 4unction %. Mutations result in 4unctional epider*olysis #ullosa with pyloric atresia '. Plectin is a *e*#er of the plakin fa*ily (. Plectin is a constituent protein of the des*oso*al plaLue 0 1(' Aprocrine &lands are adne<al &lands that are Luiescent until pu#erty. 9nce acti3e: they secrete their contents #y what *echanis*. 1. ?acuolar e<ocytosis $. Passi3e diMusion %. 0ecapitation secretion '. Autocrine secretion (. Folocrine secretion C 1(( ,hat is the *ost a#undant colla&en found on fetal skin. 1. /ype 1 Colla&en $. /ype 11 Colla&en %. /ype 111 Colla&en '. /ype 1? Colla&en (. /ype ?11 Colla&en C 1() 9nce a keratinocyte lea3es the #asal cell layer: the nor*al transit ti*e to stratu* corneu* is at least: 1. A days $. 1' days %. $1 days '. $C days (. %( days B 1(A Se#aceous &lands are located in each of the followin& locations e<cept: 1. Eipple $. 8a#ia *inora %. Pal*s '. +yelids (. Buccal *ucosa C 1(C 1n which of the followin& locations would you #e least likely to identify *elanocytes. 1. Stria 3ascularis of the ear $. 1ris %. 8epto*enin&es '. Hetina (. Pericardiu* + 1(D ,hich of the followin& is true re&ardin& BPA&1. 1. 1t is patho&enic in cicatricial pe*phi&oid $. 1t is a *e*#er of the plakin fa*ily %. 1t is patho&enic in pe*phi&oid &estationis '. 1t is not patho&enic in paraneoplastic pe*phi&us (. 1t coprecipitates with plako&lo#in B 1)! Sur&ery: Parturition: 2e3er: Kwashiokor and Fyper3ita*inosis A are all causes of: 1. /elo&en eRu3iu* $. Ana&en eRu3iu* %. Alopecia areata '. Andro&enetic alopecia (. Both telo&en and ana&en eRu3iu* A 1)1 ,hich of the followin& &lands is not under neural control. 1. Se#aceous &lands $. Apocrine &lands %. +ccrine &lands '. Sali3ary &lands (. Ceru*inous &lands A 1) 1)$ /he for*ation of &ranulation tissue depends on the presence of: 1. Eeutrophils $. 2i#ronectin %. Colla&en type 1 '. Platelets (. Colla&en type 1? B 1)% /he function of &lycosa*ino&lycansproteo&lycans in the der*is is: 1. He&ulate water"#indin& capacity $. 1nteract with der*al dendrocytes %. 2acilitate C98?11 #indin& to the anchorin& plaLues '. 2acilitate *ast cell de&ranulation (. He&ulate ly*phocyte traTckin& A 1)' /he *ain colla&en co*ponent of the #ase*ent *e*#rane is: 1. Colla&en 1? $. Colla&en 111 %. Colla&en 1 '. /enascin"O (. Colla&en ?11 A 1)( ,hat is the si&ni6cance of the critical line of Au#er. 1. 1t is the location of the insertion of the erector pili *uscle $. the #ulk of the *itotic acti3ity in the hair occurs a#o3e this line %. the inner root sheath is for*ed a#o3e this line '. 1t is the widest dia*eter of the hair #ul# (. 1t is where keratini;ation 6rst occurs in the hair 0 1)) Se#aceous &lands secrete se#u* 3ia: 1. Folocrine *echanis* $. +<ocrine *echanis* %. +ndocrine *echanis* '. +<ostosis (. Mecrocrine *echanis* A 1)A Se#aceous &lands: 1. Hespond to che*ical sti*uli such as hor*ones $. Hespond to choliner&ic neural acti3ity: e<clusi3ely %. Hespond to adrener&ic neural acti3ity: e<clusi3ely '. Hespond to #oth adrener&ic and choliner&ic sti*uli (. Hespond to the local release of cytokines fro* in-a**atory cells A 1)C /he classical types of +hlers" 0anlos Syndro*es =/ype 1 and $> lead to 3aryin& de&rees of hypere<tensi#le skin: easy #ruisin&: wide: atrophic scars: and hyper*o#ile 4oints. /he underlyin& defect in this disorder is a *utation in: 1. +lastin $. 2i#rillin 1 %. ABCC) '. Colla&en ? (. Cystathionine #eta"synthase 0 1)D Choose the correct answer re&ardin& *elanin and skin color: 1. 1n #lack and #rown skin the *elanoso*es are s*aller in dia*eter and len&th $. 2acultati3e skin color is the a*ount of cutaneous *elanin pi&*ent &enerated accordin& to cellular &enetics %. 1n white skin the *elanoso*es for* &roups within the secondary lysoso*es '. +u*elanin produces a yellow chro*ophore (. /he nu*#er of *elanocytes increases with one e<posure to B?A3isi#le li&ht C 1A! ,hich of the followin& *arkers are speci6c and relia#le for Merkel 1. C0$! $. FMB"'( + 1A cells. %. C0%' '. C0% (. CK$! 1A1 Mei#o*ian &lands are: 1. Modi6ed se#aceous &lands $. 2ound e3erywhere e<cept on the pal*s and soles %. Se#aceous lo#ules that feed into a lactiferous duct '. Modi6ed ceru*inous &lands (. Modi6ed apocrine &lands A 1A$ 5lo*us cells are pri*arily found: 1. on handsfeet $. on the trunk %. on the lateral thi&hs '. on the &enital skin (. on the face A 1A% +ccrine &lands are found in all the followin& areas of the #ody e<cept: 1. A<illae $. Pal*s %. 8a#ia *inora '. Scalp (. Cutaneous lip C 1A' Merkel cells are *echanoreceptors found in areas of hi&h"tactile sensiti3ity. /his i**unohistoche*ical *arker is restricted to Merkel cells in the skin and is thus a relia#le *arker for these cells: 1. Keratin A $. Keratin $! %. S"1!! '. 2actor O111a (. 8S?+"1 B 1A( 8+M0% is *utated in which of the followin& syndro*es. 1. Buschke 9llendorf $. Al#ri&ht Fereditary 9steodystrophy %. 5olt; '. McCune"Al#ri&ht (. Menkes A 1A) BP$%! is a *e*#er of of which of the followin& fa*ilies. 1. Plakin $. 1nte&rin %. 8a*inin '. Colla&en (. +lastin A $.1**unoder*atolo&y # Question Answers Corre ct Answ er 1 /he hu*an leukocyte anti&en that has the closet association with psoriasis is: 1. F8A"B(1 $. F8A"0Q% %. F8A"B$A '. F8A"Cw) (. F8A"0H1 0 $ Ferpes si*ple< 3irus"related erythe*a *ultifor*e has #een associated with an increased freLuency of: 1. F8A"BA $. F8A"BC %. F8A"B1% '. F8A"B1( (. F8A"B$A 0 % /he *ost co**on autoi**une disorder in patients with chronic hepatitis C infection is: 1. Autoi**une thyroiditis $. 1diopathic thro*#ocytopenic purpura %. Hheu*atoid arthritis '. S4J&renJs syndro*e (. Syste*ic lupus erythe*atosus A ' /he #est screen for classical pathway co*ple*ent de6ciency or dysfunction is: 1. C0'C0C ratio $. CF(! %. C$ esterase le3els '. /otal C' le3els B 1C (. Seru* electophoresis ( ,hich 3irus is *ost closely associated with KaposiUVWs sarco*a in F1?"infected patients. 1. Fu*an herpes 3irus $ $. Cyto*e&alo3irus %. Fu*an herpes 3irus ) '. Fu*an herpes 3irus C (. +pstein"Barr 3irus 0 ) ,hich of the followin& diseases occur with an increased freLuency in persons de6cient in C$. 1. Psoriasis $. 0er*atitis Ferpetifor*is %. Andro&enetic Alopecia '. 08+ (. 8einerGs disease 0 A ,hich /"cell su#set is co**only found in SJ;ary syndro*e. 1. C0'PC0AP $. C0CPC0A" %. C0'"C0AP '. C0'PC0A" (. C0CPC0AP 0 C ,hich of the followin& is not true a#out the eMects of ultra3iolet radiation on the i**une syste*. 1. B? radiation causes an increase in nu*#er of 8an&erhans cells in the epider*is $. B? radiation causes nuclear 0EA da*a&e %. +Mects can #e de*onstrated #y the e<a*ple of reacti3ation of latent herpes si*ple< infection after sun e<posure '. B? radiation acts to suppress the i**une syste* #oth locally and syste*atically (. +Mects can #e de*onstrated #y the a#ility of an anti&en to induce an aller&ic hypersensiti3ity reaction when applied to skin which has #een e<posed to low doses of B? radiation A D Ma4or histoco*pati#ility co*ple< =MFC> Class 1 *olecules: 1. Are induci#le on keratinocytes $. Co*ple<ed with anti&en tri&&er cytoto<ic / cells %. Are reco∋ed #y receptors on C0'P / cells '. Bear peptides deri3ed fro* patho&ens taken up into 3esicles (. All of the a#o3e B 1! ,hich cytokine is the *ain *acropha&e" acti3atin& cytokine. 1. /E2 $. 12E"&a**a %. 18"' '. 18"1! (. ly*photo<in B 11 1**unocyto*as are: 1. 8ow &rade B"cell ly*pho*as $. A&&ressi3e B"cell ly*pho*as %. 8ow &rade /"cell ly*pho*as '. A&&ressi3e /"cell ly*pho*as (. EK cell ly*pho*as A 1$ A pre3iously healthy child presents with palpa#le purpura: arthritis: and 3o*itin&. Sou suspect a hypersensiti3ity 3asculitis characteri;ed #y: 1. Peri3ascular 1&A $. P"AECA autoanti#odies %. 5ranulo*as and eosinophilia '. 1n6ltration of destruction of 3essels #y atypical ly*phocytoid and plas*acytoid cells (. Eectroti;in& &ranulo*atous 3asculitis A 1% +fali;u*a# is an anti#ody which is directed a&ainst 82A1 on the /"cell: #lockin& this *oleculeGs interaction with: 1. C0'! $. BA %. 82A% '. 1CAM"1 (. P"selectin 0 1' ,hat cytokine is *ost critical for the 1. 1nterleukin"$ C 1D de3elop*ent and *aturation of eosinophils. $. 1nterleukin"' %. 1nterleukin"( '. 1nterferon"alpha (. 1nterferon"&a**a 1( ,hich of the follwin& is a che*otactic factor for eosinophils. 1. /E2 $. 18$ %. C(a '. Plas*ino&en acti3ator (. 18C C 1) ,hen atte*ptin& to identify 8an&erhans cells in a speci*en: which *arker is *ost helpful. 1. C01 $. C0' %. C0A '. C0C (. C0$! A 1A Su#acute cutaneous lupus erythe*atosus has #een associated with the in&estion of which of the followin& dru&s. 1. Phenytoin $. Allopurinol %. /er#ina6ne '. /ri*ethopri*sulfa*etho<a;ole (. Aurano6n C 1C Anti"Ho =SS"A> anti#odies are *ost co**only found in: 1. Mi<ed connecti3e tissue disease $. +osinophilic fasciitis %. 0ru&"induced syste*ic lupus erythe*atosus '. Fo*o;y&ous C$ de6ciency (. Eeonatal lupus erythe*atosus + 1D 9f the followin& co*ple*ent co*ponents: the *ost powerful neutrophil che*oattractant is: 1. C% $. C(a %. C%a '. C(#: C): CA: CC: CD (. C'a B $! ,hich of the followin& suture *aterials induces the least in-a**ation. 1. Sur&ical &ut $. Poly&lycolic acid =0e<on> %. Poly&lycan D1! =?icryl> '. Polypropylene =Prolene> (. Silk 0 $1 ,hich of the followin& /F$ cytokines is a B cell &rowth factor. 1. 18"' $. 18"( %. 18"1! '. 18"1% (. 12E"&a**a ). A $$ /his skin disease has #een shown to #e associated with reduced Beta $ defensin. /he dia&nosis is: 1. Atopic der*atitis $. Psoriasis %. 8epro*atous leprosy '. /u#erculoid leprosy (. Su#acute cutaneous lupus erythe*atosus ). A $% 8inear 1&A disease is *ost closely associated with which of the followin& *edications. 1. +rythro*ycin $. ?anco*ycin %. Strepto*ycin '. A;ithro*ycin (. Clarithro*ycin ). B $' +osinophils are typically seen in the cutaneous in6ltrate of: 1. Kra##eJs disease $. Kaposifor* he*an&ioendothelio*a %. KawasakiJs disease '. Ki*uraJs disease (. Ki"1 ly*pho*a 0 $( /his patient says the rash is spreadin& and not controlled with topical therapy. Sou &i3e hi* a course of oral treat*ent that lasts: 1. 1 week $. $ weeks %. % weeks '. ' weeks (. ( weeks C $) ,hich of the followin& diseases does E9/ respond with a /h1"type responses. 1. 8eish*aniasis which self"resol3es $. 8epro*atous 8eprosy B $! %. /u#erculoid 8eprosy '. Aller&ic contact der*atitis (. Psoriasis $A An&iocentric EK/"cell ly*pho*a in children *ay present as: 1. Papular acroder*atitis of childhood $. Acropustulosis of infancy %. Childhood der*ato*yositis '. Fydroa 3accinifor*e (. +n coup de sa#re 0 $C /he tar&et anti&en of chronic #ullous disease of childhood is: 1. BPA5 1 D$%! kd BPA5> $. DA kd 8A0"1 =a co*ponent of BPA5$> %. Colla&en type ?11 '. Alpha ) #eta ' inte&rin (. Plectin B $D ,hich of the followin& i**uno&lo#ulin crosses the placenta. 1. 1&A $. 1&0 %. 1&+ '. 1&5 (. 1&M 0 %! Anti"centro*eric anti#odies are associated with which rheu*atolo&ic disease. 1. CH+S/ $. Mi<ed connecti3e tissue disease %. S8+ '. 0er*ato*yositispoly*yositis (. Pro&ressi3e syste*ic sclerosis A %1 1n adult patients with Fenoch"SchJnlein purpura with 1&A 3asculitis: which of the followin& co*plications is *ost likely to occur. 1. Pul*onary he*orrha&e $. Fe*orrha&ic cystitis %. Peripheral neuropathy '. Mesan&ial nephropathy (. 2acial ede*a 0 %$ ,hich *onoclonal &a**opathy is *ost co**only associated with erythe*a ele3atu* diutinu*. 1. 1&A $. 1&0 %. 1&+ '. 1&5 (. 1&M A %% Psoriasis aMects o3er $N of the worldJs population and has a stron& association with which F8A class 1 haplotype. 1. F8A"0H' $. F8A"0H1 %. F8A"C,) '. F8A"B$A (. F8A"0Q) C %' 1*iLui*od induces which of the followin& cytokines. 1. 1nterleukin"1! $. 1nterferon"alpha %. 1nterleukin"$ '. 1nterleukin"' (. 1nterleukin"( B %( Fista*ine is a #iolo&ic a*ine produced #y which of the followin& cells. 1. Monocytes $. +osinophils %. Basophils '. Platelets (. Basophils and Platelets + %) ,ith which F8A type is psoriasis *ost de6niti3ely linked. 1. F8A"B(1 $. F8A"BC %. F8A"0H' '. F8A"0H% (. F8A"Cw) + %A ,hich of the followin& F8A alleles is *ost stron&ly associated with BehcetJs disease. 1. F8A"B$A $. F8A"B(1 %. F8A"0Qw$ '. F8A"0H1 (. F8A"0H' B %C All of the followin& state*ents re&ardin& *a4or histoco*pati#ility co*ple< *olecules are true +OC+P/: 1. MFC class 11 *olecules #ind sta#ly to peptides deri3ed fro* proteins sythesi;ed and de&raded in the cytosol $. MFC class 1 *olecules #earin& 3iral peptides are reco∋ed #y cytoto<ic /" cells that su#seLuently kill the infected cell %. Class 1 *olecules such as F8A"A: B: A $1 and C: are present on all nucleated cells '. /he MFC is located on chro*oso*e ) in hu*ans (. MFC Class 11 *olecules #earin& peptides are reco∋ed #y /F1 or /F$ cells. %D /he &ene for E+M9 =E2"kappa #ets essential *odulator> is *utated in: 1. Papillon"8efJ3re syndro*e $. Pachyonychia con&enital type 11 %. 0yskeratosis con&enital '. EoonanJs syndro*e (. 1ncontinentia pi&*enti + '! /opical tacroli*us and pi*ecroli*us are used to treat atopic der*atitis and other in-a**atory skin conditions. 9n which of the followin& ions is the in-a**atory pathway #locked #y these *edications dependent. 1. Sodiu* $. Potassiu* %. Calciu* '. Seleniu* (. Kinc C '1 ,hich of the followin& is a criterion for the dia&nosis of BehcetGsdisease. 1. 1n-a**atory #owel disease $. B3eitis %. Con4uncti3itis '. Easal septal perforation (. 8o#ular panniculitis B '$ All of the followin& state*ents re&ardin& Eatural Killer =EK> cells are true +OC+P/: 1. EK cells ha3e properties of innate and acLuired i**unity $. EK cells e<press C0 $ *olecules %. EK cells are lar&e &ranular ly*phocytes '. EK cells *ediate tu*or lysis (. EK cells *ediate lysis of 3iral" infected cells B '% Ma4or histoco*pati#ility co*ple< class 1 *olecules #ind to: 1. Peptides deri3ed fro* proteins synthesi;ed and de&raded in the cytosol $. Peptides deri3ed fro* proteins de&raded in endocytic 3esicles %. Peptides e<ternal to the cell *e*#rane '. 1**uno&lo#ulin + (. Eone of these answers are correct A '' ,hich cytokine is *ost i*portant in recruitin& neutrophils. 1. 1nterleukin"1 $. 1nterleukin"$ %. 1nterleukin"' '. 1nterleukin"C (. 1nterleukin"1! 0 '( ,hich of the followin& dietary supple*ents *ay inhi#it platelet function. 1. ?ita*in A $. ?ita*in C %. ?ita*in 0 '. ?ita*in + (. ?ita*in K 0 ') Sensation is intact in this lesion: #ut a 2ite stain is positi3e. /his lesions is associated with which of the followin&: 1. 18"' $. 18"( %. 18"1! '. All of these answers are correct (. Eone of these answers are correct 0 'A ,hich cytokine is predo*inantly responsi#le for the 7arish"Fer<hei*er reaction. 1. /E2"alpha $. 18"$ %. 18"( '. 18") (. 18"1$ A 'C All the followin& conditions e<hi#it a /"helper cell 1 =/h1> cytokine secretion pro6le e<cept: 1. Psoriasis $. Syste*ic lupus erythe*atosus %. 5ranulo*atous leprosy '. Hheu*atoid arthritis (. Multiple sclerosis B 'D All of the followin& state*ents re&ardin& 8an&erhans cells are true +OC+P/: 1. /hey are hi&hly pha&ocytic $. /hey e<press C01 on their surface %. /hey are found in so*e areas of ly*ph nodes and spleen '. /hey ha3e a hi&h density of Class 11 A $$ *olecules on their surface (. Eone of these answers are correct (! ,hich of the followin& cytokines is a &eneral down"re&ulator of /F1 i**unity. 1. 18"' $. 18"( %. 18"1! '. /E2 (. 12E"&a**a C (1 All of the followin& stains can #e reacti3e in this condition e<cept 1. C0O111a $. C0%1 %. C0%' '. Ble< europaeus (. 2actor ?111"related anti&en A ($ ,hich of the followin& is true of 8an&erhan cells. 1. /hey are the pri*ary anti&en presentin& cell in the epider*is $. /hey are pri*arily in3ol3ed in the innate i**une response %. /hey do not e<press the C01a *arker '. /hey contain intranuclear #ir#eck &ranules (. /hey are increased on the pal*s: soles: &enitalia: and #uccal *ucosa A (% /his wo*an should ha3e a workup for: 1. 8y*pho*a $. Eephrolithiasis %. Pancreatic cancer '. Fe*ochro*atosis (. /halasse*ia 0 (' ,hich of the followin& is associated with hepatitis C infection. 1. +ssential *i<ed cryo&lo#uline*ia $. Hheu*atoid arthritis %. Helapsin& polychondritis '. ,e&enerJs &ranulo*atosis (. 0er*ato*yositis A (( /he phar*acolo&ic acti3ity of tacroli*us includes: 1. Phosphorylation of E2A/ =nuclear factor of transcription> $. Bindin& and inhi#ition of E2 kappa B %. 1nhi#ition of interleukin"1 &ene transcription '. Acti3ation of calcineurin (. 1nhi#ition of interleukin"$ &ene transcription + () Proteins in the alternate co*ple*ent pathway include: 1. 2actor B $. properdin %. C% '. 2actor B and properdin (. 2actor B: properdin and C% + (A All of the followin& state*ents re&ardin& *ast cells are true +OC+P/: 1. Mast cells reside near s*all #lood 3essels $. Mast cells protect *ucosal surfaces a&ainst patho&ens %. Mast cells release su#stances that aMect 3ascular per*ea#ility '. Mast cells ha3e receptors for certain fra&*ents of co*ple*ent on their surface (. Eone of the a#o3e =all are true> + (C ,hich of the followin& i**une"*ediated e3ents has #een de*onstrated in psoriasis 3ul&aris. 1. Clonal e<pansion of C0CP / cells $. 0ecrease der*al 8an&erhans cells %. 0ownre&ulation of keratin 1) '. 1ncrease /h$ C0'P / cells (. 0ecreased production of interferon" &a**a A (D ,hich cytokine is che*otactic for neutrophils. 1. 18"$ $. 18"% %. 18"( '. 18") (. 18"C + )! All of the followin& state*ents re&ardin& 1. EeutrophilsG *a4or function is A $% neutrophils are true +OC+P/: anti&en presentation $. Eeutrophils ha3e receptors for 1&5 and co*ple*ent %. Eeutrophils are &ranulocytes '. Eeutrophils are the *ost a#undant leukocytes (. Eone of these answers are correct )1 Calcipotriene"induced i*pro3e*ent in psoriasis is associated with increased lesional le3els of which cytokine. 1. 1nterleukin"$ $. 1nterleukin"C %. /u*or necrosis factor '. 1nterleukin"1! (. 1nterluekin"1$ 0 )$ Pe*phi&us is associated with which F8A type=s>. 1. F8A"0Hw) $. F8A"BC %. F8A"B(1 '. All of these answers are correct (. Eone of these answers are correct A )% ,hich cytokine is not upre&ulated in atopic der*atitis patients. 1. 18"1% $. 18"' %. 18"( '. 18"1! (. 12E"&a**a + )' Purpuric contact der*atitis is *ost likely to #e associated with: 1. Eickel $. 2or*aldehyde %. P"phenylenedia*ine '. Propylene &lycol (. Sor#ic acid B )( Seru* 1&A anti#odies to tissue trans&luta*inase occur in: 1. Bullous pe*phi&oid $. 8inear 1&A disease %. Pe*phi&us foliaceus '. Bullous lupus erythe*atosus (. 0er*atitis herpetifor*is + )) 0er*atitis herpetifor*is is associated with which F8A type=s>. 1. F8A"BC $. F8A"0H% %. F8A"0Qw$ '. All of the a#o3e (. Eone of the a#o3e 0 )A ,hich of the followin& #est descri#es the *echanis* of action for in-i<i*a#. 1. 1nhi#ition of calcineurin"*ediated dephosphorylation of transcription factors $. 1nhi#ition of retinoic acid '" hydro<ylase acti3ity %. 1nhi#ition of tu*or necrosis factor alpha acti3ity '. Selecti3e eli*ination of acti3ated / cells 3ia #indin& to hi&h aTnity 18"$ receptor (. Selecti3e /"cell up"re&ulation of 18"' and 18"( production C )C ,hich co**on contact aller&en is detected 3ia the di*ethyl&lyo<i*e test. 1. Ben;ocaine $. Chro*ates %. 2or*aldehyde '. Eickel (. Hhus 0 )D /his disease is *ost associated with: 1. 5a**a interferon $. 18"' %. 18"( '. 18"1% (. Eone of these answers are correct A A! /oll"like receptors =/8Hs> ha3e #een found to play an i*portant role in innate i**unity. /his has #een utili;ed in the de3elop*ent of *edications freLuently used in der*atolo&y. /he *echanis* of what *edication in3ol3es acti3ation of /8HA. 1. Clo#etasol $. /acroli*us %. Cyclosporine '. ("2luorouracil =("2B> (. 1*iLui*od + A1 MFC Class 11 *olecules are present on which of the followin& cell types: 1. B cells $. / cells A $' %. EK cells '. Mast cells (. All of the a#o3e A$ ,hich cytokine is upre&ulated in lesions of tu#erculoid leprosy. 1. 18 $ $. 18 ' %. 18 ( '. 18 1! (. Eone of the answers are correct A A% /his patient recently de3eloped this rash. Sou decide to patch test her: #ut in the *eanwhile you tell her to a3oid: 1. Cha*o*ile $. Pri*in %. A#ietic acid '. Ben;ocaine (. Cinna*on + A' An C *onth"old #a#y with diMuse purpura is ad*itted to the hospital for her third episode of #acterial *enin&itis. ,hich co*ponent of her i**une syste* is i*paired. 1. C0' P / cells $. Eatural killer cell acti3ation %. C0C P / cells '. Co*ple*ent acti3ation (. Anti#ody production 0 A( /he *ost useful pair of i**unohistoche*ical stains to distin&uish #etween 0er*ato6#ro*a protu#erans =02SP> and a 6#rous histiocyto*a would #e: 1. Synaptophysin: chro*o&ranin $. C0%': factor O111a %. C0%': C0%1 '. C0%1: C0% (. ?i*entin: synaptophysin B A) Eai3e / cells e<press which of the followin& surface *olecules: 1. C01D $. C0$! %. C0AD '. C0'(H9 (. C0'(HA + AA ,hich cytokine is up"re&ulated in this &eo*etric: ec;e*atous der*atitis. 1. 18"$ $. 18"' %. 18"( '. 18"A (. 18"1! A AC ,hich of the followin& is an e<a*ple of a delayed hypersensiti3ity reaction. 1. Aller&ic contact der*atitis $. Anaphyla<is %. 8ate< aller&y '. /ransfusion reaction (. Seru* sickness A AD ,hich syste*ic anti"in-a**atory a&ent speci6cally #locks the a#ility of / cells to lea3e the 3asculature and enter the skin. 1. +tanercept $. 1n-i<i*a# %. +fali;u*a# '. Alefacept (. Eone of the a#o3e C C! Helapsin& polychondritis is an autoi**une disease associated with i**unity to which type of colla&en. 1. 1 $. 11 %. 111 '. 1? (. ?11 B C1 A $) year"old *an presents with a history of recurrent episodes of tar&etoid: erythe*atous: ede*atous *acules: patches: and plaLues on the ar*s: le&s: pal*s: and soles. /he *ost likely etiolo&ic a&ent is: 1. Par3o3irus B1D $. Ferpes si*ple< 3irus %. Co<sackie3irus '. Cyto*e&alo3irus (. Fu*an i**unode6ciency 3irus B C$ /he endothelial li&and for cutaneous ly*phocyte anti&en =C8A> is: 1. 1ntercellular adhesion *olecule 1 =1CAM"1> $. 8"selectin %. +"selectin '. ?ascular cell adhesion *olecule =?CAM"1> (. 8eukocyte functional anti&en =82A %> C C% 5ene rearran&e*ent analysis is usedful for deter*inin&: 1. 8y*phocyte clonality in *ycosis fun&oides $. 8y*phocyte acti3ity %. 5ene 2unction '. 5ene Mutations (. / cell receptor status A C' /he *ost de6niti3e F8A association with 1. F8A"Cw) A $( psoriasis is: $. F8A"B$A %. F8A"B1% '. F8A"B1A (. F8A"B%A C( Aller&ic contact der*atitis is caused #y /"cell response to topical e<posure to co*pounds that for* co*ple<es with host proteins =haptens>. ,hich cell is responsi#le for initial sensiti;ation of the /"cells. 1. Melanocyte $. 8an&erhans Cell %. B"cell '. Mast Cell (. Macropha&e B C) ,hich of the followin& causes a photoaller&ic contact der*atitis that is e<acer#ated #y B?A radiation. 1. Ascor#ic acid $. /itaniu* dio<ide %. 9<y#en;one '. Kinc o<ide (. 0ihydro<yacetone C CA /his patient has a licheni6ed plaLue in the lower *id a#do*en as well as these two e<c;e*atous plaLues. /his patient needs: 1. Patch testin& $. A steroid %. An anti3iral '. A K9F scrapin& (. An antifun&al A CC ,hich cytokine is responsi#le for acti3atin& natural killer cells. 1. 1nterleukin ' $. 1nterleukin $ %. 1nterferon"alpha '. /erferon"&a**a (. /u*or necrosis factor"alpha B CD /he classical co*ple*ent pathway: 1. Can #e acti3ated in the a#sence of anti#ody $. Can #e acti3ated #y 1&5' %. Can #e acti3ated #y 1&M '. 1ncludes C% and factor B (. 0oes not cause *e*#rane da*a&e C D! Psoriatic arthritis is *ost co**only associated with which F8A. 1. F8A"B$A $. F8A"Cw) %. F8A"Aw1D '. F8A"Bw%( (. Eone of these options are correct A D1 ,hich of the followin& su#stances is located in the core of an eosinophil. 1. +osinophilic cationic protein $. +osinophil"deri3ed neuroto<in %. +osinophil pero<idase '. Ma4or #asic protein (. Chy*ase ). 0 D$ ,hich co*ponent of the / cell receptor is associated with superanti&en reco&nition. 1. 0"#eta $. 7"alpha %. 7"#eta '. ?"alpha (. ?"#eta + D% ,hich of the followin& is the tar&et anti&en in pe*phi&us 3ul&aris. 1. 0es*o&lein % $. 0es*oplakin %. Cadherin '. /ype O?11 colla&en (. 0es*o&lein 1 A D' A *ale infant presents with thro*#ocytopenia: ec;e*a: and recurrent infections. Sou suspect which of the followin& i**unode6ciency disorders. 1. Ata<ia telan&iectasia $. 0i"5eor&e ano*aly %. Fyper"1&M syndro*e '. 8einerX Ys disease (. ,iskott"Aldrich syndro*e + D( Anti"epili&rin =la*inin (> anti#odies *ay #e seen in: 1. Pe*phi&oid &estationis $. Pe*phi&us 3e&etans %. 2o&o sel3a&e* '. Cicatricial pe*phi&oid (. Paraneoplastic pe*phi&us 0 D) All of the followin& state*ents re&ardin& /oll receptors are true +OC+P/: 1. /oll receptors are present on *acropha&es and dendritic cells $. /oll $ receptors are typically acti3ated #y lipopolysaccharide %. /oll ' receptors are typically acti3ated #y &ra* ne&ati3e #acteria B $) '. Euclear factor kappa B =E2KB> is the 6nal co**on pathway of toll receptors (. Eone of the a#o3e =all are true> DA 2or this patient: you reLuest that the la# perfor* indirect i**uno-uorescence usin& what su#strate. 1. Monkey esopha&us $. Hat #ladder %. 5uinea pi& esopha&us '. Mouse epitheliu* (. Fep"$ cells C DC ,hich of the followin& cytokines: toðer with 18"': pro*otes isotype switchin& fro* 1&M to 1&+. 1. 18"( $. 18"1! %. 18"1% '. /E2 (. 12E"&a**a C DD /he i**uno&lo#ulin *ost co**only found in *ucous secretions is: 1. 1&A $. 1&0 %. 1&+ '. 1&5 (. 1&M A 1! ! /"cell aner&y occurs if: 1. Sti*ulation #y a MFC Class 111 *olecule is in3ol3ed $. MFC/CH en&a&e*ent occurs without costi*ulatory *olecules %. 2as8 is #ound on the /"cell '. MFC Class 1 or 11 is #ound in the presence of 18"$ (. A F8A"0M facilitator is not in3ol3ed with the #indin& B 1! 1 ,hich is not a feature of *ast cells. 1. +<presses c"kit $. Hesponds to HAE/+S %. Produces 18"C '. Produces prosta&landin 0$ (. Stains with napththol chloro"acetate esterase B 1! $ +la#oration of which of the followin& cytokines is characteristic of /F$ response. 1. 1nterferon"C =&a**a> $. 1nterleukin"1 %. 1nterleukin"$ '. 1nterleukin"' (. 1nterleukin"1$ 0 1! % 0er*atitis Ferpetifor*is is *ost co**only associated with which F8A. 1. F8A"0H% $. F8A"B$A %. F8A"BC '. F8A"Bw%( (. F8A"0Q=A1Z!(!1: B1Z!$> + 1! ' Keratinocytes e<press what class of *a4or histoco*pati#ility co*ple< under nor*al conditions. 1. MFC Class 1 $. MFC Class 11 %. MFC Class 111 '. MFC Class 1? (. MFC Class ? A 1! ( A de6ciency of this co*ple*ent co*ponent *ay result clinically in suscepti#ility to pyo&enic infections: &lo*erulonephritis: and partial lipodystrophy: 1. C1 +sterase 1nhi#itor $. C% %. C' '. C(! (. Properidin B 1! ) ,hich anti#ody is *ost co**only found in circulation of patients with atopic der*atitis. 1. 1&A $. 1&0 %. 1&+ '. 1&5 (. 1&M C 1! A ,hich of the followin& i**uno&lo#ulins cannot acti3ate the co*ple*ent pathway. 1. 1&M $. 1&51 %. 1&5$ '. 1&5% (. 1&5' + 1! C /he *a4or histoco*pati#ility co*ple< =MFC>consists of a linked set of &enes encodin& for MFC Class 1: Class 11: Class 111: and Class 1B. ,hich of the followin& isare true: 1. Class 11 *olecules are present on all nucleated cells $. Class 1 *olecules are present on erythrocytes 0 $A %. Class 1 *olecules are e<pressed on BC cells: *onocytes and dendritic cells '. /he le3el of Class 1 and 11 e<pression can #e *odulated #y cytokines (. /he MFC re&ion is located on chro*oso*e 1A in hu*ans 1! D All of the followin& state*ents are true re&ardin& cells of the innate i**une syste* +OC+P/: 1. Eeutrophils ha3e receptors for 1&5 $. Basophils are a type of &ranulocyte: as are neutrophils %. 18"( downre&ulates the functions of eosinophils '. 8an&erhans cells are poorly pha&ocytic (. 8an&erhans cells e<press C01 on their surface C 11 ! /his patient de3eloped an acute 3esicular rash after eatin& a *an&o. She has returned for a routine follow"up. She needs to #e careful of e<posure to: 1. 5ink&o fruit $. Croton %. Ha&weed '. /ea tree oil (. All of these answers are correct A 11 1 ,hich anti#ody can #ind the 2c+H1 portion of *ast cells: #asophils: 8an&erhans cells: der*al dendritic cells. 1. 1&A $. 1&0 %. 1&+ '. 1&5 (. 1&M C 11 $ ,hich paraprotein is found *ost co**only in patients with pyoder*a &an&renosu*. 1. 1&5 $. 1&A %. 1&M '. 1&+ (. 1&0 B 11 % /he hu*an *a4or histoco*pati#ility co*ple< =MFC> is located on chro*oso*e: 1. $ $. ) %. D '. 11 (. 1A B 11 ' Chronic idiopathic urticaria is associated with which F8A type=s>. 1. F8A"0H' $. F8A"0HB' (% %. F8A"0QC '. all of the a#o3e (. none of the a#o3e 0 11 ( A 3ery co**on pentadecacatechol sensiti;er is found in all of the followin& plantsplant co*ponents e<cept: 1. 5in&ko fruit pulp $. Poison su*ac %. Man&o fruit pulp '. Cashew oil (. 1ndian *arkin& nut oleoresin C 11 ) ,hich of the followin& features of 1&5 is true. 1. 1&5 is not an opsoni;in& anti#ody $. 1&5 is the only class of i**uno&lo#ulin that can pass throu&h the placenta %. 1&5 cannot acti3ate the co*ple*ent cascade '. 1&5 represents 1(N of the total protein in seru* (. 1&5 is the second i**uno&lo#ulin synthesi;ed #y the fetus B 11 A Patients with a type 1 reaction to late< *ay ha3e cross"reactions with which of the followin& foods. 1. A3ocado $. Forseradish %. Cashews '. Parsnips (. 5arlic A 11 C Eatural killer =EK> cells eli*inate infected cells in all of the followin& ways e<cept: 1. EK cells adhere to and kill tar&et cells coated with 1&5 $. EK cells secrete perforins %. EK cells secrete &ran;y*e '. EK cells secrete *yelopero<idase (. EK cells do not tar&et cells e<pressin& *a4or histoco*pati#ility 0 $C =MFC> class 1 *olecules 11 D /he *ost likely tar&et for e<foliati3e to<in A in #ullous i*peti&o is: 1. 0es*o&lein % $. 8a*inin ( %. 0es*o&lein 1 '. Colla&en ?11 (. Ces*ocollin C 1$ ! ,hich of the followin& B cell receptors is in3ol3ed in i**uno&lo#ulin isotype switchin&. 1. C0'! $. C01D %. C0$! '. C01(' (. C0$$ A 1$ 1 ,hat is the #est screenin& test for hereditary an&ioede*a. 1. C1 esterase $. C' %. CF(! '. C$ (. C% B 1$ $ /he *ain cytokine secreted #y /h1 C0CP eMector /"cells is: 1. 18"1 $. 18"$ %. 12E"&a**a '. 18"' (. 18"( C 1$ % ,hich cytokine is pri*arily responsi#le for sti*ulation of neutrophils. 1. 18"1 $. 18"' %. 18"( '. 18") (. 18"C + 1$ ' /he anti#ody produced in the early sta&es of anti#ody responses is: 1. 1&A $. 1&0 %. 1&+ '. 1&5 (. 1&M + 1$ ( /his lesion is hypoesthetic and is associated with which of the followin&: 1. 5a**a interferon $. 1nterleukin $ %. 1nterleukin 1$ '. All of these answers are correct (. Eone of these answers are correct 0 1$ ) Mononuclear pha&ocytes residin& in tissues: 1. Are called *acropha&es $. Pha&ocytose forei&n anti&ens and de&rade the* into peptides %. Present anti&en to /"cells '. Produce cytokines: which recruit other in-a**atory cells (. All of the a#o3e + 1$ A ,hich syste*ic anti"in-a**atory a&ent tar&ets C0$P acti3ated / cells for apoptosis. 1. +tanercept $. 1n-i<i*a# %. +fali;u*a# '. Alefacept (. Eone of the a#o3e 0 1$ C ,hich cytokine is responsi#le for fe3er in patients with sun#urn. 1. 18"1 $. 18"( %. 18"1! '. 18"11 (. /E2"#eta A 1$ D /his patient had anti#odies to 1C! kd anti&en. /he likely dia&nosis is: 1. Bullous pe*phi&oid $. Pe*phi&us 3ul&aris %. Pe*phi&us foliaceous '. +rythe*a *ultifor*e (. Bullous lichen planus A 1% ! Anti 7o"1 anti#odies are directed a&ainst which of the followin&. 1. /opoiso*erase $. 8ysyl o<idase %. 5yrase '. Fistidyl transfer HEA synthetase (. /elo*erase 0 1% 1 Ferpes &estationis is *ost co**only associated with which F8A. 1. F8A"0H% $. F8A"B$A %. F8A"B(1 '. F8A"0HD A $D (. F8A"0QC 1% $ /he putati3e *echanis* of action of topical *acrolide i**uno*odulators is inhi#ition of: 1. 8y*phokine production $. Prosta&landin secretion %. Anti&en presentation '. Eeutrophil *i&ration (. 8y*phocyte *i&ration A 1% % A patient had anti#odies to des*o&lein %: #ut no anti#odies to des*o&lein 1 or des*plakin. /he likely dia&nosis is: 1. Pe*phi&us 3ul&aris $. Pe*phi&us foliaceous %. Bullous pe*phi&oid '. 0er*atitis herpetifor*is (. +rythe*a *ultifor*e A %. 5enoder*atoses # Question Answers A 1 Mucosal neuro*as: pheochro*ocyto*a and *edullary thyroid carcino*a in a patient with a *arfanoid #ody ha#itus is associated with which of the followin& &ene defects. 1. Menin $. H+/ proto"onco&ene %. P/+E '. BF0 (. S/K11 B $ ,hich of the followin& syndro*es is associated with he*atolo&ic a#nor*alities. 1. Stur&e ,e#er disease $. Klippel"/renaunay"Parks",e#er %. Kasa#ach"Merritt syndro*e '. Blue ru##er #le# ne3us syndro*e (. Bloo*Js syndro*e C % /he 6ndin& of G*altese crossesG in the 1. Alkaptonuria B %! urine is characteristic of which of the followin& conditions. $. 2a#ry disease %. 5aucher disease '. Eei*ann"Pick disease (. Funter syndro*e ' ,hich of the followin& i**uno&lo#ulins is co**only decreased in ,iskott"Aldrich syndro*e. 1. 1&A $. 1&0 %. 1&+ '. 1&M (. 1&5 0 ( /he nucleotide e<cision 0EA repair pathway is defecti3e in which disease: 1. Bourne3illeGs disease $. Se3ere co*#ined de6ciency syndro*e %. 5riscelli syndro*e '. Oeroder*a pi&*entosa (. S4o&ren"8arssen syndro*e 0 ) ,hich type of epider*olysis #ullosa si*ple< is associated with early death. 1. ,e#er"Cockayne $. 5enerali;ed =Koe#ner> %. 0owlin&"Maera '. 9&na 3ariant (. Eon"Ferlit; 3ariant ). C A Mosaic *utations in P/+E are seen in which of the followin& conditions. 1. 5ardner syndro*e $. Proteus syndro*e %. 1ncontinentia pi&*enti '. Eoonan syndro*e (. Beckwith",ieder*an syndro*e ). B C ,hich of the followin& is a feature of Eeuro6#ro*atosis type 11. 1. Con&enital hypertrophy of the retinal pi&*ent epitheliu* $. 8isch nodules %. 7u3enile posterior su#capsular lenticular opacities '. 8ester iris (. 9ptic &lio*as C D A patient with colon cancer is dia&nosed with Muir"/orre syndro*e. ,hich of the followin& cutaneous lesions *i&ht the patient ha3e. 1. Keratoacantho*as $. Se#orrheic keratoses %. /richole**o*as '. Arsenical keratoses (. Basal cell carcino*as ). A 1! An infant with dou&hy: redundant skin and short sparse hairs is likely to show which features on <"ray. 1. Metaphyseal widenin& in the lon& #ones $. Sphenoid win& dysplasia %. Periosteal thickenin& '. 9steopoikilosis (. Stippled epiphyses A 11 ,hich ocular 6ndin& *ay #e seen in a patient with this skin condition. 1. Co**a"shaped corneal opacities $. Hetinitis pi&*entosa %. Con&enital hypertrophy of the retinal pi&*ented epitheliu* '. An&ioid streaks (. Pin&ueculae 0 1$ Fo*ocystinuria is caused #y a defect in: 1. Phenylalanine hydro<ylase $. Biotinidase 0 %1 %. Folocar#o<ylase synthetase '. Cystathione #eta"synthetase (. 5pD1"pho< 1% A patient with *elano*a and a *ali&nant &lio*a is dia&nosed with 8i" 2rau*eni syndro*e. ,hich of the followin& tu*ors occurs *ost freLuently in this disease. 1. Hha#do*yosarco*a $. Adrenocortical carcino*a %. 8un& carcino*a '. Breast carcino*a (. 8euke*ia A 1' ,hich keratins are e<pressed in the supra#asal pal*oplantar epider*is. 1. Keratins 1 and D $. Keratins 1 and 1! %. Keratins ' and 1% '. Keratins ( and 1' (. Keratins C and 1C A 1( /he *ain cause of death in patients with dyskeratosis con&enita is which of the followin&. 1. 9ral sLua*ous cell carcino*a $. 8euke*ia %. Henal cell carcino*a '. Pancytopenia (. Atherosclerotic heart disease 0 1) ,hat is the classic radiolo&ic 6ndin&s associated with this disorder. 1. 0ural calci6cations $. Calci6cations of the fal<"cere#ri %. /ra*"track calci6cations of the te*poral and occipital corte< '. 9steopatha striata (. 9steopoikilosis C 1A A % year"old #oy presents with the 6ndin&s seen in the i*a&e. Fe also has thro*#ocytopenia with purpura and a history of recurrent pyo&enic #acterial infections. ,hat is the *ost likely dia&nosis in this child. 1. Chronic 5ranulo*atous disease $. ,iskott"Aldrich syndro*e %. Fyper"1&+ syndro*e '. Se3ere co*#ined i**unode6ciency (. 8einer syndro*e B 1C ,hich of the followin& condition is E9/ found in ?on"Fippel 8indau syndro*e. 1. Connecti3e tissue ne3i $. Bilateral retinal he*an&io#lasto*as %. Cere#ellarCES he*an&io#lasto*as '. Henal cell carcino*a (. Pheochro*ocyto*a A 1D ,hat is the *ost co**on ocular 6ndin&s seen in this condition. 1. An&ioid streaks $. Phako*as %. 8isch nodules '. Bitot[s spots (. Colo#o*a A $! A patient with CroweJs si&n and an optic &lio*a has which of the followin& disorders. 1. Eeuro6#ro*atosis 1 $. Eeuro6#ro*atosis 11 %. ,atson syndro*e '. /u#erous sclerosis (. 8ester iris syndro*e A $1 MaMucci syndro*e is has characteristic 3enous *alfor*ations of the distal e<tre*ities and #eni&n endochondro*as which can co*pro*ise #one stren&th and lead to chondrosarco*as. /he defect causin& this #elie3ed to #e the P/FP/FrP type 1 receptor which is inherited in which 1. Sporadic $. Autoso*al do*inant %. Autoso*al recessi3e '. O"linked do*inant (. O"linked recessi3e A %$ *anner. $$ ,hich cutaneous 6ndin& is seen in patients with phenylketonuria. 1. An&ular sto*atitis $. 1chthyosis %. Pi&*ent dilution of hair and skin '. Phyrnoder*a (. +rosi3e diaper der*atitis C $% A patient with renal cell carcino*a caused #y *utations in fu*arate hydratase de6ciency likely suMers which of the followin& conditions. 1. ?on"Fippel"8indau syndro*e $. Cowden syndro*e %. Birt"Fo&&"0u#e syndro*e '. 2a*ilial *ultiple cutaneous leio*yo*atosis (. Multiple endocrine neoplasia 0 $' A patient presents with focal sy**etric pal*oplantar keratoder*a: thickened: hyperkeratotic 6n&ernails and toenails with a IpincerI appearance and freLuent staph and candida paronychial infections: follicular hyperkeratosis of the knees and el#ows and oral leukokeratosis. /he patients *other and &randfather #oth ha3e si*ilar skin 6ndin&s. ,hich syndro*e is descri#ed. 1. 7adassohn"8ewandowsky syndro*e $. 7ackson"8awler syndro*e %. Schafer"Branauer syndro*e '. Pachyonychia con&enita tarda (. Eone of the options are correct A $( An infant &irl of short stature and shortened 'th and (th *etacarpals is #ein& e3aluated for coarctation of the aorta and horseshoe kidneys. Physical e<a*ination *ost likely re3eals: 1. ,e##ed neck $. Alopecia %. Fe*an&io*a '. 5iant con&enital *elanocytic ne3us (. Arachnodactyly A $) A patient with $! nail dystrophy: steatocysto*a *ultiple< and natal teeth likely has a *utation in the &enes codin& for: 1. Keratins ( $. 8a*inin ( %. Plakophilin 1 '. Keratins )# @ 1A (. Keratins ) @1) 0 $A 8ow"cystine content in hair and nails *ay contri#ute to the phenotype seen in: 1. ,ilsonJs disease $. MenkeJs Kinky Fair syndro*e %. /ay Syndro*e '. Eethertons (. B4ornstad C $C +pider*olysis #ullosa with *uscular dystrophy is caused #y *utations in which of the followin&. 1. Keratins ( and 1' $. Plectin %. 8oricrin '. Colla&en A (. Colla&en 1A B $D ,hich of the followin& hereditary skin disorders is associated with the HAS" +HK"MAPK pathway. 1. Costello syndro*e $. Hoth*und"/ho*pson Syndro*e %. Carney co*ple< '. /u#erous Sclerosis (. 5riscelli syndro*e A %! ,hat &ene defect would you e<pect to 6nd in a child with white forelock: dystopia canthoru*: and upper li*# a#nor*alities. 1. Pa<% $. M1/2 %. S9O1! '. +ndothelin"% (. C"kit proto"onco&ene A %% %1 Bnderlyin& defect for the disease shown in picture is 1. A/P$A$ $. A/P$C1 %. BPA51 '. BPA5$ (. Colla&en type 1A B %$ 0ental ena*el pits are seen in which of the followin& conditions. 1. Fypo*elanosis of 1to $. 8etterer"Siwe disease %. /u#erous sclerosis '. 7ackson Sertoli syndro*e (. Fyper"1&+ syndro*e C %% Hefsu* syndro*e is due to a de6ciency in phytanyl coen;y*e A hydro<ylase. /reat*ent for this condition is: 1. 0iet low in &reen 3e&eta#les: dairy and ru*inant fats $. 0iet hi&h in &reen 3e&eta#les: dairy and ru*inant fats %. +n;y*e replace*ent '. Eo treat*ent is a3aila#le at this ti*e (. A3oid phenylalanine A %' A 1) year"old &irl presents with a fa*ily history of 5ardner syndro*e. Fer *other is 3ery concerned that her dau&hter *ay ha3e the syndro*e as it runs in her fa*ily and she has *any skin co*plaints. 5ardner syndro*e has #een linked to defects in #eta" catenin *ediated transcription. ,hich of the followin& ocular 6ndin& is dia&nostic for 5ardner syndro*e. 1. 8isch nodules $. 8ester iris %. Con&enital Fypertrophy of the Hetinal Pi&*ent +pitheliu* '. An&ioid streaks (. Hetinal detach*ent C %( A teena&e fe*ale presents with the co*plaint of Inail fun&usI. 9n e<a*: she has trian&ular lunulae: pal*oplantar hyperhidrosis: *icronychia and an a#sent patella. ,hich of the followin& screenin& tests should you order 6rst. 1. Brinalysis $. CBC %. 2astin& lipids '. Henal ultrasound (. O"ray of the knees: el#ows and pel3is A %) Mutation in la*in A =nuclear en3elope protein> has #een found in: 1. Peut;"7e&hers syndro*e $. Buschek"9llendorf syndro*e %. Pro&eria =Futchinson"5ilford> '. Al#ri&htJs syndro*e (. Marfan syndro*e C %A 1n chronic &ranulo*atous disease: the dia&nosis is *ade #y which of the followin& tests. 1. Assay for fu*arate hydratase $. Eitro#lue tetra;oliu* reduction assay %. Assay for sphin&o*yelinase '. Skin #iopsy (. Assay for &lucocere#rosidase B %C A %"year old #oy has freLuent a#scesses: chronic ear infections: and se3ere ec;e*a. Fe has an older #rother with si*ilar pro#le*s. ,hat is the &ene *utation. 1. Stat % &ene $. 2B8E ( &ene %. 18$ receptor &ene '. 8+M0% &ene (. ,AS &ene A %D Hetinal he*an&io#lasto*as are found in which syndro*e: 1. 9sler",e#er"Hendu disease $. ?on"Fippel 8indau disease %. Kasa#ach"Merritt syndro*e '. Klippel"/renaunay ,e#er B %' syndro*e (. Stur&e",e#er syndro*e '! A 11 year"old fe*ale patient with hypoparathyroidis* is referred to your clinic secondary to chronic *ucocutanous candidiasis which is refractory to standard treat*ents. /he patient also has *ala#sorption and se3ere chronic diarrhea. Sou deter*ine that she has autoi**une polyendocrinopathy"candiasis" ectoder*al dystrophy syndro*e=AP+C+0>. ,hat is the &ene defect for AP+C+0. 1. OAP1!1 &ene $. A1H+ &ene %. C51"(C &ene '. A89O &ene (. PAFO &ene B '1 /he <"linked recessi3e type of dyskeratosis con&enita is: 1. 0yskerin $. /+HC %. C0KE$A '. P/+E (. Menin ). A '$ ,hich &enetic defect could e<plain cutaneous 6ndin&s in addition to a#nor*al i**uno&lo#ulin le3els: recurrent respiratory infections: hypo&onadis*: and an increased risk of leuke*ia and ly*pho*a. 1. HecQ8% $. +HCC) %. ,AS &ene '. EA0PF o<idase (. Adenosine dea*inase ). A '% Most co**on *ali&nancy to de3elop in a patient with trichole**o*as: acral 3errucous papules and co##le" stonin& of #uccal and &in&i3al *ucosa. 1. /hyroid cancer $. Breast cancer %. Colon cancer '. 8y*pho*a (. Melano*a B '' /he *ost co**on cutaneous neoplas* associated with Muir /orre Syndro*e is: 1. Se#aceous carcino*a $. Se#aceous adeno*a %. Keratoacantho*a '. Basal cell carcino*a with se#aceous diMerentiation (. SLua*ous cell carcino*as B '( /he *ost co**on cutaneous association with *onilethri< is: 1. +c;e*a $. Fypopi&*entation %. Fyperpi&*entation '. Keratosis Pilaris (. Atrophy 0 ') A $!"year old *ale with a history of pheochro*ocyto*a and *edullary thyroid cancer presents with *ucosal papules. Fis o3erall #ody appearance is *ost likely to de*onstrate: 1. Cushin&oid features $. Marfanoid features %. Short stature '. 8ipodystrophy (. Bnilateral li*# shortenin& B 'A An infant presents with poikiloder*a on his face: #uttocks: ar*s and le&s. Fe is also noted to ha3e a hypoplastic thu*# and no radius. Searly ophthal*olo&ic e<a*ination is indicated #ecause of the infant is at risk for de3elopin&: 1. 5lauco*a $. Cataracts %. Su#capsular lens displace*ent '. Copper deposition (. Macular de&eneration B 'C /he co*#ination of &astrointestinal 1. Eicolau"Balus syndro*e C %( polyposis: nail atrophy: alopecia: &enerali;ed pi&*entation of skin: and *elanotic *acules of the 6n&ers is characteristic of which of the followin& syndro*es. $. Peut;"7e&hers syndro*e %. Cronkhite"Canada syndro*e '. Cowden syndro*e (. Bannayan"Hiley"Hu3alca#a syndro*e 'D ,hat phenotype results fro* a low acti3ity of dou#le stranded HEA adenosine dea*inase. 1. ,aarden#er&Gs syndro*e type $ $. Pie#aldis* %. /iet; syndro*e '. dyschro*atosis sy**etrica hereditaria (. oculocutaneous al#inis* type ' 0 (! Anodontia is a #one 6ndin& seen in which of the followin& conditions: 1. Fypo*elanosis of 1to $. 8etterer"Siwe disease %. /u#erous sclerosis '. 7ackson Sertoli syndro*e (. Fyper"1&+ syndro*e A (1 0ystrophic epider*olysis #ullosa is associated with *utations in colla&en ?11. /rau*a or friction induced #listerin& in these patients ha3e a plane a splittin& in the: 1. Su#la*ina densa $. Stratu* spinosu* %. 8a*ina lucida '. Stratu* #asale (. Eone of these answers are correct A ($ A patient with *ultiple se#aceous adeno*as should #e screened with which of the followin& e<a*inations. 1. Hetinal e<a*ination $. 8aryn&oscopy %. Colonoscopy '. MH1 of the spine (. Henal ultrasound C (% A 1) year"old &irl presents with a fa*ily history of 5ardner syndro*e. Fer *other is 3ery concerned that her dau&hter *ay ha3e the syndro*e as it runs in her fa*ily and she has *any skin co*plaints. ,hat is likelihood that this &irl has 5ardner syndro*e #ased on what you now a#out the inheritance pattern and the fact that her father is unaMected and her *other is a hetero;y&ote for this condition. 1. (!N $. $(N %. 1!N '. A(N (. D!N A (' ,hich *ali&nancy is associated with Cowden syndro*e. 1. Colon cancer $. Henal cancer %. 8un& cancer '. 93arian cancer (. Basal cell cancer A (( ,hich of the followin& disorders is associated with delayed separation of the u*#ilical cord. 1. 8eukocyte adhesion de6ciency type 1 =8A0"1> $. 1**unedysre&ulation: polyendocrinopathy: enteropathy: <"linked =1P+O> %. Se3ere co*#ined i**unode6ciency disorder=SC10> '. O"linked a&a**a&lo#uline*ia (. Myelopero<idase de6ciency A () ,hich of the followin& is true re&ardin& tu#erous sclerosis. 1. Confetti"like *acules are typically present at #irth 0 %) $. 2acial an&io6#ro*as are the *ost co**on cutaneous *anifestation %. Fypo*elanotic *acules =ash leaf spots> ha3e a decreased nu*#er of *elanocytes '. Periun&ual 6#ro*as are considered a *a4or feature in the dia&nosis of tu#erous sclerosis (. ) or *ore hypo*elanotic *acules =ash leaf spots> are considered a *a4or feature in the dia&nosis of tu#erous sclerosis (A Cutaneous osteo*as are seen in which syndro*e. 1. ,aarden#ur& syndro*e $. 8+9PAH0 syndro*e %. Carney co*ple< '. Al#ri&ht hereditary osteodystrophy (. 5aucherJs syndro*e 0 (C /he &ene P/+E =phosphatase and tensin ho*olo& deleted on chro*oso*e ten> is i*plicated in which of the followin& syndro*es. 1. Cowden $. Banayan"Hiley"Hu3alca#a %. Proteus '. All of the options are correct (. Eone of the options are correct 0 (D A sporadic syndro*e aMectin& transcriptional coacti3ator CH+B" #indin& protein is: 1. Hu#instein"/ay#i syndro*e $. Cornelia de 8an&e syndro*e %. Eonne"Milroy disease '. MaMucci syndro*e (. Blue ru##er #le# ne3us syndro*e A )! A dou#le row of eyelashes is associated with: 1. 8y*phede*a"distichiasis syndro*e $. Cornelia de 8an&e syndro*e %. Hu#instein"/ay#i syndro*e '. Hussell"Sil3er syndro*e (. Funters syndro*e A )1 Co**a shaped corneal opacities are seen in what disease. 1. Hefsu* Syndro*e $. S4o&ren"8arson Syndro*e %. Pseudo<antho*a elasticu* '. O"linked ichthyosis (. Proteus syndro*e 0 )$ Oeroder*a pi&*entosu* =OP> 3ariant is diMerent than classic OP in which of the followin& way. 1. 0efecti3e 0EA nucleotide e<cision repair of the &lo#al &eno*e $. 0efecti3e post"replication repair %. 1ncreased chro*oso*al #reaka&e and sister chro*atid e<chan&es '. 0efecti3e 0EA nucleotide e<cision repair of acti3ely transcri#in& &enes (. 8ow 1&M B )% A 1) *onth"old &irl presents with patchy alopecia: whorled 1. Brain MH1 $. Alkaline phosphatase 0 %A erythe*atous scaly eruption: and asy**etric li*# shortenin&. ,hat la#oratory or radiolo&ic test *ay aid in dia&nosis. %. Chest radio&raph '. Bone 6l*s (. Co*plete #lood count )' ,hat is the inheritance pattern of der*atosis with acantholysis. 1. Autoso*al do*inant $. Autoso*al recessi3e %. O"linked do*inant '. O"linked recessi3e (. Sporadic A )( A patient presents with focal sy**etric pal*oplantar keratoder*a: thickened: hyperkeratotic 6n&ernails and toenails with a IpincerI appearance and freLuent staph and candida paronychial infections: follicular hyperkeratosis of the knees and el#ows and oral leukokeratosis. /he patients *other and &randfather #oth ha3e si*ilar skin 6ndin&s. ,hat is the defect in PC type 1. 1. Keratin )a1) $. Keratin )#1A %. Keratin 11! '. Keratin $e1! (. Eone of these options are correct A )) A $ year old &irl presents with sunken eyes: lar&e ears: *icrocephaly and a photodistri#uted eruption on her face. +ye e<a* re3eals Jsalt and pepperJ retina. /he &ene responsi#le for this syndro*e codes for a: 1. /ranscription factor $. Surface &lycoprotein %. 8ysoso*al protease '. 0EA helicase (. Mis*atch repair &ene 0 )A 7unctional epider*olysis #ullosa with pyloric atresia is associated with *utations in: 1. /he alpha") su#unit of inte&rin $. /he #eta"' su#unit of inte&rin %. Both su#units of inte&rin can ha3e *utations causin& this type of 4unctional epider*olysis #ullosa '. Plectin (. 8a*inin ( C )C ,hich of the followin& is caused #y a defect in a &ap 4unction protein. 1. +pider*olysis #ullosa si*ple< $. Failey"hailey %. +rythrokeratoder*a 3aria#ilis '. 0yskeratosis con&enita (. Bullous ichthyosis of sie*ens C )D ,hich of the followin& elastic tissue diseases de*onstrates calci6ed elastic 6#ers. 1. Cutis la<a $. Marfan syndro*e %. Anetoder*a '. Pseudo<antho*a elasticu* (. Buschke"ollendorf syndro*e 0 A! Christ"Sie*ens"/ouraine Syndro*e is *ost co**only linked with defects in which of the followin& &enes. 1. E+M9 $. +ctodysplasin =+0A> %. +HCC$ '. A/PAA (. Eone of these options are correct B A1 ,hich of the followin& conditions is worsened #y in&estion of lithiu*. 1. 0arierJs 0isease $. Failey"Failey 0isease %. Fai*"Munk syndro*e '. Fereditary ly*phede*a =Eonne"Milroy disease> (. +pider*olytic hyperkeratosis A %C A$ 9n cutaneous e<a*: an&iokerato*a corporis diMusu* is characteristic of which of the followin& conditions. 1. Sialodosis $. 2ucosidosis %. 2a#ry disease '. All of these options are correct (. Eone of these options are correct 0 A% ,hat is the inheritance pattern of chronic &ranulo*atous disease. 1. Autoso*al recessi3e $. Autoso*al do*inant %. O"linked recessi3e '. O"linked do*inant (. Sporadic C A' Pre*ali&nant leukoplakia of the oral *ucosa is associated with: 1. Bloo* syndro*e $. ,erner Syndro*e %. Oeroder*a Pi&*entosu* '. 0yskeratosis Con&enita (. Hoth*und"/ho*son syndro*e 0 A( +lectron *icroscopic e<a*ination of a hair shaft re3eals a canal"like &roo3e alon& the shaft of a trian&ular"shaped hair. /his patient has: 1. Eetherton[s syndro*e $. Menke[s Kinky Fair syndro*e %. Spun"&lass hair '. /richothiodystrophy (. B4ornstad syndro*e C A) A *entally retarded patient is found to also ha3e a *alar rash and downward lens displace*ent. Another clinical feature *i&ht include: 1. Fypertrichosis $. Short stature %. Multiple ne3i '. Periodontitis (. 0eep 3enous thro*#oses + AA ,hich of the followin& si&ns is not a criteria for the dia&nosis of Eeuro6#ro*atosis type 1. 1. Bilateral 3esti#ular schwanno*as $. A<illary frecklin& %. 9ptic &lio*as '. 5reater than ( cafJ"au"lait *acules (. Sphenoid dysplasia A AC /he de3elop*ent of which *ali&nancy is *ost co**only associated with ly*pho*atoid papulosis. 1. Eon"Fod&kinGs ly*pho*a $. Mycosis fun&oides %. Multiple *yelo*a '. 1**uno#lastic ly*pho*a (. ,aldenstro*Gs *acro&lo#uline*ia B AD Cru*pled ears are associated with which disorder. 1. +hlers"0anlos Syndro*e $. Marfan syndro*e %. Con&enital contractural arachnodactyly '. Cutis la<a (. Buschke"9llendorf syndro*e C C! ,hich of the followin& is caused #y a *utation in a &ene that leads to defecti3e E2"KB acti3ation. 1. M+E 11a $. Pie#aldis* %. Chediak"Fi&ashi '. ?ohwinkelJs (. 1ncontinentia pi&*enti + C1 9donto&enic cysts and pal*oplantar pits are seen in: 1. 5ardnerUVWs syndro*e $. 5orlin Syndro*e %. Bloo*UVWs Syndro*e '. 5olt; Syndro*e (. Hefsu* syndro*e B %D C$ A '( year old wo*an with history of uterine 6#roids presents with painful 6r* papules of the left upper ar*. Biopsy of one of these lesions re3eals s*ooth *uscle fascicles. She notes a history of renal cell cancer in her fa*ily. /he &ene i*plicated in this syndro*e has which of the followin& functions. 1. /elo*ere *aintenance $. 1n3ol3ed in the Kre#Gs cycle %. 1nhi#its */9H si&nallin& '. 1*pairs folliculin function (. +ncodes a deu#iLuitinatin& en;y*e B C% ,hich *ali&nancy is seen in appro<i*ately 1("$!N of people with the disease characteri;ed #y a defect in a parathyroid hor*one receptor protein. 1. 9steosarco*a $. An&iosarco*a %. Chondrosarco*a '. Hha#do*yosarco*a (. +pitheliod sarco*a C C' A patient with pseudoherpetic keratitis and a painful PPK would i*pro3e with a diet low in: 1. /yrosinePhenylalanine $. 5lycine %. Cytosine '. Biotin (. Kinc A C( Colla&en 111 is *utated in which type=s> of +hlers"0anlos syndro*e =+0S>. 1. All of the answers are correct $. Eone of the answers are correct %. Beni&n Fyper*o#ile =type 111> '. ?ascular =type 1?> (. Periodontitis =type ?111> ). A C) A child presents with preti#ial hyperpi&*entation: ata<ia: decreased *otor coordination: cirrhosis: and decreased *otor coordination. /he physical e<a* which would re3eal the *ost speci6c 6ndin& for this disease is: 1. Fearin& test $. Slit"la*p eye e<a* %. +K5 '. Colonoscopy (. Henal ultrasound ). B CA ,hich of the followin& diseases is caused #y a *utation in a &ap 4unction protein. 1. Striated PPK $. Schopf"Schul;"Passar&e syndro*e %. Mal de Meleda '. ?ohwinkel syndro*e =classic> (. ?ohwinkel syndro*e =ichthyotic> ). 0 CC /he followin& en;y*e defect is *ost co**only seen in CF180 Syndro*e. 1. %"#eta"hydro<ysteroid dehydro&enase $. %"#eta"hydro<ysteroid iso*erase %. Aryl sulfatase + '. EA0 o<ido reductase (. 0EA helicase ). A CD +hlers"0anlos Syndro*e with con&enital adrenal hyperplasia is caused #y *utations aMectin& which of the followin&. 1. 8ysyl hydro<ylase $. Colla&en ( %. 2i#ronectin '. 8ysyl o<idase (. /enascin"O ). + D! A % year old #oy has white hair of the central frontal scalp and depi&*ented 1. 1t is an autoso*al recessi3e disorder of *elanocyte B '! sy**etrical patches on the knees since #irth. Eo ocular a#nor*alities or deafness are noted. ,hat is true of this disorder. de3elop*ent $. /here is typically no pro&ression of depi&*ented patches %. He&ression of the white forelock has #een noted '. 9ne for* of this condition is associated with iris pi&*entary a#nor*alities (. Hesults fro* a *utation in a tu*or suppressor &ene ). D1 Fereditary Fe*orrha&ic /elan&iectasia syndro*e is trans*itted in an autoso*al do*inant fashion and can ha3e two 3ariants. /ype 1 is linked to defects in FF/1: the endo&lin &ene. /ype 11 is linked to defects in FF/$: the A8K1 &ene. ,hat feature that diMerentiates type 1 fro* type 11 clinically. 1. /ype 1 fa*ilies ha3e an increase incidence of pul*onary arterio3enous 6stulas $. /ype 11 fa*ilies ha3e an increased incidence of pul*onary arterio3enous 6stulas %. /ype 1 fa*ilies ha3e an increased incidence of hepatic arterio3enous *alfor*ations '. /ype 11 fa*ilies ha3e a decreased incidence of hepatic arterio3enous *alfor*ations (. Eone of the answers are correct ). A D$ A teena&e fe*ale presents with the co*plaint of Inail fun&usI. 9n e<a*: she has trian&ular lunulae: pal*oplantar hyperhidrosis: *icronychia and an a#sent patella. ,hich of the followin& &ene defects is *ost likely in this patient. 1. 8MO1B $. C98(A1 %. ++C1 '. 8KB1 (. E+M9 ). A D% Heticulate pi&*entation of skin: poikiloder*a: alopecia: nail atrophy: pre*ali&nant oral leukoplakia: and a 2anconi"type pancytopenia resultin& in early death in addition to posterior fossa *alfor*ations is characteristic of which of the followin& syndro*es. 1. 0yskeratosis con&enita $. Foyeraal"Freidarsson syndro*e %. Bloo* syndro*e '. Cockayne syndro*e (. ,iskott"Aldrich syndro*e ). B D' 2indin&s of dysplastic ne3i and *elano*a inherited in an autoso*al do*inant fashion is linked with which of the followin&. 1. Breast *ali&nancy $. /hyroid *ali&nancy %. Pancreatic *ali&nancy '. Henal cell carcino*a (. Colon carcino*a ). C D( ,hich type of epider*olysis #ullosa is associated with *itten defor*ities of the hands. 1. 0o*inant dystrophic $. Hecessi3e dystrophic %. ,e#er"Cockayne '. Ferlit; type (. 5enerali;ed atrophic #eni&n epider*olysis #ullosa =5AB+B> ). B D) Pre*ature a&in& seen in CockayneJs syndro*e is caused #y a *utation in 1. /rans&luta*inase $. A/M &ene + '1 which &ene. %. Patched &ene '. E+M9 &ene (. 0EA helicase &ene ). DA 1n one 3ariant of epider*olysis #ullosa si*ple<: those aMected ha3e *uscular dystrophy in addition to the skin 6ndin&s. ,hich protein *utation has #een linked to this 6ndin&. 1. Plako&lo#in $. Plakophilin %. Plectin '. 0es*o&lein (. 0es*ocollin ). C DC A patient with a port wine stain co3erin& one enlar&ed le& likely has which of the followin& associated sy*pto*s. 1. 8y*phatic and deep 3enous insuTciency $. ?iscero*e&aly with o*phalocele %. Bilateral retinal he*an&io#lasto*as '. +nchondro*as (. 0istichiasis ). A DD Patients with 0arierJs disease are at increased risk for: 1. KaposiJs 3aricellifor* eruption $. Melano*a %. 0ecreased life span '. Basal cell carcino*a (. 8ipid a#nor*alities ). A 1! ! 0efects in 2i#rillin $ are linked with: 1. Con&enital contractural arachnodactyly $. Cutis 8a<a %. Arthrochalasis *ultiple< con&enita '. 9ccipital horn syndro*e (. 8ipoid proteinosis ). A 1! 1 /he #est screenin& test for he*ochro*atosis is: 1. 2erritin $. Fe*atocrit %. Co*plete Blood Count '. /ransferrin (. Copper le3els ). A 1! $ A A year old #oy presents to your oTce with short and sparse hair. Fe is also short for his a&e. A hair *ount re3eals alternatin& li&ht and dark #ands under polari;ed li&ht. ,hich of the followin& state*ents is correct re&ardin& this condition. 1. +ye#rows and eyelashes are not aMected $. /here is no increased risk of skin cancer %. A*ino acid analysis of hair re3eals hi&h sulfur content '. /here is no increased hair fra&ility. (. 0ark #ands represent air ). B 1! % ,hich of the followin& conditions is inherited in an O"linked do*inant =O0> *anner. 1. Chodrodysplasia punctata $. CF180 Syndro*e %. 2ocal der*al hypoplasia '. All of the answers are correct (. Eone of the answers are correct ). 0 '$ 1! ' ,hat *edication *ay e<acer#ate this autoso*ally do*inant: acnatholytic disorder. 1. Phenytoin $. 8ithiu* %. 9ral contracepti3es '. Anti"*alarials (. Corticosteroids ). B 1! ( Pruritus is S4o&ren 8arsson syndro*e is attri#uted to accu*ulation of what *olecule=s>. 1. Bile salts $. Fista*ine %. 8eukotriene '. All of these answers are correct (. Eone of these answers are correct ). C 1! ) Menkes kinky hair syndro*e is associated *ost co**only with which of the followin& hair a#nor*alities. 1. Pili torti $. /richorrhe<is nodosa %. /richorrhe<is in3a&inata '. Pili trian&ulati et canaliculati (. Eone of these options are correct ). A 1! A /he presence of natal teeth and pincer nails su&&ests which disease entity. 1. Con&enital syphillis $. /halido*ide e<posure in utero %. 1ncontinentia pi&*enti '. Pachyonychia con&enita (. Anhidrotic ectoder*al dysplasia ). 0 1! C Papillon"8efe3re and Fai*"Munk syndro*es ha3e which of the followin& sy*pto*s. 1. Hi&ht"3entricular cardio*yopathy $. Pseudoainhu* %. +sopha&eal cancer '. +ccrine syrin&o6#radeno*a (. Periodontitis with tooth loss ). + 1! D Painful crises and GwhorledG corneal opacities are seen with which of the followin& en;y*e a#nor*alities. 1. Fo*o&entisic acid o<idase $. Alpha"&alactosidase A %. 5lucocere#rosidase '. 1duronate sulfatase (. 5lucoronidase ). B 11 ! 8a*ellar ichthyosis is caused #y a defect in trans&luta*inase 1. 1t can present as a colloidion #a#y at #irth with su#seLuent lar&e thick plates of scale especially on -e<ures: ectropion and ecla#iu*. 1f two unaMected carrier parents ha3e a child: how likely is their child to ha3e this condition. 1. $(N $. A(N %. 9nly *ale oMsprin& are aMected '. 9nly fe*ale oMsprin& are aMected (. Eone of these answers are correct ). A 11 1 +pista<is in early childhood to teens followed #y *ultiple cutaneous and &astrointestinal telan&ectasias descri#es which of the followin& syndro*es. 1. Fereditary Fe*orrha&ic /elan&iectasia syndro*e $. MaMucci syndro*e %. CH+S/ syndro*e '. Ata<ia telan&ectasia (. 2a#ry disease ). A '% 11 $ A thirty"year"old wo*an presents with new pro&ressi3ely worsenin& headaches. Bpon e<a*: you notice *ultiple acral keratotic papules and papillo*as of the ton&ue. ,hat *ali&nancy does this patient need to #e screened for. 1. colon cancer $. o3arian cancer %. #reast cancer '. #asal cell carcino*a (. *elano*a ). C 11 % ,hat nail chan&e is seen in patients with Mal de Meleda Syndro*e. 1. 9nycholysis $. 8on&itudinal rid&in& %. Koilonychia '. leukonychia (. ptery&iu* ). C 11 ' ,hich of the followin& is caused #y a defect in keratins ' @ 1%. 1. ,hite spon&e ne3us $. +pider*olysis #ullosa si*ple< %. +pider*olysis #ullosa si*ple< with *yotonic dystrophy '. 7unctional +B with pyloric atresia (. Clouston[s syndro*e ). A 11 ( /he syndro*e characteri;ed #y &enerali;ed *ild hyperkeratosis: erythe*atous keratotic plaLues: pal*oplantar keratoder*a: non" pro&ressi3e sensorineural deafness: pro&ressi3e #ilateral keratitis with secondary #lindness is: 1. K10 syndro*e $. ?ohwinkel syndro*e %. +rythrokeratoder*a 3aria#ilis '. CF180 syndro*e (. Hefsu* syndro*e ). A 11 ) ,hich of the followin& is caused #y a defect in lysoso*al transport: 1. oculocutaneous al#inis* 1 $. oculocutaneous al#inis* 11 %. Pie#aldis* '. Chediak"hi&ashi (. Bloo*Js syndro*e ). 0 11 A /he *ost co**on ocular association with cutis *ar*orata telan&iectatica con&enital is: 1. Cataracts $. 5lauco*a %. Hetino#lasto*a '. Corneal opacity (. An&ioid streaks ). B 11 C Painful crises and GwhorledG corneal opacities are seen with which of the followin& en;y*e a#nor*alities. 1. Fo*o&entisic acid o<idase $. Alpha"&alactosidase A %. 5lucocere#rosidase '. 1duronate sulfatase (. 5lucoronidase ). B 11 D ,hich of the followin& *edications is a terato&en associated with a aplasia cutis con&enita. 1. Propranolol $. Methi*a;ole %. 8ithiu* '. Alcohol (. ,arfarin ). B 1$ ! ,hat cutaneous *anifestation is associated with fa*ilial cere#ral ca3erno*as. 1. ?errucous he*an&io*s $. 5lo*eruloid he*an&io*as %. hyperkeratotic cutaneous C '' capillary"3enous *alfor*ations=FCC?M> '. se&*ental facial he*an&io*as (. /ufted an&io*as ). 1$ 1 13ory"colored papules #etween the an&les of the scapulae are characteristic of which syndro*e: 1. Furler $. Scheie %. MorLuio '. Funter (. San6lippo ). 0 1$ $ ,hat is the characteristic radio&raphic 6ndin& in type 1 5aucher disease. 1. +nchondro*as $. 9steopoikilosis %. Melorheostosis '. +hrlen*eyer -ask defor*ity (. Supernu*erary 3erte#rae with e<tra ri#s ). 0 1$ % A patient with Klinefelter Syndro*e *ay #e e<pected to e<perience which of the followin&: 1. Hecurrent pul*onary infections $. Hecurrent le& ulcers %. Pul*onary 3al3e stenosis '. 5astroesopha&eal re-u< (. Scarrin& alopecia ). B 1$ ' 0er*ato6#rosis lenticularis disse*inata and osteopoikilosis are 6ndin&s seen with *utations of which of the followin& &enes. 1. 8+M0% $. 2i#rillin $ %. ABCC) '. 8ysyl hydro<ylase (. 8ysyl o<idase ). A 1$ ( /he E+M9 &ene is defecti3e in Bloch" Sul;#er&er syndro*e. ,hat other syndro*e has #een linked with defects in the E+M9 &ene. 1. Fypohidrotic ectoder*al dysplasia with i**une de6ciency $. Fypo*elanosis of 1to %. /u#erous sclerosis '. ,aarden#ur& syndro*e (. Pie#aldis* ). A 1$ ) ,hich of the followin& su#types of +hlers"0anlos Syndro*e =+0S> is associated with early de*ise. 1. /ype 1 =5ra3is> $. /ype ' =?ascular> %. /ype ( =O"linked> '. /ype A =Arthrochalasis *ultiple< con&enita> (. /ype 1! =2i#ronectin> ). B 1$ A A patient presents with se3eral li&ht #lue cyst"like lesions on the eyelid. /hey consult their list of pro#le*s and #rin& up plantar hyperkeratosis and dysplastic toenails. 9n oral e<a*: you note that they ha3e #oth upper and lower dentures. /he patient relates that after losin& their I#a#y teethI: only % teeth &rew in their place. ,hat syndro*e does this person *ost likely ha3e. 1. Schopf"Schul;"Passar&e $. 5ardner syndro*e %. Fypohidrotic ectoder*al dysplasia '. Cowden syndro*e (. Cronkhite"Canada ). A '( 1$ C 8a*ellar ichthyosis is caused #y *utations in which of the followin& &enes. 1. Steroid sulfatase $. 2atty aldehyde o<idoreductase %. Phytanoyl coen;y*e A hydro<ylase de6ciency '. Arylsulfatase + (. /rans&luta*inase ). + 1$ D ,hich of the followin& pairs of diseases are caused #y *utations in 0EA helicases. 1. Bloo* syndro*e and Hoth*und"/ho*pson syndro*e $. Oeroder*a pi&*entosu* and Cockayne syndro*e %. Muir"/orre syndro*e and Birt" Fo&&"0u#e syndro*e '. 0yskeratosis con&enita and Peut;"7e&hers syndro*e (. Cowden syndro*e and Bannayan"Hiley"Hu3alca#a syndro*e ). A 1% ! ,hat is the &ene defect in this condition: which is also called Mendes da Costa syndro*e. 1. Keratin 1 and 1! $. SP1EK( %. S8BHP"1 '. Conne<in $) (. Conne<in %1 ). + 1% 1 A patient with *ultiple deeply pi&*ented papules has a skin #iopsy which re3eals an epitheloid #lue ne3us. /he ne<t appropriate step is: 1. Heassure the patient and follow up as needed $. Schedule prophylactic e<cision of the lesion %. Be&in a *ali&nancy work"up '. Hefer to &enetics (. 9rder an echocardio&ra* ). + 1% $ ,hat condition is associated with this 6ndin& of in-a**atory keratotic facial papules which *ay result in scarrin& and atrophy. 1. Chloracne $. Syste*ic lupus erythe*atosus %. Keratosis pilaris '. HeiterGs syndro*e (. Blerythe*a ophryo&enes ). + 1% % ,hich of the followin& is caused #y a defect in cathepsin C. 1. Hu#enstein"/ay#i $. Bullous 1cthyosis of Sie*ens %. Muir"/orre '. Papillon"8efe3re (. Eail"Patella syndro*e ). 0 1% ' Mutations aMectin& the ?+52 receptor" % cause which of the followin& disorders. 1. Fereditary ly*phede*a =Eonne"Milroy disease> $. 8y*phede*a"distichiasis syndro*e %. 8y*phede*a and ptosis '. Eoonan syndro*e (. Fereditary he*orrha&ic telan&iectasias ). A 1% A ( *onth old &irl presents with failure 1. 8einer syndro*e C ') ( to thri3e. She has had life"lon& atopic der*atitis treated with topical hydrocortisone crea* and has persisent hypernatre*ia. 9n your e<a*: she has &enerali;ed erythe*a and scalin& of her #ody and trichorrhe<is in3a&inata on e<a*ination of hairs fro* her eye#rows. ,hich syndro*e is she *ost likely to ha3e. $. 9*enn Syndro*e %. Eetherton Syndro*e '. ,iskott"Aldrich Syndro*e (. Se3ere atopic der*atitis ). 1% ) ,hich of the followin& are cutaneous features of Marfan syndro*e. 1. 8oose skin and cru*pled ears $. 0er*ato6#rosis lenticularis and striae %. 2at herniation and cutaneous atrophy '. Scleroder*oid chan&es and dyspi&*entation (. Striae and elastosis perforans serpi&inosa ). + 1% A Eon"#ullous icthyosifor* erythroder*a is caused #y which of the followin& *utations: 1. /rans&luta*inase"1 &ene =/5M1> $. 1$H"lipo<y&enase &ene =A89O1$B> %. 8ipo<y&enase"% &ene =A89O+%> '. Both 1$H"lipo<y&enase &ene =A89O1$B> and lipo<y&enase"% &ene =A89O+%> are correct (. All of these answers are correct ). + 1% C 1n patients with diMuse con&enital he*an&io*atosis: the *ost co**on site for e<tracutaneous in3ol3e*ent is the : 1. 8i3er $. /hyroid %. 8un&s '. Colon (. Brain ). A 1% D ,hich syndro*e is characteri;ed #y hyperhidrosis: lack of pain sensation: hypersali3ation: and a#sent fun&ifor* papillae. 1. /urner Syndro*e $. Eoonan Syndro*e %. Hiley"0ay '. Hu#instein"/ay#i syndro*e (. Cornelia de lan&e Syndro*e ). C 1' ! +rythe*atous keratotic plaLues of K10 Syndro*e *ost co**only occur in which location. 1. 2ace $. Eeck %. Chest '. Back (. A#do*en ). A 1' 1 ,hich syndro*e is characteri;ed #y #road thu*#s: a lar&e #eaked nose: and capillary *alfor*ation. 1. Klinefelter $. Proteus syndro*e %. Bloo* syndro*e '. Hu#instein"/ay#i (. +hlers"0anlos syndro*e ). 0 1' A (! year *an presents with 1. Copper le3els C 'A $ &enerali;ed *etallic"&rey hyperpi&*entation. Fis past *edical history includes dia#etes: hepato*e&aly and arryth*ias. 8a#oratory tests should include: $. 8ead le3els %. 1ron le3els '. Arsenic le3els (. Cyanide le3els ). 1' % Patients with he*ochro*atosis are at increased risk for which of the followin&. 1. ?i#rio 3ulni6cus infections $. Sersenia infections %. Polyarthritis '. 5enerali;ed *etallic"&rey hyperpi&*entation (. All of these options are correct ). + 1' ' A child presentin& with the scalp 6ndin&s shown and a ri&ht ar* hypoplasia would #e dia&nosed with which of the followin& syndro*es. 1. Ada*s"9li3er syndro*e $. BartGs syndro*e %. Pro&eria '. 0unni&an syndro*e (. Eone of these options are correct ). A 1' ( 2a*ilial *acular and lichen a*yloidosis is a feature of which of the followin& conditions. 1. Sipple syndro*e $. Peut;"7e&hers syndro*e %. Marfan syndro*e '. 0yskeratosis con&enita (. Birt"Fo&&"0u#e syndro*e ). A 1' ) IMousyI odor of urine is characteristic of which of the followin& syndro*es. 1. Phenylketonuria $. Funter disease %. Alkaptonuria '. Maple Syrup Brine disease (. Furler disease ). A 1' A ,hich of the followin& la#oratory test *i&ht pro3e useful in the dia&nosis of 2a#ry disease: 1. Co*plete #lood count with diMerential $. 2astin& lipids %. Brinary sedi*ent e<a* with polari;in& li&ht *icroscopy '. Stool &uaiac (. Bleedin& ti*e ). C 1' C ,hat is the *ode of trans*ission for la*ellar ichthyosis. 1. Autoso*al do*inant $. Autoso*al recessi3e %. O"linked do*inant '. O"linked recessi3e (. Sporadic ). B 1' D ,hich of the followin& is E9/ a co*plication of Kasa#ach"Merritt Syndro*e: 1. /hro*#ocytopenia $. CF2 %. Ata<ia '. 0isse*inated intra3ascular coa&ulation (. 5astrointestinal #leedin& ). C 1( ! A se3en *onth old infant dia&nosed with ec;e*a on her face returns for a diaper"rash follow"up. A one"*onth 1. Stool sa*ples $. 2ontanelle e<a*ination %. +<a*ination of pal*s and soles C 'C trial of topical antifun&als has failed to i*pro3e the infantJs syste*s. /he part of the physical e<a* that *i&ht pro3e *ost useful include: '. Palpation of a#do*en (. Fearin& test ). 1( 1 +pider*al ne3us syndro*es inheritance pattern is: 1. Sporadic $. O"linked recessi3e %. O"linked do*inant '. Autoso*al recessi3e (. Autoso*al do*inant ). A 1( $ ,hich disease is found *ore co**only in *others of patients with chronic &ranulo*atous disease. 1. Sarcoidosis $. +rythe*a nodosu* %. Chur&"Straus disease '. ,e&enerGs disease (. 0iscoid lupus erythe*atous ). + 1( % Pseudo<antho*a elasticu* is caused #y *utations in which of the followin& &enes. 1. 2i#rillin 1 $. +lastin &ene %. 8ysyl o<idase '. ABCC) &ene (. Colla&en ( ). 0 1( ' 2indin&s of eyelid papules =strin& of pearls> and a hoarse cry in infants is characteristic of which of the followin& syndro*es. 1. 8ipoid proteinosis $. A*yloidosis %. Pseudo<antho*a elasticu* '. 0isse*inated <antho*as (. Eone of these answers are correct ). A 1( ( Mutations in calciu* transporters cause which pair of diseases. 1. +rythrokeratoder*ia 3aria#ilis and pro&ressi3e sy**etric erythrokeratoder*ia $. 8a*ellar ichthyosis and non#ullous con&enital ichthyosifor* erythroder*a %. Hefsu* syndro*e and S4o&ren" 8arsson syndro*e '. Chondrodysplasia punctata and CF180 syndro*e (. 0arierJs disease and Failey" Failey disease ). + 1( ) ,hich of the followin& is not classically associated with pheochro*octyo*a. 1. Multiple +ndocrine Eeoplasia /ype 11A $. Multiple +ndocrine Eeoplasia /ype 11B %. ?on"Fippel"8indau Syndro*e '. Co## Syndro*e (. Eeuro6#ro*atosis ). 0 1( A Patients with <"linked icthyosis are *ore prone to &ettin& which two *ali&nancies. 1. Pancreatic and acute ly*phocytic leuke*ia =A88> $. Pancreatic and acute *yelo&enous leuke*ia =AM8> %. /esticular and AM8 0 'D '. /esticular and A88 (. Henal cell and A88 ). 1( C Fypere<tesi#le skin: &apin& wounds: ci&arette"paper scars: *olluscoid pseudotu*ors: and calci6ed su#cutaneous nodules are characteristic of which syndro*e. 1. Marfan syndro*e $. Con&enital contractural arachnodactyly %. +hlers"0anlos Syndro*e '. Cutis la<a (. Pseudo<antho*a elasticu* ). C 1( D ,hich 6ndin& would you e<pect in a child with dyskeratosis con&enita. 1. 1ncreased risk for pancreatic cancers $. 9ral leukoplakia %. Firsutis* '. Eor*al he*atolo&ic status (. Eor*al nails ). B 1) ! ,hat is this syndro*e which is histolo&ically characteri;ed #y widely dispersed &ranular *aterial a*idst nor*al 6#ers. 1. +hlers 0anlos Syndro*e $. Pseudo<antho*a +lasticu* %. Buschke"9llendorf Syndro*e '. 2ocal 0er*al Fypoplasia (. 8ipoid Proteinosis ). B 1) 1 Beare"Ste3enson cutis &yrata syndro*e is linked with *utations in: 1. 2i#ro#last &rowth factor receptor $ $. BSC8$ %. 8MEA '. A/PAA (. Eone of these answers are correct ). A 1) $ 1n a patient suspected of ha3in& *ultiple endocrine neoplasia type 11#: which la# test would #e appropriate. 1. Calcitonin $. 5luca&on %. Parathyroid hor*one '. Calciu* (. Cortisol ). A 1) % Pseudo<antho*a elasticu* =PO+> can #e trans*itted in an autoso*al do*inant: recessi3e or sporadic *anner. ,hich of the followin& &enes is *utated in PO+. 1. ABCC) $. MAE1 %. Colla&en 111 '. Colla&en ? (. Colla&en 1 ). A 1) ' A patient with this autoso*al recessi3e disorder caused #y a defect in helicase is an increased risk for which *ali&nancy. 1. Acute leuke*ia $. Henal cell carcino*a %. Medullary thyroid carcino*a '. SLua*ous cell carcino*a of the lun& (. Prostate carcino*a ). A 1) ( ,hat 6ndin& is seen on #rain i*a&in& of patients with Papillon"8efe3re Syndro*e. 1. /ra* track calci6cations $. Calci6cation of the fal< cere#ri %. Calci6cation of the hippoca*pus '. Calci6cation of the dura 0 (! (. A&enesis of the corpus callosu* ). 1) ) ,hich of the followin& syndro*es is characteri;ed #y follicular atrophoder*a: hypohidrosis: hypotrichosis and *ultiple #asal cell carcino*as. 1. Ba;e< syndro*e $. Ho*#o syndro*e %. Has*usen syndro*e '. 5orlin syndro*e (. 1ncontinentia Pi&*enti ). A 1) A An infant presents with *ultiple con&enital he*an&o*as in an &enerali;ed distri#ution. ,hat is the *ost serious associated condition. 1. Con&esti3e Feart 2ailure $. 9#structi3e 4aundice %. Portal hypertension '. All of the answers are correct (. Eone of the answers are correct ). A 1) C A trian&ular"shaped lunula is a characteristic 6ndin& in which disease. 1. 0arierGs disease $. 0yskeratosis con&enita %. +pider*al ne3us syndro*e '. 1ncontinentia pi&*enti (. Eail"patella syndro*e ). + 1) D 1n ata<ia telan&ectasia: the A/M &ene is *utated. /he product of the A/M &ene is an en;y*e which: 1. Hesponds to 0EA da*a&e #y phosphorylatin& key 0EA repair su#strates $. Binds transfor*in& &rowth factor #eta protein %. 1s the ?+52 receptor % '. Participates in E2"kB acti3ation (. 1s an inhi#itor of 51 cyclinCdk co*ple<es ). A 1A ! A 1) year"old &irl presents with a fa*ily history of 5ardner syndro*e. Fer *other is 3ery concerned that her dau&hter *ay ha3e the syndro*e as it runs in her fa*ily and she has *any skin co*plaints. 5ardner syndro*e has #een linked to defects in #eta" catenin *ediated transcription. ,hich of the followin& &enes dysfunction is responsi#le. 1. APC $. S/K11 %. CS80 '. P/CF (. ABCC) ). A 1A 1 Fypoplasia of the #reast can #e seen in which disease. 1. Anhidrotic ectoder*al dysplasia $. MaMucci syndro*e %. Con&enital syphilis '. Marfan syndro*e (. 9steo&enesis i*perfecta ). A 1A $ ,hich of the followin& syndro*es are linked to a P/+E &ene *utation. 1. Banayan"Konana $. Proteus %. Cowden '. All of these options are correct (. 8her*itte"0uclos disease ). 0 1A % /he *ost co**on neoplas* seen in MaMucci Syndro*e is: 1. +nchondro*as $. An&iosarco*as A (1 %. 9steosarco*as '. 8y*phan&iosarco*as (. chondrosarco*a ). 1A ' ,hat is the *ost co**on &enetic defect associated with this syndro*e. 1. Eeuro6#ro*in $. Merlin %. /u#erin '. Fa*artin (. 2olliculin ). A 1A ( A patient with thyroid carcino*a and co##lestone"like chan&es of the oral *ucosa will also likely ha3e: 1. /richoepithelio*as $. 2i#rofolliculo*as %. /richole**o*as '. Cylindro*as (. Syrin&o*as ). C 1A ) ,hat is the underlyin& &ene defect for this trans&rediens for* of pal*oplanter keratoder*a 1. S8BHP"1 $. /9C &ene %. Plako&lo#in '. Keratin type 1 (. Keratin type D ). A 1A A ,hich of the followin& is E9/ part of the Carney co*ple<. 1. Pe& or conical teeth $. Cardiac: cutaneous or *a**ary *y<o*as %. Pi&*ented skin lesions '. +ndocrine a#nor*alities (. Pri*ary pi&*ented nodular adrenocortical disease ). A 1A C 5iant lysoso*al &ranules are seen in which disease. 1. Chediak"Fi&ashi syndro*e $. 5riscelli syndro*e %. Pie#aldis* '. 1ncontinentia pi&*enti (. Carney co*ple< ). A 1A D 9steopathia striata is seen in which of the followin& disorders. 1. 5aucherJs disease $. Al#ri&htJs syndro*e %. Aplasia cutis con&enita '. 2ocal der*al hypoplasia (. Scleroder*a ). 0 1C ! Medullo#lasto*a is seen in which syndro*e. 1. 5ardnerJs syndro*e $. Multiple endocrine neoplasia $# %. Muir"/orre syndro*e '. Basal cell ne3us syndro*e (. Eeuro6#ro*atosis /ype 1 ). 0 1C 1 /he *ost co**on cardio3ascular defect in patients with Eoonan syndro*e is: 1. Atrial septal defect $. ?entricular septal defect %. +nlar&ed aorta '. Pul*onic 3al3e stenosis (. Aortic stenosis ). 0 1C 0er*ato6#rosis lenticularis 1. +hlers"0anlos syndro*e + ($ $ disse*inata is seen in which of the followin& conditions. $. Marfan syndro*e %. Pseudo<antho*a elasticu* '. 2ocal der*al hypoplasia (. Buschke"9llendorf syndro*e ). 1C % A %$ year"old wo*an is ( weeks pre&nant and is dia&nosed with hyperthyroidis*. Fer doctor &i3es her a prescription for on *ethi*a;ole 1! *& P9 tid. ,hich of the followin& fetal a#nor*alities could #e caused #y this e<posure. 1. Aplasia cutis con&enita $. Menin&ocele %. +ncephalocele '. Spina #i6da (. 0er*oid cyst ). A 1C ' A patient has recurrent infections and is found to ha3e a disorder of pha&ocytic cells fro* an ina#ility of pha&ocytes to under&o the respiratory #urst needed to kill certain types of #acteria and fun&i. Carriers of this disease are also at risk for which condition. 1. Breast cancer $. ?itili&o %. 0iscoid lupus '. /hyroid disease (. Cataracts ). C 1C ( ,hich of the followin& is correct a#out ne3oid #asal cell carcino*a syndro*e. 1. known as 5ardner syndro*e $. autoso*al recessi3e %. *ultiple keratoacantho*as '. calici6cation of the fal< (. odonto&enic steato*as ). 0 1C ) Sou recei3e a hospital consult fro* the &astroenterolo&y ser3ice for a '$"year old wo*an with esopha&eal cancer. /hey would like your opinion on the yellow: thickened areas on her pal*s and soles in areas of pressure. ,hen you speak with her: she says that her father had si*ilar pro#le*s and it runs in her fa*ily. ,hich of the followin& is defecti3e. 1. /9C &ene $. 0es*oplakin %. Plako&lo#in '. Conne<in %!.% (. Conne<in %1 ). A 1C A A '"year old #oy presents with &enerali;ed white scale. /he *other reports that her son was #orn with a ti&ht *e*#rane en3elopin& his #ody. Peripheral #lood s*ear is within nor*al li*its. ,hat is the *ost likely dia&nosis. 1. Con&enital ichthyosifor* erythroder*a $. Eeutral lipid stora&e disease %. 8a*ellar ichthyosis '. Eetherton syndro*e (. 1chthyosis 3ul&aris ). A 1C C Patients with 4unctional epider*olysis #ullosa ha3e #een found to ha3e *utations in: 1. 8a*inin ( $. Bullous pe*phi&oid anti&en $ %. Colla&en 1A '. BP1C! (. All of the answers are correct ). + 1C D Keratins 1 and 1! are i*portant inter*ediate 6la*ents in supra#asal keratinocytes. 5enetic *utation of these keratins can lead to which of the followin& disorders. 1. Eetherton syndro*e $. S4o&ren"8arsson syndro*e %. Pachyonychia con&enita type 1 '. +pider*olytic hyperkeratosis Bullous ichthyosifor* erythroder*a (. ,hite spon&e ne3us 0 (% ). 1D ! ,hich syndro*e is due to a defecti3e secreted *a**ilian 8y)uPAH"related protein"1. 1. EethertonGs syndro*e $. Hefsu*Gs syndro*e %. S4o&ren"8arsson syndro*e '. Mal de Meleda syndro*e (. Fai*"Munk syndro*e ). 0 1D 1 /he co*#ination of painful pal*oplantar keratoder*a and pseudoherpetic keratitis is characteristic of which of the followin& syndro*es. 1. Ea<os syndro*e $. ?ohwinkel syndro*e %. Hichner"Fanhart syndro*e '. Fowel"+3ans syndro*e (. Schopf"Schul;"Passar&e syndro*e ). C 1D $ A de6ciency in sialophorin: a surface &lycoprotein: is thou&ht to play a role in which &enetic disorder. 1. ,iskott"aldrich syndro*e $. Chediak"hi&ashi syndro*e %. ,erner syndro*e '. Bloo* Syndro*e (. Cornelia de 8an&e syndro*e ). A 1D % ,hich of the followin& is defecti3e in +hlers"0anlos syndro*e =+0S> with con&enital adrenal hyperplasia. 1. /enascin"O $. 8ysyl o<idase %. 8ysyl hydro<ylase '. Eone of these answers are correct (. All of these answers are correct ). A 1D ' ,hat protein is de6cient in the condition shown. 1. Calciu* A/PJase 11C1 $. Calciu* A/PJase 11A$ %. P+O"A '. SP1EK( (. 0es*o&lein % ). A 1D ( JCoast of MaineJ cafJ au lait *acules are characteristic of which condition 1. Fypo*elanosis of 1to $. Carney co*ple< %. McCune"Al#ri&ht syndro*e '. 5aucherJs syndro*e (. /u#erous sclerosis ). C 1D ) 0yshcro*atosis sy**etrica hereditaria =0SF> is a disorder characteri;ed #y asy*pto*atic hypo" and hyperpi&*ented *acules on the face and dorsal e<tre*ities. Mutations in which of the followin& &enes is associated with this condition. 1. 0KC $. P/+E %. A0AH1 '. +HCC' (. S/K11 ). C 1D A Bilateral o3arian cystadeno*as and parotid *ono*orphic adeno*as are seen in which of the followin& conditions. 1. Cowden syndro*e $. Proteus syndro*e %. ?on"Fippel"8indau syndro*e '. Eoonan syndro*e (. Beckwith",ieder*an syndro*e ). B 1D C ,hich of the followin& is E9/ a characteristic skin 6ndin& in patients with 0own Syndro*e. 1. Syrin&o*as $. +lastosis perforans serpi&inosa %. Sin&le pal*ar crease + (' '. 2lat nipples (. S*all ton&ue ). 1D D /his for* of +B si*ple< has a defect in actin asse*#ly: not keratin for*ation. 1. 0owlin& Meara $. ,e#er"Cockayne %. Koe#ner '. Kindler (. 9&na ). 0 $! ! Mutations in nuclear la*ins are associated with which clinical feature. 1. 8ipo*atosis $. Sarco*as %. 8y*phede*a '. Aplasia cutis con&enita (. 8ipodystrophy ). + $! 1 Brticaria pi&*entosa is linked to a defect in the c"kit protoonco&ene. ,hat autoso*al do*inant skin disease also has #een linked to this defect. 1. Pie#aldis* $. Fypo*elanosis of 1to %. ,aarden#ur& syndro*e '. Fer*ansky"Pudlak syndro*e (. 1ncontinentia pi&*enti ). A $! $ A ' year"old #oy presents with $ soft: dark"#lue: co*pressi#le nodules on his e<tre*ities. Fis *other has noted that these lesions ha3e increased sweatin& and that they were present at #irth. Eo one else in the fa*ily has had si*ilar skin lesions. ,hat step is indicated 6rst to help deter*ine the dia&nosis. 1. Stool &uiac $. MH1 of the a#do*en %. CBC '. 1**ediate referral to a &astroenterolo&ist (. Biopsy of a skin lesion ). A $! % A BSC8$ &ene *utation with the cutaneous 6ndin&s of &enerali;ed lipodystrophy: hyperlipe*ia: hepato*e&aly: acanthosis ni&ricans: ele3ated #asal *eta#olic rate and non" ketotic insulin resistant dia#etes *ellitus are characteristic of which of the followin& syndro*es. 1. Berardinelli"Seip con&enital lipodystrophy $. 2a*ilial partial lipodystrophy %. B4ornstad syndro*e '. All of the answers are correct (. Eone of the answers are correct ). A $! ' /he treat*ent for acroder*atitis enteropathica is: 1. Kinc supple*entation $. 1ron supple*entation %. ?ita*in B1 supple*entation '. ?ita*in B1$ supple*entation (. Phle#oto*y ). A $! ( +ctopia lentis =downward displace*ent of the lens> is characteristic of: 1. Marfan syndro*e $. Fo*ocystinuria %. Phenylketonuria '. Multiple Car#o<ylase de6ciency (. +hlers"0anlos syndro*e ). B $! ) A Puerto Hican wo*an is seen in clinic for a pruritic rash on her trunk. A punch #iopsy is perfor*ed. /he #iopsy site continues to #leed: with he*ato*a for*ation. /he #leedin& is e3entually controlled. 9n further 1. Fer platelets lack dense #odies: causin& e<cess #leedin& $. Fer intrinsic factor is de6cient %. Fer 2actor ?111 le3els are low '. She is con&entially de6cient in platelets A (( e<a*: her skin and hair are li&ht #rown. She has a history of &ranulo*atous colitis. ,hat it the *ost likely reason she had e<cess #leedin& with a si*ple procedure. (. Eone of the answers are correct ). $! A ,hich of the followin& syndro*es de*onstrate atrophoder*a 3er*iculatu*. 1. /u;un Syndro*e $. H9MB9 Syndro*e %. Eicolau"Balus Syndro*e '. Braun"2alco"Mar&hescu Syndro*e (. All of these answers are correct ). + $! C /he arylsulfatase C &ene is *utated in which disease. 1. O"linked ichthyosis $. Hefsu* syndro*e %. Fai*"Munk syndro*e '. Ea<os syndro*e (. 5riscelli syndro*e ). A $! D Mucosal *ali&nancy is a co*plication of: 1. 9ral hairy leukoplakia $. ,hite spon&e ne3us %. 0yskeratosis con&enital '. Chronic candidiasis (. 2ocal epithelial hyperplasia ). C $1 ! 2ocal 0er*al Fypoplasia =5olt; Syndro*e> can diMerentiated fro* 1ncontinentia Pi&*enti #y 1. /ype of inheritance $. Presence of 8inear lesions alon& the lines of Blaschko %. Presence of #listerin& lesions '. Fair and teeth a#nor*ality (. +ye and CES a#nor*ality ). C $1 1 ,hich &ene is defecti3e in ,iskott" Aldrich syndro*e. 1. ,AS $. CSBA %. CSBB '. EC21 (. EC2$ ). A $1 $ ,hich 3ascular disorder is characteri;ed #y facial 3ascular *alfor*ation and ipsilateral intracranial and retinal arterio3enous *alfor*ations=A?Ms>. 1. Stur&e",e#er syndo*e $. PFAC+S %. +ncephalotri&e*inal an&io*atosis '. Bonnet 0echaune Blanc syndro*e (. ?on 8ohui;enJs disease ). 0 $1 % ,hat condition is associated with a *utation in COCH'. 1. Ata<ia telan&iectasia $. 8eukocyte adhesion de6ciency %. Chronic &ranulo*atous disease '. SC10 (. ,F1M syndro*e ). + $1 ' ,hich of the followin& is caused #y a *utation in a &ene which codes for steroid sulfatase. 1. 2a#ry disease $. 8esch"nyhan disease %. O"linked ichthyosis '. 8a*ellar icthyosis C () (. Chediak"Fi&ashi ). $1 ( ,hich optha*olo&ic disease is associated with this disorder. 1. 5lauco*a $. +ctopia lentis %. Cataracts '. Posterior su#casular lentiular opacity (. Hetinitis pi&*entosa ). A $1 ) 1n which of the followin& 5enoder*atoses would one 6nd cutaneous hyperpi&*entation: #lue lunulae and Kayser"2leishcher rin&s: 1. MarfanGs 0isease $. Fe*ochro*atosis %. 5aucherGs 0isease '. ,ilsonGs 0isease (. 9steo&enesis 1*perfecta ). 0 $1 A ,hich of the followin& syndro*es is associated with *arkedly increased 1&+ le3els: cold a#scesses and a characteristic coarse facies. 1. ,iskott"Aldrich syndro*e $. Chronic &ranulo*atous disease %. 7o# syndro*e '. Se3ere co*#ined i**unode6ciency (. 8einerGs disease ). C $1 C 1chthyosis linearis circu*-e<a is one of the 6ndin&s seen in the syndro*e caused #y which of the followin& &enes. 1. SP1EK( $. S8BHP1 %. 57B$ '. 8MO1B (. CS80 ). A $1 D 1chthyosis hystri< is characteri;ed #y the followin& &ene defects. 1. Keratins 1 and D $. Keratins 1 and 1! %. Keratins ( and 1' '. Keratins ) and 1) (. Eone of these answers are correct ). B $$ ! ,hich of the followin& syndro*es is associated with cutis *ar*orata. 1. EethertonJs $. Papillon"8efe3re syndro*e %. Fe*ansky"Pudlak syndro*e '. MaMucci syndro*e (. Cornelia de 8an&e syndro*e ). + $$ 1 Spastic ditetraple&ia is associated with which of the followin& disorders. 1. S4o&ren"8arsson syndro*e $. O"linked ichthyosis %. 8a*ellar ichthyosis '. K10 syndro*e (. Hefsu* syndro*e ). A $$ $ Ea<os syndro*e is characteri;ed #y a ri&ht sided cardio*yopathy: wooly hair: and keratoder*a. /he epider*al structure defecti3e in Ea<os syndro*e is: 1. 0es*o&lein 1 $. 0es*o&lein % %. Plako&lo#in '. 0es*oplakin (. Keratin 11! ). C $$ % A 11 year"old fe*ale patient with hypoparathyroidis* is referred to your 1. +ctoder*al dysplasia $. 1nsulin dependent dia#etes 0 (A clinic secondary to chronic *ucocutanous candidiasis which is refractory to standard treat*ents. /he patient also has *ala#sorption and se3ere chronic diarrhea. Sou deter*ine that she has autoi**une polyendocrinopathy"candiasis" ectoder*al dystrophy syndro*e=AP+C+0>. Since only two of three *a4or criteria are needed to *ake this dia&nosis: this patient ha3in& de*onstrated hypoparathyroidis* and chronic *ucocutanous candidiasis is dia&nosed with AP+C+0. ,hat is the third *a4or criteria that would Lualify a patient to *eet the dia&nosis of AP+C+0. *ellitus %. Chronic autoi**une hepatitis '. AddisonGs disease (. Fypothyroidis* ). $$ ' ,hat is the *ost likely nail 6ndin&s in a patient who has this autoso*al do*inant disease with these keratotic papules and co##lestonin& of the oral *ucosa. 1. Koilonychia $. Hed and white lon&itudinal #ands %. Melanonychia '. Falf and half nails (. Pincer nails ). B $$ ( /he &ene defect in 5riscelli Syndro*e is: 1. Myosin ?a or Ha#$Aa $. 8SS/ or CFS1 %. P &ene '. /HP1 (. Eone of these answers are correct ). A $$ ) A patient with a white: spon&y o3er&rowth of the #uccal *ucosa that has passed in an autoso*al do*inant fashion is *ost likely related to a *utation in which of the followin&. 1. Keratin 11! $. Keratin )#1A %. Keratin '1% '. Keratin )a1) (. Eone of these options are correct ). C $$ A Sphenoid win& dysplasia is seen in: 1. /u#erous sclerosis $. /ay Syndro*e %. Mafucci syndro*e '. E2"1 (. E2"$ ). 0 $$ C Adenosine dea*inase de6ciency is associated with which of the followin& disorders. 1. ,iskott Aldrich syndro*e $. 5out %. 7o# syndro*e '. Oeroder*a pi&*entosu* (. Se3ere co*#ined i**unode6ciency ). + $$ D A child presents with sparse: short hair and sensorineural deafness. 9n *icroscopic e<a*ination of the hair: 1. B4ornstad syndro*e $. Menkes kinky hair syndro*e %. Ar&ininosuccinic aciduria A (C pili torti is noted. ,hich of the followin& syndro*es is the *ost likely dia&nosis. '. /richothiodystrophy (. Eone of the options are correct ). $% ! +yelid strin& of pearls are seen in which of the followin& conditions. 1. 2ocal der*al hypoplasia $. 8ipoid proteinosis %. Futchinson"5ilford syndro*e '. Beare"Ste3enson cutis &yrata syndro*e (. 5aucherJs disease ). B $% 1 Beckwith",ieder*an syndro*e is characteri;ed #y which of the followin& triads. 1. Fe*an&io#lasto*as: renal cysts and renal cell carcino*a $. +pista<is: telan&ictases: and &astrointestinal tract #leedin& %. +nlar&ed li*#: port wine stain: and deep 3enous thro*#osis '. 9*phalocele: 3enous *alfor*ations: and ata<ia (. +<o*phalos: *acro&lossia: and &i&antis* ). + $% $ A child has ichthyosis and is found to ha3e leukocytes with 3acuoles 6lled with lipids on peripheral s*ear. She *ost likely has: 1. Chanarin"0orf*an syndro*e $. 1chthyosis hystri< %. Ea<os disease '. 1chthyosis #ullosa of Sie*ens (. 1chthyosis 3ul&aris ). A $% % An&ioid streaks on retinal e<a* are characteristic of which of the followin& syndro*es. 1. Pseudo<antho*a elasticu* $. Choroid *alfor*ations %. +yelid papillo*as '. 8ester iris (. Salt @ pepper retinitis pi&*entosa ). A $% ' ,hich of the followin& syndro*es is associated with trichole**o*as. 1. Birt"Fo&&"0u#e $. Brooke"Spie&ler %. Bannayan"Hiley"Hu3acal#a '. Base< (. Has*ussenGs ). C $% ( A child presents with the hair 6ndin& seen in the i*a&e in addition to #rittle nails: keratosis pilaris: a#nor*al teeth and cataracts. ,hich of the followin& a#nor*alities is the *ost likely *utated. 1. Keratin 11! $. Keratin hF#1hF#) %. Keratin )1) '. Keratin )1A (. Keratin $e ). B $% ) /he &ene defect in 8+9PAH0 syndro*e is: 1. P/PE11 $. PHKAH1A %. K1P$ '. A/M (. Eeuro6#ro*in ). A $% A He&ardin& the inheritance of +hlers" 0anlos syndro*e: which su#type is inherited in an O"linked recessi3e 1. /he type with the lysyl o<idase de6ciency $. /he type with the lysyl A (D *anner. hydro<ylase de6ciency %. /he type with a colla&en ( defect '. /he type with a colla&en % defect (. /he type with a defect in procolla&en a*inopeptidase ). $% C Patients with ho*ocystinuria are de6cient in: 1. Alpha &alactosidase A $. /rans&luta*inase %. 8ysyl o<idase '. Cystathionine synthetase (. Steroid sulfatase ). 0 $% D A patient presents with #ilateral retinal he*an&io#lasto*as and a capillary *alfor*ation on her neck. ,hich &ene *utation is *ost likely. 1. ?F8 $. +ndo&lin %. P/FP/FrP type 1 receptor '. ?+52 receptor"% (. M2F1 ). A $' ! A 1C yo *an presents for e3aluation of foot lesions. /here are thick hyperkeratotic plaLues sy**etrically on only the wei&ht #earin& plantar surfaces. ,hat test=s> should this patient #e referred for. 1. Fead C/ $. /horacic C/ %. Fepatic ultrasound '. +ndoscopy (. Knee 6l*s ). 0 $' 1 ,hich disease can clinically *i*ic pella&ra #ut is inherited in an autoso*al recessi3e fashion and is due to a defect in the transport of neutral a*ino acids. 1. ,ilsons $. Fe*ochro*atosis %. Fartnup 0isease '. 2a#ry (. 5aucherJs ). C $' $ Meleda is an island oM the coast of Croatia. 1ts inha#itants ha3e an increased freLuency of a *alodorous trans&radiens pal*oplantar keratoder*a in a stockin&"&lo3e distri#ution. /his condition is autoso*al recessi3e with a defect in: 1. S8BHP1 &ene $. Keratin 1D %. /9C &ene '. 8oricrin (. Cathepsin C ). A $' % Co**a"shaped corneal opacities are characteristic of which type of ichthyosis. 1. 1chthyosis 3ul&aris $. O"linked ichthyosis %. 8a*ellar ichthyosis '. Eon#ullous con&enital ichthyosifor* erythroder*a (. Hefsu* syndro*e ). B $' ' Acropi&*entation of 0ohi is characteri;ed #y: 1. Heticulated pi&*entation of the a<illae: neck: and &roin $. 8inear pal*ar pits and pi&*ented *acules on 3olar and dorsal hands and feet %. Pi&*ented and depi&*ented *acules on the distal dorsal e<tre*ities and face '. Fyperpi&*ented *acules on C )! the lips and oral *ucosa (. 2laccid: super6cial pustules that #urst and lea3e pi&*ented *acules ). $' ( Patients with Fer*ansky"Pudlak syndro*e *ay e<perience which of the followin& syste*ic co*plications as a result of their disease. 1. Arterio3enous *alfor*ations $. Pul*o*ary 6#rosis %. 5astroesopha&eal re-u< disease '. Aortic stenosis (. Hectal a#scesses ). B $' ) A patient with *ultiple lenti&ines and #lue ne3i *ay also ha3e: 1. 0eafness $. Pul*onary 3al3e stenosis %. Atrial *y<o*a '. Mental retardation (. 51 *ali&nancy ). C $' A 5er*line K1881E *ethylation is associated with which of the followin& syndro*es. 1. Bannayan"Hiley"Hu3alca#a Syndro*e $. Proteus Syndro*e %. Cowden Syndro*e '. Birt"Fo&&"0u#e Syndro*e (. 5alli"5alli 0isease ). C $' C Pri*ary pi&*ented nodular adrenocortical disease and psa**o*atous *elanotic schwanno*as are characteristic of which of the followin& syndro*es. 1. Fypo*elanosis of 1to $. Carney co*ple< %. McCune"Al#ri&ht syndro*e '. 5aucherJs syndro*e (. /u#erous sclerosis ). B $' D 0e6ciency of 6la&&rin is the stron&est known predisposin& &enetic factor for the de3elop*ent of atopic der*atitis. ,hat is the *ode of inheritance of *utations in 6la&&rin. 1. Autoso*al do*inant $. Autoso*al se*ido*inant %. Autoso*al recessi3e '. O"linked do*inant (. O"linked recessi3e ). B $( ! Ee3oid #asal carcino*a syndro*e =5orlin syndro*e> is autoso*al do*inant trans*itted *utation of the patched &ene. Sy*pto*s include innu*#era#le #asal cell carcino*as: painful odonto&enic 4aw keratocysts: pal*oplantar pits: frontal #ossin&: #i6d ri#s and what other #ony a#nor*ality. 1. Polyostotic 6#rous dysplasia $. Stippled epiphyses %. Calci6cation of fal< cere#ri '. 9steopoikilosis (. Sphenoid win& dysplasia ). C $( 1 A patient Buschke"9llendorM syndro*e has osteopoikilosis and which cutaneous 6ndin&. 1. ,a<y papules alon& the eyelids $. CafJ au lait *acules %. Port wine stain '. 7u3enile elasto*a (. +pider*al ne3i ). 0 $( $ /he dia&nostic test for chronic &ranulo*atous disease is: 1. 0i*ethyl&lyo<i*e test $. Eitro#lue tetra;oliu* reduction assay %. Fista*ine skin test B )1 '. Bone *arrow #iopsy (. Potassiu* hydro<ide ). $( % +pider*olysis #ullosa si*ple< is caused #y #listerin& in which structure. 1. 5ranular layer keratinocyte $. Spinous layer keratinocyte %. Basal layer keratinocyte '. 8a*ina densa (. Su#la*ina densa ). C $( ' A child with phenylketonuria likely presents with which cutaneous pro#le*s. 1. Blue"&ray &enerali;ed hyperpi&*entation $. Alopecia uni3ersalis %. 5enerali;ed hypopi&*entation '. 5enerali;ed hyperpi&*entation (. 8e& ulcers ). C $( ( ,hich of the followin& 6ndin&s is characteristic of a *utation in la*in A. 1. 8ipoatrophic scleroder*oid skin $. Alopecia %. Cranio*e&aly with s*all face '. Se3ere pre*ature atherosclerosis with early death (. All of the answers are correct ). + $( ) A patient with port wine stain on a lower e<tre*ity: he*ihypertrophy of the li*# and ly*phatic and deep 3enouse insuTciency of the aMected li*# would #e considered to ha3e Klippel"/renaunay",e#er syndro*e. ,hat additional feature would need to #e present to de6ne the patient as ha3in& Parkes",e#er syndro*e. 1. Arterio3enous 6stulas $. Multiple cafe"au"lait *acules %. Macro&lossia '. Cutis *ar*orata (. 0istichiasis ). A $( A ,hat is the &enetic defect of this autoso*al do*inant disorder. 1. P/+E $. P/PE11 %. Calciu* A/Pase $A$ '. Calciu* A/Pase $C1 (. SP1EK( ). 0 $( C A patient with *ultiple facial trichile**o*as is at risk of which of the followin& cancers. 1. Basal cell carcino*a $. Breast carcino*a %. Acute leuke*ia '. Cylindro*a (. 9ral sLua*ous cell carcino*a ). B $( D Birt"Fo&&"0u#e syndro*e is *ost stron&ly associated with which of the followin& *ali&nancies. 1. Basal cell carcino*a $. Medullo#lasto*a %. Henal cell carcino*a '. /richoepithelial carcino*a (. +ccrine syrin&o6#roadeno*a ). C $) ! Adenosine dea*inase de6ciency is seen in which i**unode6cient disease. 1. ,iskott"Aldrich syndro*e $. Chronic &ranulo*atous disease %. 7o# syndro*e '. Se3ere co*#ined i**unode6ciency syndro*e 0 )$ (. 8eineriGs disease ). $) 1 /yrosinase positi3e al#inis* =oculocutaneous al#inis* type $> is caused #y a *utation in which of the followin&: 1. /yrosinase $. P &ene %. /yrosinase related protein 1 '. C"kit (. E+M9 ). B $) $ 2indin&s of *ilia: cylindro*as and the condition shown in the patholo&y i*a&e are characteristic of which of the followin& syndro*es. 1. 5orlinGs syndro*e $. 2a*ilial cylindro*atosis %. Brook"Spie&ler syndro*e '. Has*usen syndro*e (. Ho*#o syndro*e ). C $) % +nchondro*as and chondrosarco*as are *ost stron&ly associated with which of the followin& syndro*es. 1. Proteus syndro*e $. 9sler",e#er"Hendu syndro*e %. MaMucci syndro*e '. Eonne"Milroy disease (. Blue ru##er #le# ne3us syndro*e ). C $) ' ,hich eye 6ndin&s would #e e<pected in an indi3idual with this disorder associated with atherosclerosis. 1. 0endritic corneal ulcerations $. Salt and pepper retinitis pi&*entosa %. +ctopia lentis '. An&oid streaks (. Keratoconus ). 0 $) ( /he coast of Maine cafe au lait *acule is a co**on 6ndin& in which of the followin& syndro*es. 1. /u#erous Sclerosis $. Eeuro6#ro*atosis type 1 %. McCune"Al#ri&ht syndro*e '. ,atson syndro*e (. Hussell"Sil3er syndro*e ). C $) ) 1chthyosifor* erythroder*a in lines of Blaschko: follicular atrophoder*a: and stippled epiphyses are characteristic of which of the followin& disorders. 1. Hefsu* syndro*e $. Chondrodysplasia punctata %. +rythrokeratoder*ia 3aria#ilis '. Eetherton syndro*e (. S4o&ren"8arsson syndro*e ). B $) A ,hich of the followin& *etals is de6cient in the seru* of patients with Menkes kinky hair syndro*e. 1. Copper $. 1ron %. Seleniu* '. Kinc (. Biotin ). A $) C A patient with *yotonic dystrophy and *ultiple skin lesions *ost likely has acti3atin& *utations in which of the followin&. 1. Beta"catenin $. 0es*oplakin %. Plako&lo#in '. 0es*o&lein (. Alpha )"#eta ' inte&rin ). A $) D ,hich of the followin& #ony defect is found in CF180 syndro*e. 1. Polyostotic 6#rous dysplasia $. Stippled epiphyses %. Calci6cation of fal< cere#ri B )% '. 9steopoikilosis (. Sphenoid win& dysplasia ). $A ! ,hat is the *ost likely &ene *utation in this indi3idual who has *i&ratory patches and 6<ed plaLues as depicted in this picture. 1. Conne<in $) $. Conne<in %!.% and %1 %. Calciu* A/Pase $C1 '. 8oricrin (. Keratins 1 and 1! ). B $A 1 A 1$ year"old #oy with pits on his pal*s and lateral 6n&ers *ay ha3e: 1. Arsenic e<posure $. A hereditary keratoder*a %. A coryne#acteria infection '. An inherited cancer syndro*e (. Secondary syphilis ). 0 $A $ ,hich of the followin& syndro*es is O" linked do*inant. 1. Anhidrotic ectoder*al dysplasia $. 0yskeratosis con&enita %. O"linked icthyosis '. 9rofaciodi&ital syndro*e 1 (. Menkes kinky hair syndro*e ). 0 $A % Conne<in %! =57B) &ene> is defecti3e in which of the followin& syndro*es. 1. K10 syndro*e $. ?ohwinkel syndro*e %. ?ohwinkel syndro*e 3ariant '. Clouston syndro*e (. +rythrokeratoder*a 3aria#ilis ). 0 $A ' Patients with Hussell"Sil3er syndro*e e<hi#it: 1. Clinodactyly of 6fth 6n&er $. Pe& teeth %. 9steopathia striata '. Broad thu*#s (. Shortened 'th and (th *etacarpals ). A $A ( ,hich of the followin& diseases is seen only in fe*ales. 1. 5riscelli syndro*e $. Pie#aldis* %. Fypo*elanosis of 1to '. 1ncontinentia pi&*enti (. Carney co*ple< ). 0 $A ) /he characteristic dental 6ndin&s in patients with tu#erous sclerosis are: 1. Pe& teeth $. Anodontia %. +na*el pits '. 9donto&enic cysts (. Hetention of pri*ary teeth ). C $A A Sou are consulted on a patient with possi#le Eethertons Syndro*e. ,hich location of the #ody would *ost likely ha3e hairs de*onstratin& trichorrhe<is in3a&inata. 1. Scalp $. +ye#row %. +yelash '. All of these answers are correct (. Eone of these answers are correct ). B $A C /o help dia&nose trichothiodystrophy: which of the followin& le3els are 1. Ar&inine $. Fistidine + )' decreased in hairs of aMected indi3iduals. %. Phenylalanine '. 5lycine (. Cysteine ). $A D ,hich one of the followin& is the *ost co**on onco&enic 3irus in patients with epider*odysplasia 3errucifor*is. 1. FP?"( $. FP?"C %. FP?"1% '. FP?"1) (. FP?"%% ). A $C ! A patient with hypohydrosis and hyperpyre<ia: anodontia: and sparse hair has which syndro*e: 1. 0yskeratosis con&enita $. Pachyonychia con&enita %. Anhidrotic ectoder*al dysplasia '. Fidrotic ectoder*al dysplasia (. Papillon 8efe3re ). C $C 1 Sou are e<a*inin& a child with *ild al#inis*: i**unode6ciency and sil3er &rey hi&hli&hts in his hair. Sou dia&nose the child with Chediak" Fi&ashi syndro*e. ,hy are you con6dent that this isnJt 5riscelli syndro*e. 1. 5iant lysoso*al &ranules are present in neutrophils in the #lood s*ear $. 5riscelli syndro*e does not ha3e al#inis* as a feature %. 5riscelli syndro*e has no chan&es in hair color '. All of these answers are correct (. Eone of these answers are correct ). A $C $ MenkeJs kinky hair syndro*e is caused #y a defect in: 1. Mitochondrial &ene $. 0EA helicase %. 5ap 4unction protein '. Copper /ransportin& A/Pase (. Proto"onco&ene ). 0 $C % All of the followin& disorders are e<acer#ated #y B? radiation e<cept: 1. Bloo* syndro*e $. FartnupJs disease %. Hefsu* syndro*e '. Cockayne syndro*e (. Hoth*und"/ho*opson syndro*e ). C $C ' A patient with Bloo* Syndro*e is *ost likely to ha3e which la#oratory a#nor*alities: 1. 0ecreased i**uno&lo#ulins $. Macrocytic ane*ia %. +le3ated 1&+ '. /hro*#ocytopenia (. Positi3e AEA ). A $C ( ,hich of the followin& eye 6ndin&s is caused #y the rupture of BruchGs *e*#rane. 1. An&ioid streaks $. Blue sclerae %. Hetinal detach*ent '. Huptured &lo#e (. Keratoconus ). A $C ) Pili trian&uli et canaliculi is characteristic of which of the followin& syndro*es. 1. Bnco*#a#le hair syndro*e $. B4ornstad syndro*e %. Menkes kinky hair syndro*e A )( '. 8einers disease (. EethertonGs syndro*e ). $C A Patients with pro&eria typically die of which of the followin& conditions. 1. 1nfection $. Metastatic carcino*a %. Atherosclerotic heart disease '. Eail atrophy (. Pro&ressi3e syste*ic sclerosis ). C $C C ,hat is the &enetic defect of this syndro*e. 1. ABCA1$ &ene $. /rans&luta*inase 1 =/5M1>. %. 57B$ =conne<in $)> &ene. '. Steroid sulfatase =S/S> &ene (. Pro6la&&rin &ene ). A $C D Pal*oplantar keratoder*a with deafness is caused #y a defect in which &ene. 1. S8BHP"1 $. Plakophilin %. Mitochondrial serine transferase HEA '. 8ysoso*al papain like cysteine proteinase (. Bnknown ). C $D ! 1n #iopsies fro* #listers in patients with 4unctional epider*olysis #ullosa: the split is found in the: 1. Basal cell layer of the epider*is $. 8a*ina lucida %. 8a*ina densa '. SLua*ous cell layer of the epider*is (. Eone of the answers are correct ). B $D 1 ,hich of the followin& is the 6rst sy*pto* of ata<ia telan&iectasias. 1. Con4uncti3al telan&ictases $. 2acial telan&iectases %. Fe*atolo&ic *al&i&nancy '. Cere#ellar ata<ia (. Breast cancer ). 0 $D $ ,hich of the followin& is not a feature of Cockayne syndro*e. 1. 0war6s* $. Salt and pepper retina %. 1ncreased sister chro*atid e<chan&e '. 1ncreased risk of skin cancer (. Poikiloder*a ). 0 $D % A patient with coarse facies: #road nasal #rid&e: and e<tensi3e ec;e*a *i&ht #e e<pected to ha3e which a#nor*al la#oratory 3alue. 1. Fypertri&lyceride*ia $. Fi&h Copper le3els %. 8ow Kinc le3els '. Fi&h 1&+ le3els (. Ane*ia ). 0 $D ' Patients with Chondrodysplasia punctata can ha3e 6ndin&s of stippled epiphyses on O"ray e<a*ination. ,hich other <"linked do*inant condition can ha3e stippled epiphyses. 1. CF180 syndro*e $. 1ncontinentia Pi&*enti %. 2ocal 0er*al Fypoplasia '. 5olt; syndro*e (. Ba;e< syndro*e ). A )) $D ( ,hich of the followin& features is not associated with Cornelia de 8an&e Syndro*e. 1. Eor*al intelli&ence $. Characteristic facies with downturned *outh: hirsutis*: synophrys: tricho*e&aly: ante3erted nostrils: lon& philtru* and low set ears %. Cryptorchidis* '. 2ifth 6n&er clinodactyly (. Hecurrent lun& infections ). A $D ) Cutaneous *enin&io*as ha3e #een associated with what syndro*e. 1. Eeuro6#ro*atisis $. Cowden %. Eeurocutaneous *elanosis '. 5orlin (. 5lo*an&io*atosis ). A $D A ,hat is the *ost likely syndro*e that this wo*an has in this photo&raph. 1. A. Klippel /renaunay ,e#er $. 2ilariasis %. Eeuro6#ro*atosis '. Proteus syndro*e (. /urner syndro*e ). A $D C A patient presents with star6sh keratoses: pseudoainhu*: honeyco*#ed PPK: and &enerali;ed ichthyosis. ,hat is the *ost likely &enetic defect. 1. Conne<in %1 $. Conne<in $) %. Conne<in %! '. 8oricrin (. Conne<in %% ). 0 $D D A 1A yo *an presents with facial acne that he would like treated. Sou notice that he has 6ne #rown scale on his neck and do a co*plete skin e<a*. /his scale is present on the re*ainder of his #ody: sparin& his pal*s: soles and -e<ural areas. Fe infor*s you that his uncles on his *otherJs side ha3e si*ilar skin 6ndin&s. Fe is not concerned a#out the skin and would like to proceed with acne treat*ent only. ,hat other clinical e<a* should you perfor* to screen for a potential *ali&nancy to which this patient is at hi&her risk for acLuirin&. 1. /esticular $. A#do*inal %. 8y*ph node '. 8un& (. Hectal ). A %! ! ,hich of the followin& conditions is inherited in an O"linked recessi3e *anner. 1. +pider*olysis #ullosa si*ple< $. 1chthyosis 3ul&aris %. S4o&ren"8arsson syndro*e '. ,iskott"Aldrich Syndro*e (. EethertonJs Syndro*e ). 0 %! 1 ,hich of the followin& is a potentially serious co*plication of the #lue ru##er #le# ne3us syndro*e. 1. 0e3elop*ent of chondrosarco*as $. 0e3elop*ent of an&iosarco*as %. 5astrointestinal he*orrha&e '. 0e3elop*ent of ly*phede*a (. 0e3elop*ent of -uid retention C )A ). %! $ 1chthyosis #ullosa of Sie*ens is a condition characteri;ed #y fra&ile #listers at #irth: hyperkeratotic plaLues on el#owsknees later in life: and a &ene *utation in: 1. Keratin $e $. Keratin (1' %. Keratin )a1) '. Keratin 11! (. Eone of these options are correct ). A %! % Cronkhite"Canada is associated with &astrointestinal polyposis: nail atrophy: alopecia: &enerali;ed pi&*entation of skin and *elanotic *acules on the 6n&ers. ,hich of the followin& descri#es its inheritance pattern. 1. Autoso*al recessi3e $. Autoso*al do*inant %. O"linked recessi3e '. O"linked do*inant (. Sporadic ). + %! ' A child presents with *acro&lossia: e<opthal*os and &i&antis*. Fe has a history of o*phalocele repair and has circular depressions on the ri* of the posterior helices. Althou&h this syndro*e is *ost often trans*itted in a sporadic *anner: 1(N of cases ha3e defects in which &ene. 1. K1P$ $. A/M %. ?F8 '. 28/' (. 29OC$ ). A %! ( ,hat is the &ene defect in harleLuin fetus. 1. /rans&luta*inase $. Steroid sulfatse %. ABCA1$ '. ABCC) (. Eone of these answers are correct ). C %! ) Hetention of pri*ary teeth a dental 6ndin& of which of the followin& conditions. 1. Fypo*elanosis of 1to $. 8etterer"Siwe disease %. /u#erous sclerosis '. 7ackson Sertoli syndro*e (. Fyper"1&+ syndro*e ). + %! A /he hair a#nor*ality shown in the i*a&e is characteristic of which of the followin& diseases. 1. Ea<os disease $. /richothiodystrophy %. B4ornstad syndro*e '. Monilethri< (. All of the options are correct ). B %! C Sellow"#rown depositions in 0esce*etGs *e*#rane of the corneas is dia&nostic for: 1. Fe*ochro*atosis $. Eeuro6#ro*atosis %. Fepatolenticular de&eneration '. Pseudo<antho*a elasticu* (. 0ia#etes *ellitus ). C %! D Medullo#lasto*as are seen in which of the followin& syndro*es. 1. Ba;e< syndro*e $. 2a*ilial cylindro*atosis %. Eicolau"Balus syndro*e '. Birt"Fo&&"0u#e syndro*e (. Ee3oid #asal cell carcino*as syndro*e ). + )C '. 5eneral 0er*atolo&y # Question Answers Ans 1 /he *ost co**on 6ndin& in patients with syste*ic sclerosis is: 1. Mi&ratory polyarthritis $. +sopha&eal dysfunction %. Pul*onary 6#rosis '. Henal disease (. Cardiac conduction defects ). Show +<plaination B $ /he causati3e or&anis* for *ucocutaneous leish*aniasis is: 1. 8eish*ania aethiopica $. 8eish*ania infantu* %. 8eish*ania #rasiliensis '. 8eish*ania tropica (. 8eish*ania *a4or ). Show +<plaination C % /he 6rst site in #ody that shows yellowish pi&*entation in carotenoder*a is 1. 2ace $. /runk %. Pal*s and soles A )D '. Eails (. Sclera ). Show +<plaination ' 1ncreased chylo*icrons are a feature of which type of hyperlipoproteine*ia. 1. /ype 1 $. /ype 11a %. /ype 11# '. /ype 111 (. /ype 1? ). Show +<plaination A ( ,hich anti#ody is D%N speci6c for S4o&renGs syndro*e. 1. anti"Ho $. anti"8a %. anti"fodrin '. anti"Schir*er (. H2 ). Show +<plaination C ) Solid con-uent pal*oplantar keratosis: sal*on" oran&e follicular papules and diMuse sy**etrical in3ol3e*ent with characteristic s*all islands of nor*al skin within aMected areas are all clinical characteristics of what skin disease. 1. 8ichen Sclerosis $. 0er*ato*yosistis %. Phrynoder*a '. Heiter\]^ ^Ys syndro*e (. Pityriasis ru#ra pilaris ). Show +<plaination + A /his wo*an has restricted a#ility to open her *outh with ti&ht #ound down skin of her hands and pul*onary 6#rosis. 1n addition to a positi3e AEA with a nucleolar pattern: what is the *ost co**on autoi**une anti#ody that is associated with pul*onary 6#rosis. 1. /opoiso*erase 1 anti#odies =for*erly Scl"A!> $. 2i#rillarin anti#odies %. Anti"B%HEP anti#odies '. Anti"PM"Scl anti#odies (. Anti"thyro&lo#ulin anti#odies ). Show +<plaination A C ,hich of the followin& is a feature of Schnit;lerGs Syndro*e. 1. Monoclonal 1&5 &a**opathy $. /hro*#oe*#olic e3ents %. Bronchospas* '. Fe*aturia (. Sensori*otor neuropathy ). Show +<plaination + D ,hich of the followin& is an e<a*ple of a lar&e 3essel 3asculitis. 1. /akayasu arteritis $. ,e&enerGs &ranulo*atosis %. Chru&"Strauss syndro*e '. Polyarteritis nodosa (. Fenoch"Schonlein purpura ). Show +<plaination A 1! 1n this patient with an autoi**une disorder: which autoanti#ody would #e indicati3e of increased risk of pul*onary disease. 1. B1HEP $. 0s0EA %. anti"7o"1 anti#ody '. Mi"$ anti#ody (. Scl"A! anti#ody ). Show +<plaination C 11 ,hich state*ent #est descri#es the *echanis* of action of spironolactone. 1. Eon"steroid inhi#itor of 0F/ #indin& to the andro&en receptor $. Pro&estin %. 5nHF a&onist '. Mineralocorticoid (. Steroid inhi#itor of andro&en receptor and andro&en #iosynthesis ). Show +<plaination + 1$ A patient presents with lesion that can #e herniated into the skin on palpation. ,hat is thou&ht to #e i*portant in the patho&enesis of this condition. 1. Calci6cation of the elastic 6#ers $. 2or*ation of e<cessi3e colla&en %. 2ocal loss of elastic tissue '. Atrophy of the epider*is (. 1n-a**ation of the panniculus ). Show +<plaination C 1% /he lesions shown here are *ost likely to: 1. 9ccur in infants and elderly patients $. 9ccur as an ad3erse reaction to *edication %. Be refractory to treat*ent '. 2ollow a streptococcal infection (. +rupt after the appearance of a Jherald patchJ ). Show +<plaination 0 1' BehcetGs disease is associated with which F8A type. 1. F8A"B(1 $. F8A"B1A %. F8A"B1% '. F8A"0H' (. F8A"Cw) A A! ). Show +<plaination 1( ,hich of the followin& is true re&ardin& this entity. 1. +<tra&enital lesions co**only in3ol3e the torso and are usually pruritic $. Childhood onset is reported in _(N of cases %. $!N of #oth *en and wo*en with this dia&nosis ha3e at least one autoi**une disease '. 5irls outnu*#er #oys $:1 (. 5enital disease represents (!N of childhood cases ). Show +<plaination C 1) AcLuired C1 esterase inhi#itor de6ciency results in: 1. A nor*al le3el of seru* C1L $. A decreased le3el of seru* C1L %. Eone of these answers are correct '. A presentation of an&ioede*a 3ery early in life (. A nor*al le3el of C' ). Show +<plaination B 1A ,hich of the followin& *edications is not associated with e<acer#atin& this condition. 1. 8ithiu* $. Prednisone %. Phenytoin '. 1sonia;id (. Propranolol ). Show +<plaination + 1C ,hich of the followin& is not a co**on food or en3iron*ental aller&en i*plicated in atopic der*atitis. 1. 0er*atopha&oides pteronyssi*us $. ,heat %. Corn '. +&&s (. 2ish ). Show +<plaination C 1D HEP anti#odies include: 1. anti"SSA: anti"centro*ere: anti"SSB $. anti"SSA: anti"SSB: anti"S*: anti"B1HEP %. anti"SSA: anti"SSB: anti"S*: anti"ds0EA '. AEA: anti"ds0EA (. anti"SSA: anti"B1HEP: anti"centro*ere ). Show +<plaination B $! A 1) year old de3elop*entally nor*al *ale presents to his pediatrician inter*ittent 3a&ue epidodes of hand and feet paresthesias and non speci6c episode of 51 distress. Fe is referred to you to e3aluate nu*erous punctate to ( sli&htly 3errucous: deep"red to #lue"#lack papules distri#uted diMusely on his trunk in a #athin& suit distri#ution. Polari;ation *icroscopy of the sedi*ent of his urine de*onstrates #irefrin&ent lipid &lo#ules =ie: renal tu#ular epithelial cells or cell fra&*ents with lipid inclusions> with the characteristic Maltese cross con6&uration. ,hat is the classic ocular 6ndin& in this disorder. 1. Pseudo"herpetic corneal ulcerations $. Brush6eld spots %. Fyperpi&*entation of the retinal epitheliu* '. Corneal opacities (. 8ester irides ). Show +<plaination 0 $1 A pre&nant wo*an in her third tri*ester presents with nonpruritic erythe*atous plaLues and pustules in the intertri&inous re&ions: the trunk: and the e<tre*ities as well as syste*ic sy*pto*s of *alaise and fe3er. ,hich co*plication is *ost associated with this condition. 1. Pre*ature la#or $. Placental insuTciency %. Eo risk to fetus or *other '. S*all for &estational a&e (. Microcephaly ). Show +<plaination B $$ A D"year"old #oy presents with calcinosis cutis: perior#ital 3iolaceous erythe*a: and periun&ual telan&iectasias. ,hich su#stance is *ost likely to #e ele3ated in this patient. 1. 2odrin $. 2i#rillarin %. /hro*#ospondin"1 '. Co*ple*ent (. /ri&lycerides ). Show +<plaination C $% 8acri*al &land in3ol3e*ent is characteristically seen in patients with: 1. 8y*pho*a $. Sarcoid %. Hosacea '. Syphilis (. Pe*phi&us ). Show +<plaination B $' A child has a cutaneous ossi6cation disorder characteri;ed #y endochondral #one for*ation: deafness: #aldness: and *ental retardation. ,hich for* of osteo*a cutis does this patient 1. 2i#rodysplasia ossi6cans pro&ressi3a $. Pro&ressi3e osseous heteroplasia %. Plate"like osteo*a cutis '. Al#ri&htGs hereditary osteodystrophy A A1 ha3e. (. Calcinosis cutis ). Show +<plaination $( ,hich of the followin& is an innate anti*icro#ial peptide e<pressed #y keratinocytes in response to in4ury or in-a**ation. 1. A"defensin 1 $. A"defensin $ %. /E2"A '. 18"' (. 18"1% ). Show +<plaination B $) /his 3ariant of a*yloidosis is deri3ed fro* de&enerated tono6la*ents of keratinocytes: 1. 8ichen a*yloidosis $. Eodular a*yloidosis %. Pri*ary syste*ic a*yloidosis '. 0ialysis"related a*yloidosis (. Secondary syste*ic a*yloidosis ). Show +<plaination A $A Pilo*atrico*as has #een associated with which of the followin& conditions. 1. Acute *yelo&enous leuke*ia $. Multiple sclerosis %. Crohns 0isease '. Myotonic dystrophy (. Pneu*othora< ). Show +<plaination 0 $C P. acnes acti3ation of which of the followin& *ay sti*ulate a pro"in-a**atory cytokine cascade. 1. /8H"$ $. /8H"( %. 18"1 '. 18"C (. /E2"!!! ). Show +<plaination A $D /eena&e #oys with se3ere: erupti3e cystic acne and fe3er: leukocytosis: *yal&ias and other constitutional sy*pto*s can ha3e which of the followin& #ony chan&e. 1. 0i*plin& a#o3e the (th MCP $. 9steolytic lesions of the cla3icle %. 9steopoikilosis '. Stippled epiphyses (. 7aw cysts ). Show +<plaination B %! An o3erwei&ht: post"*enopausal wo*an presents with intense pain in the fatty deposits on her knees: thi&hs and hips. She also descri#es swellin& of her hands and feet: associated fati&ue: and history of depression. 9n clinical e<a* the pain in the adipose tissue appears out of proportion to the clinical 6ndin&s. /he dia&nosis is: 1. An&iolipo*as $. Adiposis dolorosa %. Con&enital lipodystrophy '. 8ipoder*atosclerosis (. +rythe*a nodosu* ). Show +<plaination B %1 1ncreased 808 and ?808 is seen in which type of hyperlipoproteine*ia. 1. /ype 11# $. /ype 1 %. /ype 111 '. /ype 1? (. /ype ? ). Show +<plaination A %$ A (!"year"old fe*ale with sarcoid has enlar&e*ent of the parotid: su#*andi#ular: and lacri*al &lands that is also known as: 1. Mikulic;Gs syndro*e $. Feerfordt",aldenstro*n syndro*e %. 0arier"Houssy sarcoid '. 8of&renGs syndro*e (. BlauGs syndro*e ). Show +<plaination A %% ,hat is the *ini*u* a*ount of ti*e that you would ad3ise this wo*an to a3oid child#earin& after co*pletin& a course of isotretinoin. 1. 1 week $. 1 *onth %. ) *onths '. 1 year (. % years ). Show +<plaination B %' ,hich of the followin& leukoder*as has a nor*al nu*#er of epider*al *elanocytes. 1. Al#inis* $. ?itili&o %. Pie#aldis* '. ,aarden#ur&Gs syndro*e (. Kiprowski"Mar&olis syndro*e ). Show +<plaination A %( ,hich of the followin& is true re&ardin& topical therapies for psoriasis. 1. ?ita*in 0% analo&ues deacti3ate salicylic acid $. Hetinoids are eMecti3e in decreasin& lesional erythe*a %. Anthralin can cause irre3ersi#le stainin& of peri"lesional skin '. Calcipotriol is deacti3ated #y B? li&ht (. Anthralin inhi#its PMEs and *onocytes + A$ ). Show +<plaination %) A $! year old wo*an presents with a sunken appearance to her face and trunk with nor*al le&s. /he patient has attri#uted her appearance to stress fro* colle&e: #ut is seein& you #ecause of her fa*ilyGs concern. ,hat la# 6ndin& would you e<pect in this patient 1. 0ecreased C% nephritic factor $. 0ecreased C% %. 0ecreased C1 '. 1ncreased C1 (. 1ncreased C' ). Show +<plaination B %A +osinophilia: asth*a: neuropathy and sinus a#nor*alities are associated with which of the followin& syste*ic 3asculitidies. 1. Chur&"Strauss syndro*e $. Polyarteritis nodosa %. Microscopic polyan&iitis '. ,e&enerGs &ranulo*atosis (. Kawasaki disease ). Show +<plaination A %C An atypical 6ndin& in patients with 8of&ren syndro*e is. 1. 2e3er $. Arthritis %. Su#cutaneous /ender Eodules '. Acute 1ritis (. Beaded papules on nasal ri* ). Show +<plaination + %D Carcinoid tu*ors in which site do not lead to -ushin& or other *anifestations of the carcinoid syndro*e. 1. Appendi< $. 1leu* %. Ascendin& colon '. Hectu* (. Brochus ). Show +<plaination 0 '! Patients with this F8A type: which has the *ost de6niti3e association with psoriasis: ha3e a relati3e risk of ha3in& psoriasis that is D"1( ti*es nor*al. 1. F8A"B1% $. F8A"B1A %. F8A"Bw(A '. F8A"Cw) (. F8A"B$A ). Show +<plaination 0 '1 ,hich of the followin& would #e an i*portant dia&nostic si&n of this non"infectious disorder. 1. 8acri*al &land enlar&e*ent $. Periun&ual 6#ro*as %. Cafe"au"lait *acules '. Pe& shaped teeth (. 0ystopia canthoru* ). Show +<plaination A '$ /he risk of de3elopin& syste*ic in3ol3e*ent in a patent with nodular a*yloidosis is: 1. 1"'N $. ("1!N %. $("%!N '. (!")!N (. `D(N ). Show +<plaination B '% /his patient also has poliosis and deafness. ,hat is the associated ophthal*olo&ic 6ndin&. 1. Eor*al ophthal*olo&ic e<a* $. Hetinal detach*ent %. 8isch nodules '. Corneal opacities (. 5ranulo*atous u3eitis ). Show +<plaination + '' Sou prescri#e oral erythro*ycin to a %( year"old wo*an. Co"ad*inistration of which of the followin& *edications could lead to potential ad3erse outco*es. 1. 9ral contracepti3es $. ,arfarin %. Car#a*a;epine '. Methylprednisolone (. ,arfarin: car#a*a;epine: or *ethylprednisolone ). Show +<plaination + '( ,hat is the treat*ent of choice for this condition: 1. /opical antifun&al *edication $. Fi&h potency topical steroid %. /opical anti#iotic crea* '. 9ral anti#iotic (. +<cision ). Show +<plaination B ') ,hat is the *ost co**on paraproteine*ia in sclero*y<ede*a. 1. 1&A $. 1&5 kappa %. 1&5 la*#da '. 1&M (. 1t is e<tre*ely unco**on to see a paraproteine*ia with sclero*y<ede*a ). Show +<plaination C 'A Fistolo&ic e<a*ination of this chronic pruritic plaLue on the #ack: which of the followin& stains 1. ?on Kossa $. Cotton dyes A A% would E9/ #e positi3e. %. Crystal 3iolet '. PAS (. /hio-a3in / ). Show +<plaination 'C /he *ost eMecti3e treat*ent for sy*pto*atic ocular rosacea is: 1. 8u#ricant eye drops $. Co*#ination topical anti#iotic and corticosteroid eye drops %. 0ilute #a#y sha*poo washes '. 0o<ycycline (. Arti6cial tears ). Show +<plaination 0 'D Most often: <anthelas*a is associated with: 1. An 1&M paraprotein $. An 1&5 paraprotein %. Eo associated disease '. /ype 1 hyperlipoproteine*ia (. /ype 111 hyperlipoproteine*ia ). Show +<plaination C (! ,hat is the dia&nosis of this lesion on the penile shaft. 1. Sclerosin& ly*phan&itis $. 8oaloa %. ?aricose 3ein '. Syphilis (. 8y*pho&ranulo*a 3enereu* ). Show +<plaination A (1 ,hat is the *ost likely dia&nosis. 1. ?oi&ht line $. 8inea ni&ra %. 2uchter line '. Phytophotoder*atitis (. 1ncontinentia pi&*enti ). Show +<plaination B ($ A patient presents to the +H with skin pain and lar&e sheets of epider*al detach*ent on &reater than 1!N of his #ody surface area after startin& #actri*. Eo purpuric *acules or tar&et lesions are noted. ,hich of the followin& can help predict *ortality in this patient. 1. &lucose &reater than 1(! *&d8 $. a&e o3er '! years %. heart rate &reater than 1!! '. history of dia#etes (. BBE &reater than 1( *&d8 ). Show +<plaination B (% Patients with 5ottronJs papules and periun&ual telan&iectasias will likely ha3e a positi3e serolo&ic test to which anti#ody. 1. Anti"SM $. Scl"A! %. Anti"Ho '. Anti"centro*ere (. Anti" 7o"1 ). Show +<plaination + (' ,hat is the condition which is a dia&nostic cutaneous *anifestation of sarcoid. 1. 0iscoid lupus $. 8upus pernio %. 8upus 3ul&aris '. 5ranulo*atous rosacea (. Hhinosclero*a ). Show +<plaination B (( ,hich of the followin& state*ents co*parin& in-a**atory lesions fro* patients with psoriasis and patients with atopic der*atitis is true. 1. Fu*an J"defensin $ =FB0"$> and 88 %A le3els in psoriasis lesions are si&ni6cantly lower than in atopic der*atitis lesions $. +<pression of FB0"$ and 88 %A is ne&li&i#le in psoriatic lesions %. FB0"$ and 88 %A are si&ni6cantly ele3ated in atopic der*atitis lesions '. +<pression of FB0"$ and 88 %A in psoriasis is si*ilar to nor*al skin: whereas it is decreased in atopic der*atitis lesions (. FB0"$ and 88 %A are si&ni6cantly decreased in atopic der*atitis lesions co*pared to psoriasis lesions ). Show +<plaination + () A patient with holocar#o<ylase synthetase de6ciency is *ost at risk for de3elopin& a de6ciency in: 1. Eiacin $. Pyrido<ine %. Biotin '. Kinc (. +ssential fatty acids ). Show +<plaination C (A Sou are suspicious for a new dia&nosis of lupus in a patient recently treated with penicilla*ine. ,hich anti#odies would you e<pect to ha3e #een induced #y this *edication. 1. anti"ds0EA $. anti"histone %. anti"SSA '. anti"SSB (. anti"Mi"$ A A' ). Show +<plaination (C ,hich cellular or&anelle is aMected in the partial lidodystrophies. 1. Eucleolus $. Hou&h endoplas*ic reticulu* %. 5ol&i apparatus '. Cell *e*#rane (. Euclear la*ina ). Show +<plaination + (D Anti"7o"1 anti#odies in patients with 0er*ato*yositis are associated with: 1. Pul*onary 6#rosis $. Cardiac disease %. Photosensiti3ity '. Calcinosis (. Feliotrope rash ). Show +<plaination A )! Ci&arette s*okin& has #een shown to: 1. 1ncrease the incidence of BCC $. Eot aMect the sur3i3al of sur&ical repairs %. 0e*onstrate less solar elastosis than photoa&ed skin '. Fa3e no correlation with facial wrinklin& (. 1ncrease cutaneous 3asoconstriction ). Show +<plaination + )1 A 'C year"old *an with a lon& history of alcohol a#use and dependence presents with an&ular cheilitis: atrophic &lossitis: a scrotal der*atitis that spares the *idline and e<tends to the thi&hs: photopho#ia: and #lepharitis. ,hich nutritional de6ciency do you suspect. 1. ?ita*in B1$ $. ?ita*in C %. Pyrido<ine '. ?ita*in B$ (. 1ron ). Show +<plaination 0 )$ A $ year"old #oy with crusted skin papules is found to ha3e osteolytic defects and dia#etes insipidus. /hese features are seen in: 1. 9steo&enesis i*perfecta $. Conradi"Funner*an disease %. Metastatic s*all cell lun& carcino*a '. Fand"Schuller"Christian disease (. +pider*al ne3us syndro*e ). Show +<plaination 0 )% ,hat is the *ost co**on *ali&nancy associated with this condition in this fe*ale. 1. Brain cancer $. /hyroid cancer %. Breast cancer '. 93arian cancer (. 8i3er cancer ). Show +<plaination 0 )' /he *a4ority of patients with positi3e B1HEP autoanti#odies ha3e which dia&nosis. 1. Mi<ed connecti3e tissue disorder =MC/0> $. Hheu*atoid arthritis %. Syste*ic lupus erythe*atosus '. S4o&renGs syndro*e (. 0er*ato*yositis ). Show +<plaination C )( A '("year old wo*an fro* the Bnited States de3elops erythe*a and swellin& around her eyelids and sy**etric weakness of her shoulders and hips. ,hat *ali&nancy is o3errepresented in patients with this condition co*pared to the &eneral population. 1. 93arian Cancer $. 8un& Cancer %. 8euke*ia '. Bterine Cancer (. Breast Cancer ). Show +<plaination A )) A ($ year"old *an presents with lar&e co*edones as well as in-a**atory papules: pustules: and cysts on the *alar cheeks: postauricular scalp: and scrotu*. ,hich of the followin& could #e a cause of this presentation. 1. +rlotini# che*otherapy $. A pituitary adeno*a %. $:%:A:C tetrachloro#en;odio<in e<posure '. P/+E *utations (. 1sotretinoin o3erdose ). Show +<plaination C )A 1n the a#sence of the C1 esterase inhi#itor: which of the followin& proteins *ediates an&ioede*a *ost directly: 1. Kallikrein $. C1 %. Plas*in '. C% con3ertase (. C$ kinin ). Show +<plaination + )C /he pi&*entation shown here is *ost likely due to which of the followin& dru&s. 1. ChloroLuine $. A*iodarone %. Minocycline '. 0o<ycycline (. /MP"SMO ). Show +<plaination C )D ,hat syste*ic condition is often associated with this disease. 1. 0ia#etes *ellitus $. /o#acco use %. Alopecia areata + A( '. Coronary artery disease (. Fepatitis C ). Show +<plaination A! Mali&nancy of the aerodi&esti3e tract is *ost closely associated with which paraneoplastic der*atosis. 1. Ba;e<Gs syndro*e $. Paraneoplastic pe*phi&us %. SweetGs syndro*e '. Acanthosis ni&ricans (. 0er*ato*yositis ). Show +<plaination A A1 A patient with a recent dia&nosis of tu#erculosis on treat*ent presents with a new photosensiti3e eruption on his face: neck and upper chest. 1n addition: he has pro&ressi3e diarrhea and depression. ,hat is the etiolo&y of his sy*pto*s. 1. ?ita*in B1$ de6ciency $. 0ru& induced pella&ra %. 0ru& induced ri#o-a3in de6ciency '. Carcinoid syndro*e (. Photoaller&ic contact der*atitis ). Show +<plaination B A$ ,hich of the followin& is a clinical *anifestation of neonatal lupus erythe*atosus: 1. Henal disease $. Cere#ral 3ascular accident %. Arthritis '. /hro*#ocytopenia (. Eeutrophilia ). Show +<plaination 0 A% ,hich of the followin& is true re&ardin& neonatal lupus erythe*atosus. 1. Most cases in3ol3e #oys $. 8esions &enerally resol3e spontaneously #y ) *onths: healin& with scarrin& %. Photosensiti3ity is &enerally not a feature '. A(N of *others ha3e sy*pto*atic syste*ic lupus erythe*atous at the ti*e of deli3ery (. Con&enital heart #lock *ay #e the only *anifestation of the disease ). Show +<plaination + A' ,hat is the *ost co**on infection that can cause &uttate psoriasis. 1. Streptococcus pyo&enes $. Coccidio*ycosis %. Ferpes '. Mycoplas*a pneu*onia (. /u#erculosis ). Show +<plaination A A( /he dia&nosis is: 1. Psoriasis $. Alopecia areata %. 9nycho*ycosis '. 8ichen planus (. /etracycline"induced photo"onycholysis ). Show +<plaination A A) A ($ year old patient with lon&standin& psoriasis has #een treated with *ethotre<ate for the past ) *onths. Fe has had al*ost co*plete clearance of his disease. Fe now co*plains of a su#tle ri&ht upper Luadrant pain and nausea. 8a#oratory e3aluation of hepatic a*inotransferases =AS/ and A8/> and #iliru#in are within nor*al li*its. ,hich if the followin& nonin3asi3e tests *ay #e helpful for *onitorin& *ethotre<ate"induced hepatoto<icity. 1. C+A $. transfor*in& &rowth factor #eta %. tu*or necrosis factor alpha '. Procolla&en 111 a*inopeptide (. C% and C' ). Show +<plaination 0 AA Pro#iotics: which are cultures of potentially #ene6cial &ut *icro-ora #acteria: ha3e #een studied in the pri*ary pre3ention of which of the followin& diseases. 1. Celiac disease $. Atopic der*atitis %. Psoriasis '. Cutaneous /"cell ly*pho*a (. Asth*a ). Show +<plaination B AC ,hich of the followin& is true re&ardin& acLuired C1 esterase inhi#itor de6ciency. 1. /his condition &enerally occurs in the 6rst or second decade of life $. Seru* C1L is nor*al %. C$ and C' are #oth decreased '. C1 esterase inhi#itor *ay #e at nor*al le3els with functional i*pair*ent (. Positi3e fa*ily history is co**on ). Show +<plaination C AD A patientGs #iopsy is found to ha3e curled hair shafts with hyperkeratotic plu&s. ,hich of the followin& is not an associated feature of this condition 1. Fypochondriasis $. 5in&i3itis %. 0elayed wound healin& '. Eail chan&es (. /ender nodules on the lower e<tre*ities ). Show +<plaination 0 A) C! ,hich of the followin& *edications is *ost likely to induce a -are of this patientGs disease. 1. cipro-o<acin $. captopril %. diphenhydra*ine '. sertraline (. aceto*inophen ). Show +<plaination B C1 /he der*atosis pictured is *ost co**only associated with: 1. 1nsulin resistance $. Cushin&Gs syndro*e %. Acro*e&aly '. Polycystic o3arian syndro*e (. 5astric carcino*a ). Show +<plaination A C$ ,hich of the followin& is true re&ardin& the co*ponents of a*yloid. 1. 5round su#stance is present only in secondary cutaneous or tu*or"associated a*yloidosis $. /he a*yloid P co*ponent is present in all for*s %. Protein AA is present in pri*ary syste*ic a*yloidosis '. /he a*yloid present in secondary syste*ic a*yloid does not lose its #irefrin&ence after treat*ent with potassiu* per*an&anate (. Protein A8 loses its #ire&rin&ence after treat*ent with potassiu* per*an&anate ). Show +<plaination B C% /reat*ent of HaynaudGs pheno*enon includes: 1. Eifedipine $. Metoprolol %. B?B '. /etracyline (. Si*3astatin ). Show +<plaination A C' 1n addition to the lesions seen on the feet of this youn& *an he also had &eo&raphic ton&ue: erythe*atous plaLues on his penis and arthritis: what F8A type is associated with this syndro*e. 1. F8A"B$A $. F8A"B(1 %. F8A"0H1 '. F8A"0H% (. F8A"0Q,$ ). Show +<plaination A C( Acute he*orrha&ic ede*a of childhood is distin&uished fro* Fenoch"Schonlein Purpura #ased on: 1. /he presence of pupura on the upper trunk $. /he lack of an antecedent infection %. /he in3ol3e*ent of the syno3ia '. /he neurolo&ic co*plications (. /he lack of syste*ic features ). Show +<plaination + C) /he Iabfollicular occlusion triadI consists of: 1. Acne con&lo#ata: hidradenitis suppurati3a: kerion $. Acne con&lo#ata: chloracne: pyoder*a faciale %. Acne ful*inans: hidradenitis suppurati3a: dissectin& cellulites of the scalp '. Acne co*edonicus: hidradenitis suppurati3a: dissectin& cellulites of the scalp (. Acne con&lo#ata: hidradenitis suppurati3a: dissectin& cellulites of the scalp ). Show +<plaination + CA Child a#use is often incorrectly suspected when a youn& &irl presents with which cutaneous 6ndin&. 1. Molluscu* conta&iosu* $. 8ichen sclerosus et atrophicus %. Condylo*a accu*inata '. +cchy*oses on face and hands (. 5enital herpes si*ple< ). Show +<plaination B CC ,hich special stain is utili;ed to con6r* the dia&nosis of 8an&erhans cell histiocytosis. 1. F@+ suTcient for dia&nosis $. S1!! %. C0$! '. C01a (. Chloroacetate esterase ). Show +<plaination 0 CD Acrokeratosis paraneoplastica is *ost co**only associated with which of the followin& *ali&nancies: 1. SLua*ous cell carcino*a $. Adenocarcino*a %. /ransitional cell carcino*a '. /"cell ly*pho*a (. Breast cancer ). Show +<plaination A AA D! SweetGs syndro*e *ay #e caused #y: 1. Bleo*ycin $. Cyto<an %. 5ranulocyte colony sti*ulatin& factor '. 1ntra3enous i**une &lo#ulin (. Methotre<ate ). Show +<plaination C D1 A patient with in-a**atory #owel disease de3elops acute tender 4uicy plaLues on the head and neck: fe3er: and *alaise. /he skin lesions respond well to prednisone. 0ata shows increased +SH and neutrophilic in6ltrate with der*al ede*a on skin #iopsy. ,hich one of the followin& is a *a4or criteria for this condition. 1. 2e3er and *alaise $. Fistory of in-a**atory #owel disease %. A#rupt onset of plaLues '. 1ncreased +SH (. 5ood response to prednisone ). Show +<plaination C D$ ,hat is the treat*ent of choice for neurotic e<coriations. 1. Hisperidone $. 0o<epin %. 9lan;apine '. 5a#apentin (. 0iphenhydra*ine ). Show +<plaination B D% Spindle cell lipo*a is co**only found on the: 1. Fead $. 8ower e<tre*ities %. Buttocks '. Breast (. Posterior shoulder ). Show +<plaination + D' A patient with spontaneous occurrence of petechiae and purpura: particularly around the eyes: will also *ost likely ha3e which of the followin&. 1. 0iMuse scalin& of the scalp $. Acne %. Cheilits '. Macro&lossia (. B3eitis ). Show +<plaination 0 D( A %("year"old *an presents with scattered in6ltrati3e cutaneous plaLues: chronic fe3er: parotid &land enlar&e*ent: and a facial ner3e palsy. ,hat other 6ndin& is *ost likely on physical e<a*. 1. Anterior u3eitis $. Periun&ual telan&iectasia %. Eail pits '. Eon"scarrin& alopecia (. Condylo*a lata ). Show +<plaination A D) A %( year old *an with a history of celiac disease presents with a #eefy: red ton&ue: hyperpi&*ented pal*ar creases: and pre*ature &rey hair. ,hich of the followin& state*ents are is correct. 1. /he #est therapy is ri#o-a3in (*&day $. /his condition *i*ics folate de6ciency %. 1t is often associated with carcinoid tu*ors which di3ert tryptophan to serotonin '. /his condition can #e caused #y a;ithioprine: ("2B: and isonia;id (. +atin& raw e&& whites is a risk factor ). Show +<plaination B DA /he dia&nosis is 1. Psoriasis: $. Alopecia areata %. 9nycho*ycosis '. 8ichen planus (. /etracycline"induced photo"onycholysis ). Show +<plaination 0 DC 1n lichen planus pe*phi&oides: 1. Bullae de3elop characteristically in lesions of lon&standin& lichen planus $. Circulatin& 1&5 anti#odies react to the $%! k0a anti&en within the #ase*ent *e*#rane ;one %. /here is &ranular deposition of 1&5 and C% at the der*oepider*al 4unction '. Bullae result fro* intense lichenoid in-a**ation and e<tensi3e liLuefacti3e de&eneration of #asal keratinocytes (. ?esicles *ay de3elop de no3o on pre3iously unin3ol3ed skin ). Show +<plaination + DD ,hat is the *ost likely dia&nosis. 1. 0er*ato6#ro*asarco*a protu#erans $. 0er*ato6#ro*a %. 8o#o*ycosis '. Blasto*ycosis (. Keloid ). Show +<plaination + 1! ! ,hat is the treat*ent of choice for this patient who de3eloped acne ful*inans one *onth after 1. Add oral prednisone $. 1ncrease isotretinoin dose A AC startin& isotretinoin therapy. %. Add do<ycycline '. Acne sur&ery (. Check lipid le3els ). Show +<plaination 1! 1 A patient presents with recurrent &enital and oral ulcerations and a dia&nosis of posterior u3eitis. ,hat F8A type is associated with the dia&nosis you suspect. 1. F8A"B$A $. F8A"B(1 %. F8A"0H% '. F8A"Cw) (. F8A"0H' ). Show +<plaination B 1! $ ,hat is the *ost co**on cause of erythroder*a in non"F1? patients. 1. 0ru&s $. Bnderlyin& *ali&nancy %. Pre"e<istin& der*atoses '. 1nfection (. 1diopathic ). Show +<plaination C 1! % +ye#row &rowth: 1. 1s andro&en"dependent in *en #ut not in wo*en $. 1s re&ulated #y dehydroepiandrosterone #ut not androstenedione %. 1s not andro&en"dependent '. HeLuires con3ersion of testosterone to dihydrotestosterone in the hair follicle (. 1s re&ulated only #y testosterone and dihydrotestosterone ). Show +<plaination C 1! ' ,hich of the followin& is a *anifestation of psoriasis of the nail *atri<. 1. Splinter he*orrha&es $. J9il spotsJ %. Su#un&ual hyperkeratosis '. Pits (. 9nycholysis ). Show +<plaination 0 1! ( Conco*itant discoid lupus is found in what percenta&e of patients with su#acute cutaneous lupus. 1. (N $. $!N %. '!N '. )!N (. C!N ). Show +<plaination B 1! ) ,hich anti#ody is speci6c for CH+S/ syndro*e. 1. Anti"*itochondrial $. Anti"histone %. Anti"ds 0EA '. Anti"nucleolar (. Anti"centro*ere ). Show +<plaination + 1! A Patients with psoriasis treated with cyclosporine should #e *onitored for: 1. Sicca sy*pto*s $. Fyper*a&nese*ia %. Alopecia '. Acute interstitial pneu*onitis (. Fyperkale*ia ). Show +<plaination + 1! C A '("year"old wo*an de3elops #ilateral poikiloder*atous patches without atrophy on her lateral thi&hs. /his is *ost likely a *anifestation of what disease. 1. 8upus +rythe*atosus $. 0er*ato*yositis %. Mycosis 2un&oides '. Fepatitis C (. 0ia#etes Mellitus ). Show +<plaination B 1! D ,hich of the followin& state*ents is true re&ardin& this entity. 1. C!N of patients with early onset disease ha3e a positi3e fa*ily history $. /win concordance =identical twins> is ((N %. /he inner root sheath and *atri< of nor*al hair e<press MFC class 1 '. /he sisapho pattern in3ol3es the occipital scalp (. Atopic der*atitis is a predictor of &ood pro&nosis ). Show +<plaination B 11 ! A $( year"old pre3iously healthy *an presents with the skin 6ndin&s shown: urethritis: and one *onth of peripheral arthritis. ,hich of the followin& is true re&ardin& this condition. 1. 2e*ales and *ales are eLually aMected $. A chronic defor*in& arthritis occurs in $!N %. /E2"alpha inhi#itin& a&ents ha3e no role in the treat*ent of this condition '. Patients *ust ha3e urethritis: con4uncti3itis: and arthritis for dia&nosis B AD (. Chla*ydia cer3icitis is not associated with this condition ). Show +<plaination 11 1 ,hich of the followin& disorders is pri*arily associated with a *onoclonal &a**opathy of the 1&A type. 1. Sclero*y<ede*a $. Sclerede*a %. Eecro#iotic <antho&ranulo*a '. Schnit;lerGs syndro*e (. Pyoder*a &an&renosus* ). Show +<plaination + 11 $ /he *echanis* of action of a;ithro*ycin is: 1. 1nhi#ition of #acterial cell wall synthesis $. 1nhi#ition of HEA"dependent protein synthesis #y #indin& to the %!s ri#oso*al su#unit %. 1nhi#ition of HEA"dependent protein synthesis #y #indin& to the (!s ri#oso*al su#unit '. 1nhi#ition of 0EA"dependent HEA poly*erase (. 1nhi#ition of #acterial topoiso*erase ). Show +<plaination C 11 % A positi3e AEA with a speckled stainin& pattern correlates with stainin& of what cellular co*ponent. 1. Hi#onucleoproteins $. Eucleolar HEA %. Eati3e 0EA '. Kinetochore (. Fistones ). Show +<plaination A 11 ' A %( year old *an with plaLue psoriasis well" controlled with twice daily application of calcipotriene !.!!(N crea* noticed a -are of his psoriasis shortly after he started usin& a prescription *oisturi;er lotion for psoriasis. ,hich is the *ost likely e<planation for the apparent decreased eTcacy of calcipotriene. 1. /he *oisturi;er i*paired penetration of the calcipotriene $. /he *oisturi;er diluted the calcipotriene %. /he two e3ents are unrelated '. /he prescription *oisturi;er likely contains lactic acid. (. /he decreased eTcacy is due to tachyphyla<is ). Show +<plaination 0 11 ( /he *ost co**on associated disorder in a patient with elastosis perforans serpi&inosa is: 1. Marfan syndro*e $. 0own syndro*e %. 9steo&enesis i*perfecta '. +hlers"0anlos syndro*e (. Hoth*und"/ho*so* syndro*e ). Show +<plaination B 11 ) 8ichen planus pe*phi&oides has #een associated with an anti&en to which structure. 1. /ype O?11 colla&en $. Fe*ides*oso*e plaLue %. 0es*o&lein % '. 0es*o&lein 1 (. /ype ?11 colla&en ). Show +<plaination A 11 A ,hich of the followin& cytokines is *ost characteristic of atopic der*atitis in its chronic phase. 1. 1nterferon"&a**a $. 18"' %. 18"( '. 18"1! (. 18"1% ). Show +<plaination A 11 C Menopausal -ushin& can #e eMecti3ely treated with: 1. Eadolol $. /ryptophan %. Clonidine '. 0ana;ol (. /a*o<ifen ). Show +<plaination C 11 D ,hich of the followin& hor*ones #ind the andro&en receptor. 1. 0ehydroepiandrosterone $. Androstenedione %. 0ihydrotestosterone '. 0ehydroepiandrosterone and dihydrotestosterone (. 0ehydroepiandrosterone: androstenedione: and dihydrotestosterone ). Show +<plaination C 1$ ! Bullous lupus erythe*atosus is *ost co**only associated with anti#odies to: 1. /ype 1? colla&en $. 8a*inin ( %. 0es*o&lein 1 '. Plectin (. /ype ?11 colla&en + C! ). Show +<plaination 1$ 1 ,hich of the followin& co*ple*ent pro6les would su&&est a dia&osis of hereditary an&ioede*a =C1 esterase inhi#itor de6ciency>. 1. C$ hi&h and C' hi&h $. C1 low and C' hi&h %. C% hi&h and C1"inh decreased '. CF (! low and C1"inh hi&h (. C1"inh nor*al: C1 nor*al and C' low ). Show +<plaination + 1$ $ ,hat is the *ost co**on ocular 6ndin& in this patient with this &ranulo*atous disease. 1. Blepharitis $. Acute anterior u3eitis %. Posterior u3eitis '. Keratoconus (. Cataracts ). Show +<plaination B 1$ % Sou prescri#e do<ycyline to a $$ year"old wo*an with acne. Sour patient takes an oral contracepti3e to pre3ent pre&nancy. Sour patient read in a *a&a;ine that the do<ycycline *ay decrease the eTcacy of her contracepti3e: and asks you a#out this. ,hich of the followin& anti#iotics has #een de6niti3ely shown to reduce contracepti3e eTcacy. 1. /etracycline $. Minocycline %. A;ithro*ycin '. /MP"SMO (. Hifa*pin ). Show +<plaination + 1$ ' 1**unolo&ic a#nor*alities in atopic der*atitis include: 1. 1ncreased C0C /"cell nu*#er and function $. 1ncreased secretion of 12E"&a**a %. 0ecreased e<pression of C0$% on B cells and *onocytes '. 1ncreased secretion of 18"' (. Accentuated 0/F response ). Show +<plaination 0 1$ ( ,hat or&anis* caused this disease. 1. Micrococcus sedenditaris $. Candida al#icans %. Staphylococcus aureus '. Coryne#acteriu* tenuis (. Coryne#acteriu* diptheria ). Show +<plaination A 1$ ) Most co**on association with &enerali;ed lesions shown in attached i*a&e is 1. Fod&kinGs disease $. Eon"Fod&kinGs ly*pho*a %. 5ranulo*atous *ycosis fun&oides '. F1? infection (. 0ia#etes *ellitus ). Show +<plaination + 1$ A ,hich of the followin& is E9/ true re&ardin& pri*ary cutaneous a*yloidosis. 1. AA is not the protein co*ponent $. A*yloid is present around #lood 3essels %. A8 is not the protein co*ponent '. Eotal&ia paresthetica *ay #e associated with the *acular for* (. A*yloid found in #eni&n and *ali&nant neoplas*s does not represent pri*ary cutaneous a*yloidosis ). Show +<plaination B 1$ C ,hich contact aller&en is found in Kra;y &lue. 1. 2or*aldehyde $. 8anolin alchohol %. +thyl cyanoacrylate '. 9ctyl"di*ethyl"pa#a (. Mercapto#en;othia;ole ). Show +<plaination C 1$ D A 'C year old wo*an was recently dia&nosed with der*ato*yositis. ,hich e<a*ination would #e *ost i*portant in a work up for *ali&nancy. 1. Breast e<a* $. Chest <ray %. /hyroid e<a* '. Pel3ic e<a* (. Stool &uiac ). Show +<plaination 0 1% ! /he diMerential dia&nosis for the attached i*a&e should include all of the followin&s e<cept 1. Candidiasis $. 8ichen planus %. Contact der*atitis '. 1n3erse psoriasis (. +rythras*a ). Show +<plaination + 1% 1 After #ein& treated for se3eral *onths with do<ycycline: this patient de3elops a &ra* ne&ati3e folliculitis. ,hat is the ne<t appropriate therapy. 1. /etracycline $. Bactri* %. 1sotretinoin '. Ceftria<one (. Cefepi*e C C1 ). Show +<plaination 1% $ A '$ year"old wo*an has the 6ndin&s pictured: as well as erythe*atous patches on the upper chest and shoulders. Eo HaynaudGs: polyarthritis: pul*onary: or cardiac in3ol3e*ent is noted. ,hich anti#ody *i&ht you e<pect to #e present in this patient. 1. Anti"SHB $. Anti"7o"1 %. Anti"Mi"$ '. Anti"Ku (. Anti"8a ). Show +<plaination C 1% % Secondary syste*ic a*yloidosis: 1. Classically in3ol3es the ton&ue and perior#ital skin $. 1n3ol3es deposition of A8 protein %. Can in3ol3e deposition of #eta $" *icro&lo#ulin in the settin& of rheu*atoid arthritis '. Can #e noted on #iopsy of nor*al skin (. 1n3ol3es deposition of keratin"deri3ed a*yloid ). Show +<plaination 0 1% ' MA51C syndro*e in3ol3es: 1. Helapsin& polychondritis $. Acne con&lo#ata %. 8i3edo reticularis '. 8ipodystrophy (. Psoriatic arthritis ). Show +<plaination A 1% ( ,hat is the dia&nosis. 1. Psoriasis $. 8ichen planus %. Balanitis circinata '. Candida (. Syphilis ). Show +<plaination C 1% ) A patient has aller&ic contact der*atitis to paraphenylenedia*ine. ,hich of the followin& aller&ens *ay show a potential cross" reaction. 1. Adhesi3e $. Sulfa dru&s %. 8idocaine '. 2ra&rance (. Eickel ). Show +<plaination B 1% A A '! year"old *an presents with a co*plaint of nail chan&es for se3eral years. Fe was treated #y an outside physician with ter#ina6ne without i*pro3e*ent. 9n further Luestionin&: he reports *ornin& shoulder stiMness and #ack pain that lasts 1"$ hours and i*pro3es with acti3ity. ,hich of the followin& is true re&ardin& his condition. 1. (!N of patients present with 4oint disease prior to skin in3ol3e*ent $. Bony erosions are not co**only seen on radio&raphs %. Cyclosporine is eMecti3e in treatin& the arthritis in this condition '. A positi3e rheu*atoid factor *ay #e seen (. 7oint disease correlates with se3erity of skin in3ol3e*ent ). Show +<plaination 0 1% C Oantho*a striata pal*aris are dia&nostic of: 1. 2a*ilial hypertri&lyceride*ia =type 1?> $. 2a*ilial dys#etalipoproteine*ia =type 111> %. 2a*ilial lipoprotein lipase de6ciency =AH> '. 2a*ilial lipoprotein lipase de6ciency =A0> (. Apoprotein C11 de6ciency ). Show +<plaination B 1% D A patient presents with recurrent crops of papules that ulcerate and then spontaneously heal. ,hat i**unohistoche*ical stain would #e helpful in *akin& the dian&osis. 1. C0 ' $. C0 $! %. C0 %! '. C0 () (. C0 )C ). Show +<plaination C 1' ! Perior#ital purpura is a characteristic cutaneous *anifestation of: 1. Sarcoidosis $. A*yloidosis %. 0er*ato*yositis '. 8y*pho*a (. /hro*#ocytopenia ). Show +<plaination B 1' 1 ,hich of the followin& disorders is *ost stron&ly associated with a *onoclonal &a**opathy of the 1&5"la*#da type. 1. Sclerede*a $. P9+MS syndro*e %. Eecro#iotic <antho&ranulo*a '. ++0 (. Sclero*y<ede*a ). Show +<plaination + 1' $ /he *ost i*portant *ediator of retinoid acti3ity in the skin is: 1. HAH"alpha $. HAH"#eta C C$ %. HAH"&a**a '. HOH"alpha (. HOH"#eta ). Show +<plaination 1' % ,hich is the *ost co**on site for a#do*inal carcinoid tu*ors causin& the classic carcinoid syndro*e. 1. 1leu* $. Appendi< %. Hectu* '. Bronchus (. 8i3er ). Show +<plaination B 1' ' ,hat is the *ost co**on paraproteine*ia in sclero*y<ede*a 1. 1&A $. 1&5 kappa %. 1&5 la*#da '. 1&M (. 1t is unco**on to see paraproteine*ia with sclero*y<ede*a ). Show +<plaination C 1' ( ,hat co*plication can occur if this disease is left untreated. 1. 0e3elop*ent of a sLua*ous cell carcino*a $. /esticular torsion %. +rectile dysfunction '. 1*potence (. Pseudo"ainhu* ). Show +<plaination A 1' ) /his wo*an is #ein& treated for corticosteroid" induced rosacea with topical *etronida;ole and an oral tetracycline. She is 3ery concerned a#out the redness of her face. 7udicious use of what color concealer can reduce the appearance of redness on the skin. 1. Sellow $. 8a3ender %. Pink '. Bron;e (. 5reen ). Show +<plaination + 1' A An elderly &entle*an with rheu*atoid arthritis has a $"*onth history of recurrent painful: red: swollen ears and hearin& loss. Physical e<a* is nota#le for sparin& of the earlo#es. Sou suspect he has: 1. Chondroder*atitis nodularis helices $. Hecurrent otitis e<terna %. Helapsin& polychondritis '. Syste*ic lupus erthe*atosus (. Se3ere se#orrheic der*atitis ). Show +<plaination C 1' C Perifolicular depi&*entation is a characteristic cutaneous *anifestation of: 1. 0er*ato*yositis $. Secondary syphilis %. Scleroder*a '. 0iscoid lupus (. Sarcoidosis ). Show +<plaination C 1' D Pyoder*a &an&renosu* is *ost co**only reported with which *ali&nancy. 1. 8euke*ia $. Medullary thyroid carcino*a %. 8un& carcino*a '. Breast carcino*a (. Prostate carcino*a ). Show +<plaination A 1( ! 2irst line therapy for su#corneal pustular der*atosis is: 1. 0apsone $. /etracycline %. ?ita*in + '. /opical retinoids (. /opical steroids ). Show +<plaination A 1( 1 Syndro*e that is associated with disease shown in i*a&e is known as 1. SAPF9 syndro*e $. 2ollicular occlusion tetrad syndro*e %. 8+9PAH0 syndro*e '. EAM+ syndro*e (. PAPA syndro*e ). Show +<plaination + 1( $ A patient with this cutanous 6ndin& alon& with peri"ocular erythe*a: deltoid weakness and pul*onary disease is *ost likely to ha3e which la#oratory 6ndin&. 1. Anti"7o"1 anti#odies a&ainst histidyl"tHEA synthetase $. Anti"7o"1 anti#odies a&ainst nuclear helicase %. c"AECA a&ainst proteinase"% '. 8upus anticoa&ulant (. Anti"histone anti#odies ). Show +<plaination A 1( % ,hich of the followin& is associated with this scarrin& condition. 1. /hyroid disease $. Acne con&lo#ata %. Sarcoidosis '. Syste*ic lupus erythe*atosus (. 8ichen planus B C% ). Show +<plaination 1( ' Attached i*a&e can #e associated with all of the followin&s e<cpet: 1. S*ooth *uscle ha*arto*a $. Bnilateral #reast hypoplasia %. Acnefor* lesion '. Skeletal defect (. Cardiac defect ). Show +<plaination + 1( ( Androstenedione is produced #y: 1. /he adrenals $. /he o3aries %. /he adrenals and o3aries '. +<tra&landular con3ersion (. /he adrenals: o3aries: and e<tra&landular con3ersion ). Show +<plaination C 1( ) Patients with 0er*atitis Ferpetifor*is are *ost likely to ha3e: 1. Anti#odies to BPA&$ $. Anti#odies to trans&luta*inase % %. Mutations in trans&luta*inase 1 '. Mutations in la*inin ( (. Mutations in plectin ). Show +<plaination B 1( A /his radio&raph shows a characteristic 6ndin& of se3ere psoriatic arthritis: #one proliferation at the #ase of the distal phalan< with resorption of the tufts. ,hat is the *ost co**on for* of psoriatic arthritis. 1. Arthritis *utilans $. A<ial %. Sy**etric polyarthritis '. Asy**etric oli&oarthritis (. Sy**etric oli&oarthritis ). Show +<plaination 0 1( C /opical calcineurin inhi#itors *ay oMer se3eral #ene6ts o3er topical steroids in treatin& the scaly: licheni6ed perior#ital plaLues in this patient with atopic der*atitis: includin&: 1. More cost eMecti3e $. Quicker onset of action %. 0ecreased irritant potential '. A#sence of reports of cutaneous atrophy (. 9nce daily application ). Show +<plaination 0 1( D A % year"old &irl has a red"yellow papule on her cheek which her *other is e<tre*ely an<ious a#out. Skin #iopsy re3eals a 4u3enile <antho&ranulo*a. ,hich specialist should you refer the patient to. 1. Plastic sur&eon $. +ndocrinolo&ist %. Eeurolo&ist '. 9phthal*olo&ist (. 9ncolo&ist ). Show +<plaination 0 1) ! A patient presents with he*orrha&ic onycholysis. /he dru& class *ost co**only associated with this 6ndin& is: 1. Quinolone anti#iotics $. Syste*ic retinoids %. Calcineurin inhi#itors '. /a<anes (. /etracyclines ). Show +<plaination 0 1) 1 A %( year"old otherwise healthy *an presents with *oderate"to"se3ere plaLue psoriasis: i*pro3ed on cyclosporine. /he patient wei&hs A! kilo&ra*s and is takin& a dose of %!! *&day. Fis #aseline creatinine was !.Cc on follow"up testin& it is 1.1. All other e<a* and la#oratory para*eters are within nor*al li*its. /he patient is pleased with his treat*ent and asks to continue it. ,hich of the followin& is correct. 1. /he patient has e<ceeded the reco**eded dosa&e of cyclosporine $. Sou oMer a decrease of the cyclosporine dose to $$( *&day and close follow"up %. /he chan&e in creatinine is not si&ni6cant: no chan&e is needed '. Cyclosporine rarely has renal to<icity in youn&: healthy indi3idualsc thus you *ust work"up other causes of the increased creatinine (. /he cyclosporine should #e stopped i**ediatelyc the patient *ust a3oid cyclosporine in the future ). Show +<plaination B 1) $ ,hich of the followin& is true re&ardin& this dia&nosis. 1. /he protein deposited is deri3ed fro* 1& li&ht chains: kappa su#type $. Skin is in3ol3ed in d1!N of cases %. A diMerent protein is found in skin lesions associated with a plas*acyto*a '. Bullae: when present: are su#epider*al (. 2actor ? de6ciencies are co**only associated with this entity ). Show +<plaination 0 1) % /his (A"year old *ale co*plains of weakness when cli*#in& the stairs and this clinical presentation. ,hich cytokine has #een i*nplicated in the etiolo&y of this condition. 1. 18"1 $. 1l"1! %. 1nterferon"alpha '. 1nterferon"&a**a (. /u*or necrosis factor alpha + C' ). Show +<plaination 1) ' A %$ year old wo*an: now 1$ weeks pre&nant: presents to your oTce with pruritic scaly papules and plaLues. A #iopsy re3eals focal spon&iosis and parakeratosis in *ounds: a super6cial peri3ascular der*atitis: and e<tra3asated red #lood cells in the der*is. ,hich of the followin& is true. 1. 1t has #een associated with +B?. $. /here is often a -are post"partu* and durin& su#seLuent pre&nancies. %. /here is no increased incidence in i**unoco*pro*ised patients. '. /here is an increased risk of *iscarria&e in *others who de3eloped pityriasis rosea within the 6rst $) weeks of their pre&nancy (. Acyclo3ir *ay #e eMecti3e in this condition ). Show +<plaination + 1) ( ,hich syste*ic anesthetic can #e safely used in *astocytosis. 1. 8idocaine $. Succinylcholine %. 0"tu#ocurarine '. /hiopental (. Propofol ). Show +<plaination + 1) ) Phrynoder*a is associated with de6ciency of which 3ita*in. 1. ?ita*in A $. ?ita*in B1 %. ?ita*in B) '. ?ita*in 0 (. ?ita*in K ). Show +<plaination A 1) A ,hat type of a*yloid is deposited into the skin of this pruritic disorder. 1. A*yloid AA $. A*yloid A8 %. Keratin deri3ed '. Beta"$ *icro&lo#ulin (. /ransthyretin ). Show +<plaination C 1) C ,hich of the followin& *edications is *ost likely to induce or e<acer#ate psoriasis in your patient. 1. Fydrochlorothia;ide $. 8isinopril %. A*lodipine '. Metoprolol (. 0iltia;e* ). Show +<plaination 0 1) D /his 1! year"old &irl presents to your oTce. /hese lesions ha3e #een present for *onths. She denies any other syste*ic co*plaints. ,hat will you tell her parents. 1. /hey should e<pect her to &et *ore lesions in none sun"e<posed areas $. She is *ore likely to de3elop syste*ic lupus erythe*atosus than an adult with these lesions %. She is less likely than an adult to de3elop renal disease '. She is *ore likely than an adult to de3elop cardio3ascular disease (. Eo he*atolo&ic la#s are reLuired for e3aluation ). Show +<plaination B 1A ! ,hich of the followin&s is true a#out ne3us ane*icus 1. Bsually occurs in association with 3itili&o $. 9ccurs *ore freLuently in *en than in wo*en %. Most co**only in3ol3es the upper chest '. Hesults fro* locally decreased 3ascular reacti3ity to catechola*ines (. Contains dilated #lood 3essels ). Show +<plaination C 1A 1 Best treat*ent option for this sta#le type of 3itili&o is 1. Phototherapy with narrow #and B?B $. +<ci*er laser %. 9ral prednisone '. $!N *ono#en;yl ether of hydroLuinone (. Eitro&en *ustard ). Show +<plaination 0 1A $ A pre&nant wo*an presents with *ild in-a**atory acne which is 3ery #otherso*e to her. ,hich of the followin& is the *ost appropriate treat*ent option #ased on 20A classi6cations of *edication in pre&nancy. 1. /opical erythro*ycin#en;oyl pero<ide &el $. /opical tretinoin %. /a;arotene !.1N crea* '. A;elaic acid $!N crea* (. Bactri* ). Show +<plaination 0 1A % Sarcoidosis presentin& as u3eitis: facial ner3e palsy: fe3er and parotid &land swellin& is referred to as: 1. FeerfordtGs syndro*e $. 8of&renGs syndro*e %. 8upus pernio '. 0arier"Houssy disease (. Schau*ann syndro*e A C( ). Show +<plaination 1A ' Carcino*a of which of the followin& has #een *ost associated with erythe*a &yratu* repens 1. 8un& cancer $. Breast cancer %. Colon cancer '. Prostate cancer (. Bpper 51 tract cancer ). Show +<plaination A 1A ( Sneddon",ilkinson 0isease: 1. Most often occurs in elderly wo*en $. Harely in3ol3es intertri&inous areas %. May occur in association with an 1&5 *onoclonal &a**opathy '. Can #e treated with narrow #and B?B (. 1s usually an acute: self"li*ited condition ). Show +<plaination 0 1A ) A youn& wo*an presents with tender: erythe*atous nodules an the anterior lower e<tre*ities. ,hich of the followin& would not #e an appropriate test to consider. 1. /SF $. +SH %. AS9 '. 2un&al cultures (. PP0 ). Show +<plaination A 1A A Cutaneous *anifestations of 3ita*in 0 de6ciency include: 1. Alopecia $. 2ollicular hyperkeratosis %. +de*a '. An&ular cheilitis (. Atrophic &lossitis ). Show +<plaination A 1A C ,hich of the followin& der*atoses occurs at the latest sta&e of pre&nancy. 1. 0arkenin& of ne3i $. 8inea ni&ra %. Melas*a '. Areolar hyperpi&*entation (. Psoriasis e<acer#ation ). Show +<plaination B 1A D A co**on site for chloracne is the: 1. 9ccipital scalp $. 2orehead %. Scrotu* '. 2orear*s (. Shoulders ). Show +<plaination C 1C ! Acute he*orrha&ic ede*a of childhood often presents initially with: 1. 2acial ede*a $. 8aryn&ospas* %. Acute a#do*en '. Fe*aturia (. Fe*atoche;ia ). Show +<plaination A 1C 1 Eeutrophilic der*atoses en plaLue is often associated with which of the followin&. 1. 1&A *onoclonal &a**opathy and a #eni&n course $. 1&A *onoclonal &a**opathy and a *ali&nant course %. Myelo*a and B ly*pho*as and a #eni&n course '. 1&5 *onoclonal &a**opathy and a #eni&n course (. 1&5 *onoclonal &a**opathy and a *ali&nant course ). Show +<plaination A 1C $ ,hich of the followin& is i*portant in the patho&enesis of acne 3ul&aris. 1. Acti3ation of toll"like receptor"% #y P. acnes $. Acti3ation of toll"like receptor"$ #y M. furfur %. Acti3ation of toll"like receptor"$ #y P. acnes '. P. acnes produces lipase which clea3es cholesterol into tri&lycerides (. 0e*ode< acti3ates co*ple*ent ). Show +<plaination C 1C % A %( year"old wo*an presents with predo*inantly lower facial in-a**atory acne: hirsutis*: and irre&ular *enses. She has failed *ultiple con3entional treat*ents. 8a#oratory work"up re3eals a 3ery hi&h le3el of 0F+A"S. Sou are *ost concerned a#out: 1. Con&enital adrenal hyperplasia $. Adrenal tu*or %. Polycystic o3ary syndro*e '. /o<icity fro* prolon&ed use of spironolactone (. Pro&estin e<cess fro* oral contracepti3es ). Show +<plaination B 1C ' ,hich of the followin& cytokines is associated with follicular plu&&in& and *icroco*edo for*ation. 1. 18"' $. 18"1% %. /E2"e 0 C) '. 18"1 (. /52"ooo ). Show +<plaination 1C ( Acneifor* eruptions ha3e #een associated with which of the followin& 3ita*ins. 1. ?ita*in C $. ?ita*in + %. ?ita*in A '. Biotin (. ?ita*in B1$ ). Show +<plaination + 1C ) 9ne of your acne patients has #een treated with do<ycycline for se3eral *onths and de3elops culture positi3e &ra* ne&ati3e folliculitis. ,hat is the ne<t appropriate therapy 1. /etracycline $. Bactri* %. 1sotretinoin '. Ceftria<one (. Cefepi*e ). Show +<plaination C 1C A ,hich of the followin& is a pro&estin used in oral contracepti3es with low intrinsic andro&enic properties. 1. Eor&estrel $. Eorethindrone %. Eor&esti*ate '. 8e3onor&estrel (. Spironolactone ). Show +<plaination C 1C C A youn& &irl presents with recurrent se3ere arthritis of the ankles. She also has a lar&e ulcer on her le& and se3ere acne. ,hich &ene is *utated. 1. PS/P1P1 $. E90$ %. C1AS1 '. A1H+ (. 29OP% ). Show +<plaination A 1C D ,hich of the followin& is true re&ardin& Seip" 8awrence Syndro*e. 1. /his condition is acLuired $. Mentalco&niti3e function is unaMected %. /hyroid function a#nor*alities cause heat intolerance '. Fypertrichosis is often a feature (. Basic *eta#olic rate is depressed ). Show +<plaination 0 1D ! 1n a well"desi&ned study: the i*pact of psoriasis on health"related Luality of life was found to #e si*ilar to which of the followin& conditions. 1. 0ia#etes $. Acne 3ul&aris %. 9nycho*ycosis '. Seasonal aller&ic rhinitis (. Fypercholesterole*ia ). Show +<plaination A 1D 1 /he *ost co**on la#oratory a#nor*ality in patients treated with isotretinoin is: 1. 0ecreased white #lood cell count $. 1ncreased cholesterol %. +le3ated li3er en;y*es '. Fypertri&lyceride*ia (. +le3ated CPK ). Show +<plaination 0 1D $ AC+ inhi#itors cause an&ioede*a 3ia sti*ulation of. 1. Bradykinins $. Fista*ine %. Prosta&landins '. 8eukotrienes (. Co*ple*ent ). Show +<plaination A 1D % ,hat na*e is co**only used to descri#e this condition of nodular elastosis with cysts and co*edones. 1. Actinic &ranulo*a $. Chlorance %. Cutis rho*#oidalis nuchae '. Colloid *iliu* (. 2a3re"Hacouchot ). Show +<plaination + 1D ' ,hich auto"anti#ody correlates with this 6ndin&: shawl si&n and an o3erall fa3ora#le pro&nosis in 0er*ato*yositis. 1. Anti"Mi"$ anti#ody $. Anti"7o"1 anti#ody %. Anti"SHB anti#ody '. Anti"KB anti#ody (. Anti"P8A anti#ody ). Show +<plaination A 1D ( A %$ year"old pre&nant wo*an presents for treat*ent co*edonal and *oderately in-a**atory acne. Sou discuss treat*ent options with her: and she asks a#out the e3idence for safety in pre&nancy of 3arious treat*ents. Accordin& to the 20A classi6cations: which of the followin& treat*ents either shows 1. Ben;oyl pero<ide $. /ri*ethopri*"sulfa*etho<a;ole %. /opical tretinoin '. A;eleic acid (. /etracycline ). Show +<plaination 0 CA no risk to the fetus in controlled studies =#ut *ay show risk to ani*als>: or shows no risk in ani*al studies =#ut no hu*an studies ha3e #een conducted>. 1D ) ,hat condition does this patient ha3e &i3en the e<tent of arthritis seen in this O"ray. 1. Antiphospholipid syndro*e $. Multicentric reticulohistiocytosis %. Alpha"1"antitrypsin syndro*e '. +osinophilic fasciitis (. 0er*ato*yositis ). Show +<plaination B 1D A A pre&nant wo*an presents with the followin& condition which is #otherso*e to her. ,hich of the followin& is the *ost appropriate treat*ent option #ased on 20A classi6cations of *edication in pre&nancy. 1. /opical erythro*ycin#en;oyl pero<ide &el $. /opical tretinoin %. /a;arotene !.1N crea* '. A;elaic acid $!N crea* (. Bactri* 0S ). Show +<plaination 0 1D C /he presentation of a fore&ut carcinoid tu*or in3ol3es: 1. /he production of hista*ine: cyanotic -ush: and #ronchoconstriction $. /he production of serotonin: intense -ushin&: peptic ulcer: and lacri*ation %. /o cutaneous 6ndin&s '. /he production of kallikrein with #ronchial asth*a and an&ioede*a (. 2reLuent episodes of ton&ue swellin& and urticaria ). Show +<plaination B 1D D ,hat is the *a4or constituent of se#aceous &land lipid. 1. /ri&lyceride $. ,a< esters %. SLualene '. 2ree cholesterol (. Cholesterol esters ). Show +<plaination A $! ! 8eukokeratosis nicotina palati: 1. 1s not attri#uted to s*okin& $. Hepresents a pre*ali&nant condition %. Hesponds to intralesional steroids '. Hepresents in-a*ed *inor sali3ary &lands (. Hesol3es with tetracycline therapy ). Show +<plaination 0 $! 1 A '! year old wo*en presents to the clinic with *ultiple pustules in annular and serpi&inous patterns on the a#do*en: a<illae and &roin. Fistopatholo&y re3eals pustules #elow the stratu* corneu* with *any neutrophils and without any acantholysis. ,hat is the dia&nosis. 1. Sneddon",ilkinson disease $. HeiterGs syndro*e %. SAPF9 syndro*e '. /ransient pustular *elanosis (. Pthirus pu#is ). Show +<plaination A $! $ ,hich of the followin& for*s of an&ioede*a is *ediated #y kinin release. 1. Fereditary an&ioede*a $. Acute aller&ic an&ioede*a %. An&ioede*a associated with urticarial 3asculitis '. ESA10 an&ioede*a (. 1nfectious an&ioede*a ). Show +<plaination A $! % /he 6rst step in the for*ation of a closed co*edone is: 1. 9#struction of the pilose#aceous duct $. 1ncreased se#u* production %. Coloni;ation #y P. acnes '. 1ncreased hor*onal sti*ulation of the pilose#aceous unit (. Clo&&in& of the pore with *ake"up ). Show +<plaination A $! ' 1**unohistoche*istry of this disease would likely show: 1. Peri3ascular 1&A $. 1&M stainin& of colloid #odies %. 8inear #and of 1&A at the #ase*ent *e*#rane '. 1&M intercellularly (. 5ranular C% at the #ase*ent *e*#rane ). Show +<plaination B $! ( /he I-a& si&nI is seen in which of the followin& conditions. 1. psoriasis $. sarcoidosis %. hidradenitis suppurati3a '. kwashiorkor (. telo&en eRu3iu* ). Show +<plaination 0 $! /he hyperproliferati3e epitheliu* of this *ature 1. K1: K1! C CC ) psoriasis plaLue is associated with increased e<pression of which keratin=s>. $. K(: K1' %. K): K1) '. K1A (. K$e ). Show +<plaination $! A ,hich of the followin& is characteristic of lichen planus pi&*entosus. 1. 8esions are typically hypopi&*ented $. Most cases present in Caucasians %. 9ral in3ol3e*ent is patho&no*onic '. Can occasionally see epider*otropic /"cells (. /runk is typically spared ). Show +<plaination 0 $! C ,hat is the treat*ent of choice for this condition. 1. Eystatin $. 2lucona;ole %. Better oral hy&iene '. Penicillin (. Acyclo3ir ). Show +<plaination C $! D A $$ year"old wo*an notes irre&ular *enses and acne that has #een refractory to se3eral o3er" the"counter and prescription re&i*ens. Appropriate initial work"up includes 1. Seru* prolactin le3els $. $'"hour urine cortisol %. Seru* free and total testosterone: 8F: 2SF: androstenedione '. Seru* free and total testosterone: 8F: 2SF: 0F+A"S (. Seru* free and total testosterone: 8F: 2SF: 1A"9F: hydro<ylase ). Show +<plaination 0 $1 ! A pre*ature infant who is #ein& weaned oM #reast *ilk de3elops 3esico#ullous and ec;e*atous skin lesions and diarrhea. ,hich of the followin& is not another classic precipitant for this condition 1. Parenteral nutrition $. Stress =i.e. infection> %. 0iets with *ainly cereal &rains '. 8i3er disease (. Alcoholis* ). Show +<plaination 0 $1 1 /he dia&nosis is: 1. 9nycho*ycosis $. /rau*a"induced nail chan&es %. Chronic paronychia '. Pseudo*onal infection (. Psoriasis arthritis ). Show +<plaination + $1 $ ,hich of the followin& is true re&ardin& relapsin& polychondritis. 1. 1n3ol3e*ent is often #ilateral $. Patho&enic anti#odies ha3e not yet #een identi6ed %. /he course is chronic: yet *ortality is low '. Both se<es are eLually aMected (. Mi&ratory arthral&ias are unco**only seen ). Show +<plaination 0 $1 % Patients of which F8A type are *ore prone to dru&"induced lupus erythe*atosus when e<posed to hydrala;ine. 1. F8A"Cw) $. F8A"BA %. F8A"0H% '. F8A"0H' (. F8A"B) ). Show +<plaination 0 $1 ' Melanocytes can #e found in all of the followin& e<cept: 1. Ee3us depi&*entosa $. /yrosinase positi3e al#inis* %. Ee3us ane*icus '. ?itili&o (. Postin-a**atory hypopi&*entation ). Show +<plaination 0 $1 ( ,hich of the followin& is a *anifestation of psoriasis of the nail *atri<. 1. Splinter he*orrha&es $. 9il spots %. Su#un&al hyperkeratosis '. 9nycholysis (. Pits ). Show +<plaination + $1 ) ,hich of the followin& is true re&ardin& syndro*es associated with a*yloidosis. 1. 2a*ilial *editerranean fe3er in3ol3es A8 protein $. M+E 11a in3ol3es AA protein %. /he syndro*e that presents with renal a*yloidosis: fe3ers: li*# pains: and deafness in3ol3es AA protein '. 2a*ilial a*yloidotic polyneuropathy type 1? in3ol3es *utations in apolipoprotein A"1 (. 2a*ilial a*yloidotic polyneuropathy type 111 C CD in3ol3es *utations in &elsolin ). Show +<plaination $1 A A healthy (! year old *an presents to your oTce with % red"#rown indurated plaLues on his forehead. Biopsy re3eals a focal leukocytoclastic 3asculitis: with a diMuse in6ltration of neutrophils: eosinophils: and ly*phocytes. ,hich of the followin& is a potential treat*ent option for this patient. 1. Q"switched ale<andrite laser $. Pulse dye laser %. Q"switched ru#y laser '. Ed:SA5 laser (. 2reLuency"dou#led Ed:SA5 laser ). Show +<plaination B $1 C ,hich of the followin& is true re&ardin& atopic der*atitis. 1. 93er C!N of aMected indi3iduals present in the 6rst year of life $. Eatural *easles infection has #een noted to i*pro3e atopic der*atitis %. Most children de3elop worsenin& of atopic der*atitis if they de3elop asth*a later in childhood '. Staphylococcus aureus is found in _$(N of atopic der*atitis skin lesions (. 1ncreased e<pression of cathelicidins such as 88 %A has #een noted in atopic der*atitis ). Show +<plaination B $1 D ,hich of the followin& *edications would likely e<acer#ate your patientGs psoriasis. 1. Fydrochlorothia;ide $. 8isinopril %. A*lodipine '. Metroprolol (. 0iltia;e* ). Show +<plaination 0 $$ ! 1n the settin& of the lupus erythe*atosus" associated co*ple*ent de6ciency syndro*e: which of the followin& would *ost often #e o#ser3ed. 1. low C% and C' $. low C$ and C% %. low C$ and C' '. low C$ only (. low C% only ). Show +<plaination C $$ 1 ,hich &enoder*atosis *ay #e associated with this plaLue of skin #etween the shoulder #lades with rippled hyperpi&*ented appearance. 1. Cowden syndro*e $. Peut;"7e&hers syndro*e %. 5ardner syndro*e '. Sipple syndro*e (. 0yskeratosis Con&entia ). Show +<plaination 0 $$ $ Pyosto*atitis 3e&etans is characteristically associated with which syste*ic disease. 1. Pe*phi&us 3ul&aris $. Blcerati3e colitis %. 8ichen planus '. 8y*pho*a (. Hheu*atoid arthritis ). Show +<plaination B $$ % Patients with <antho*a disse*inaturn *ay de*onstrate: 1. +le3ated seru* #eta lipoproteins $. Sparin& of the oral *ucosa %. 1n3ol3e*ent of the pituitary &land '. Sparin& of the -e<ural areas (. 1ncreased risk of *ali&nant de&eneration ). Show +<plaination C $$ ' /he protein co*ponent of pri*ary cutaneous a*yloidosis is: 1. SAA protein $. A8 protein %. Keratin '. Colla&en (. Bp1C! ). Show +<plaination C $$ ( ,hich i**uno&lo#ulins are seen in type 111 cryo&lo#uline*ia. 1. Monoclonal 1&5 and *onoclonal 1&M $. Monoclonal 1&5 or *onoclonal 1&M %. Monoclonal 1&M and polyclonal 1&5 '. Polyclonal 1&M and *onoclonal 1&5 (. Polyclonal 1&M and polyclonal 1&5 ). Show +<plaination + $$ ) ,hat is deposited in the upper der*is in this condition. 1. AK $. A8 %. /ransthyretin '. Beta$"*icro&lo#ulin (. AA ). Show +<plaination A $$ A /he *ost appropriate treat*ent for the condition shown is: 1. /opical clinda*ycin 1N lotion $. /opical *etronida;ole !.A(N &el %. A;elaic acid $!N crea* + D! '. Salicylic acid $N wash (. Adapalene !.1N &el ). Show +<plaination $$ C Most co**on cause of these tender lesions on this 1$"year old child is 1. 1n-a**atory #owel disease $. /u#erculosis %. f#eta"he*olytic streptococcal infection '. Sersinia (. 1nfectious *ononucleosis ). Show +<plaination C $$ D ,hat is the *ost co**on 3ariant of *orphea in children. 1. PlaLue $. 5enerali;ed %. Bullous '. 0eep =*orphea profunda> (. 8inear ). Show +<plaination + $% ! /he *ost co**on cause of internal *anifestation of scleroder*a is: 1. +sopha&eal dys*otility $. Sepsis %. Pul*onary hypertension '. Acute renal failure (. HaynaudGs pheno*enon ). Show +<plaination A $% 1 Sarcoidosis presentin& with fe3er: cou&h: 4oint pains: hilar adenopathy and erythe*a nodosu* is known as: 1. +rythe*a contusifor*e $. 8oeRerGs syndro*e %. 8of&renGs syndro*e '. 0arier"Houssy sarcoid (. FeerfordtGs syndro*e ). Show +<plaination C $% $ A youn& *an presents with e<plosi3e onset of se3ere cystic acne with acute: suppurati3e nodules and plaLues that ulcerate and for* a #lackish eschar on the trunk as well as the face. ,hich of the followin& is true re&ardin& this entity. 1. ,o*en are *ore often aMected than *en $. P. acnes osteo*yelitis presents with lytic chan&es on <"rays and #one scans %. /he sternocla3icular 4oint is often in3ol3ed in this entity '. Syste*ic corticosteroids are contraindicated &i3en risk of sepsis (. Fi&h"dose isotretinoin *onotherapy is the treat*ent of choice ). Show +<plaination C $% % Sou are consulted to e3aluate this patient with tender: war* plaLues on his shins. ,hat other physical si&ns should you look for. 1. +<ophthal*os $. B3eitis %. /achycardia '. Clu##in& of the 6n&ers (. Cou&h ). Show +<plaination B $% ' ,hich autoanti#odies are associated with an increased risk of *ali&nancy in der*ato*yositis. 1. anti"SHP $. anti"Mi$ %. Anti"7o1 '. anti"1((1'! (. anti"P8"A ). Show +<plaination 0 $% ( Eatural infection with which of the followin& infectious a&ents has #een shown to *iti&ate atopic der*atitis. 1. Measles $. S. aureus %. ?aricella '. Hu#ella (. Co<sackie 3irus ). Show +<plaination A $% ) 0iarrhea: 0e*entia and a photosensiti3e der*atitis are associated with a de6ciency of which 3ita*in. 1. Eiacin $. Biotin %. /hia*ine '. Hi#o-a3in (. Pyrido<ine ). Show +<plaination A $% A /his %( year"old *an presents with the lesions shown. Fe was treated elsewhere for a diMerent skin condition. Biopsy of these lesions is likely to show: 1. 1ncreased stainin& on 2ontana Masson #ut not Perls stain $. 1ncreased stainin& on Perls stain #ut not 2ontana Masson %. 1ncreased stainin& on #oth 2ontana Masson and Perls stain '. 1ncreased *elanin at the #asal layer and within *acropha&es only (. 2i#rosis and increased *ucin deposition ). Show +<plaination C D1 $% C /he *ost nota#le histolo&ic diMerence #etween the oral and cutaneous lesions of lichen planus is the presence in oral 8P of: 1. Acanthosis $. Parakeratosis %. Co*pact orthokeratosis '. ,ed&e"shaped hyper&ranulosis (. ?acuolar chan&es at the #asal layer ). Show +<plaination B $% D Patients with chronic idiopathic urticaria should a3oid: 1. 8ate< products $. Alcohol %. Eickel sulfate '. J"#lockers (. aspirin ). Show +<plaination + $' ! Blcerati3e colitis and crohn[s disease are *ost co**only associated with which of the followin&: 1. Pyoder*a &an&renosu* $. +rythe*a ele3atu* diutinu* %. Sweet[s syndro*e '. 1&A pe*phi&us (. Sneddon",ilkinson[s disease ). Show +<plaination C $' 1 ,hich of the followin& is E9/ associated with this disease of sy**etric induration caused *ucin deposition. 1. 0ia#etes *ellitus $. Streptococcal infection %. Monoclonal &a**opathy '. Fepatitis C (. All of the answers are associated with this disease ). Show +<plaination 0 $' $ /he *ain cause of nutritional disease in de3eloped nations is: 1. Bnusual diets $. 1n-a**atory #owel disease %. Mala#sorption syndro*es '. Alcoholis* (. Psychiatric illness ). Show +<plaination 0 $' % A % *onth old presents with a diMuse 3esiculo#ullous rash with copper colored *acules on the pal*s and soles. Sou ascertain fro* the history that his *other had a nonpainful erosion on her la#ia durin& pre&nancy which spontaneously resol3ed. ,hat other sy*pto*s would you e<pect this infant to ha3e. 1. Pseudoparalysis of Parrot $. Fi&ou*enakis si&n %. Clutton 4oints '. Mul#erry *olars (. Saddle nose defor*ity ). Show +<plaination A $' ' ,hich of the followin& is a cause of i**unolo&ic urticaria. 1. Poly*y<in B $. A*o<icillin %. 1#uprofen '. 9piates (. /artra;ine ). Show +<plaination B $' ( ,hat characteristic #ody would #e found on histopatholo&y of this disease. 1. Ka*ino $. Ci3atte %. Fenderson"Patterson '. 0ono3an (. 0utcher ). Show +<plaination B $' ) /he 4oint *ost freLuently aMected in acne ful*inans is the: 1. +l#ow $. 1nter3erte#ral %. 0istal interphalan&eal 4oints of the hand '. Sternocla3icular (. Sacroiliac ). Show +<plaination 0 $' A A )$ year"old *an with chronic renal failure on he*odialysis presents with carpal tunnel syndro*e: #one cysts: and spondyloarthropathy. A dia&nosis of a*yloidosis is suspected. ,hich of the followin& is true re&ardin& his *ost likely dia&nosis. 1. AA a*yloid is likely causati3e $. A8 a*yloid is likely causati3e %. #eta $"*icro&lo#ulin is likely causati3e '. keratin"deri3ed a*yloid is likely causati3e (. A*yloid P co*ponent will not #e found in aMected tissues ). Show +<plaination C $' C /he 0unni&an 3ariant of partial lipodystrophy is caused #y a *utation in which &ene. 1. A5PA/$ $. Seipin %. Kinc *etalloproteinase '. Eeutrophil elastase (. 8MEA ). Show +<plaination + $' D /he arthritis of BehcetGs disease is characteristically: 1. Sy**etric: erosi3e polyarthritis $. Asy**etric: erosi3e polyarthritis %. Asy**etric: non"erosi3e polyarthritis C D$ '. Asy**etric: erosi3e *onoarthritis (. Sy**etric: non"erosi3e polyarthritis ). Show +<plaination $( ! A patient recently underwent parotid sur&ery and now reports unilateral -ushin& and sweatin& around *ealti*e. /he ner3e in4ured in this syndro*e is a #ranch of the 1. 2acial ner3e $. Ma<illary ner3e %. Mandi#ular ner3e '. Cer3ical ner3e (. 2rontal ner3e ). Show +<plaination C $( 1 Monotherapy for acne with topical anti#iotics is discoura&ed #ecause of: 1. Slow onset of co*edolytic action $. Potential for irritation %. 8ack of anti"in-a**atory action '. Potential for #acterial resistance (. Poor patient co*pliance ). Show +<plaination 0 $( $ ,hich of the followin& state*ents is true re&ardin& Mor#ihanGs 0isease. 1. 1t is often *isdia&nosed as cellulitis $. 1t presents with #lepharitis: con4uncti3itis: iritis: and keratitis %. Fistopatholo&y re3eals perifollicular and peri3ascular noncaseatin& epithelioid &ranulo*as '. 1t occurs around the *outh andor nose and eyes and *ay #e tri&&ered #y topical steroid use (. 1t presents with lar&e coalescent nodules and con-uent drainin& sinuses occupyin& *ost of the face ). Show +<plaination A $( % /he *ost co**on syste*ic *anifestations of syste*ic sclerosis are: 1. 5astrointestinal $. Cardio3ascular %. Pul*onary '. Henal (. Eeurolo&ic ). Show +<plaination A $( ' ,hich or&anic syste* is least likely to #e in3ol3ed in the attached i*a&e 1. 8i3er $. Spleen %. 5enitourinary '. 5astrointestinal tract (. Bone *arrow ). Show +<plaination C $( ( /he constellation of erythe*a nodosu*: #ilateral hilar ly*phadenopathy: u3eitis: fe3er: and arthritis in patients with sarcoidosis is known as: 1. 8oeRerGs syndro*e $. Schnit;lerGs syndro*e %. 8of&renGs syndro*e '. FeerfordtGs syndro*e (. Mikulic;Gs syndro*e ). Show +<plaination C $( ) A 1) year old de3elop*entally nor*al *ale presents to his pediatrician inter*ittent 3a&ue epidodes of hand and feet paresthesias and non speci6c episode of 51 distress. Fe is referred to you to e3aluate nu*erous punctate to ( sli&htly 3errucous: deep"red to #lue"#lack papules distri#uted diMusely on his trunk in a #athin& suit distri#ution. Polari;ation *icroscopy of the sedi*ent of his urine de*onstrates #irefrin&ent lipid &lo#ules =ie: renal tu#ular epithelial cells or cell fra&*ents with lipid inclusions> with the characteristic Maltese cross con6&uration. Fow is this disorder inherited. 1. Autoso*al do*inant $. Autoso*al recessi3e %. O"linked do*inant '. O"linked recessi3e (. Eot an inherited disorder ). Show +<plaination 0 $( A ,hich of the followin& is not associated with this condition. 1. Poor oral hy&eine $. Anti#iotic use %. A&in& '. /hrush (. S*okin& ). Show +<plaination C $( C Kidney disease in Fenoch"Schonlein Purpura *ay #e predicted #y: 1. 8i*ited skin in3ol3e*ent $. Spread of purpura to the upper trunk %. Syno3ial in3ol3e*ent '. Bullous lesions (. 2acial ede*a ). Show +<plaination B $( ,hich of the followin& is a feature of SneddonGs 1. ?enous thro*#oses A D% D Syndro*e. $. Fepatospleno*e&aly %. anti"Scl"A! autoanti#odies '. M. tu#erculosis infection (. C$ de6ciency ). Show +<plaination $) ! All of the followin& are true re&ardin& HeiterGYs syndro*e e<cept: 1. /he classic clinical triad is urethritis: con4uncti3itis and arthritis $. Bsually occurs in youn& wo*en of F8A"B$A &enotype %. May #e associated with keratoder*a #elnnorrha&icu* '. May #e associated with Chla*ydia tracho*atis (. Eails *ay #eco*e thick and #rittle with hea3y su#un&ual hyperkeratotic deposits ). Show +<plaination B $) 1 K3ei*"Silt;#ack test is useful in the dia&nosis of: 1. Scarlet fe3er $. Sarcoidosis %. 8eprosy '. 8eish*aniasis (. Chancroid ). Show +<plaination B $) $ /his patient was started on isotretinoin #ut failed to discontinue the tetracycline. Fe is at risk for what co*plication. 1. Acne ful*inans $. Pseudotu*or cere#ri %. 0iarrhea '. 0epression (. Myositis ). Show +<plaination B $) % ,hat #acteria is thou&ht to possi#ly play a role in the patho&enesis of this disease. 1. B. #ur&dorferi $. F. pylori %. +. coli '. S. aureus (. S. enteritidis ). Show +<plaination A $) ' All of the followin& dru&s ha3e #een reported to cause rash si*ilar to the attached i*a&e e<cept 1. Fydro<yurea $. 0"penicilla*ine %. Statins '. Phenytoin (. Captopril ). Show +<plaination + $) ( ,hich of the followin& treat*ents for acne inhi#its HEA"dependent protein synthesis #y #indin& to the (!s ri#oso*al su#unit. 1. +rythro*ycin $. /etracycline %. /ri*ethopri*"sulfa*etho<a;le '. Ben;oyl pero<ide (. A;eleic acid ). Show +<plaination A $) ) ,hich of followin& *edications *ay increase the likelihood of pseudotu*or cere#ri in patients takin& oral isotretinoin. 1. Spironolactone $. 0apsone %. A*o<icillin '. Hifa*pin (. /etracycline ). Show +<plaination + $) A A teena&er co*es into your oTce reLuestin& laser hair re*o3al of her si&ni6cant facial hair. Before you a&ree to treat her: you order which of the followin& la#oratory analyses. 1. 2SF and 8F $. 5lucose and he*o&lo#in A1C %. 2erritin and /1BC '. /estosterone and 0F+A"S (. /SF and /% ). Show +<plaination 0 $) C /he condition shown here *ay #e e<acer#ated #y the inappropriate use of which of the followin&. 1. Co*edo&enic *ake"up foundation $. Sunscreens %. +rythro*ycin $N oint*ent '. /opical corticosteroids (. Mineral oil"containin& *oisturi;ers ). Show +<plaination 0 $) D ,hat is the *ost co**on e<tracutaneous *anifestation of SweetG Ys syndro*e. 1. Arthral&ias $. Con4uncti3itis %. Henal in3ol3e*ent '. Sterile osteo*yelitis (. 2e3er ). Show +<plaination + $A A potentially dan&erous side eMect of 1. Fypokale*ia B D' ! spironolactone is: $. Fyperkale*ia %. Fypercalce*ia '. Fyponatre*ia (. Fypernatre*ia ). Show +<plaination $A 1 ,hich of the followin& receptor is the *ost i*portant *ediator of retinoid acti3ity in the skin. 1. HAH"&a**a $. HOH"alpha %. HOH"&a**a '. HAH"#eta (. HOH"#eta ). Show +<plaination A $A $ A youn& *an treated with *inocycline de3eloped #lue"#lack discoloration with acne scars at his cheeks. A Perls stain would show: 1. 1ncreased *elanin at the #asal layer of the epider*is $. Black stainin& &ranules within *acropha&es %. Blue stainin& &ranules within *acropha&es '. JMuddy #rownJ pi&*ent &ranules (. 5iant *elanoso*es ). Show +<plaination C $A % +Tcacy of oral contracepti3es is reduced #y co" ad*inistration with which of the followin& dru&s. 1. Minocycline $. 0o<ycycline %. Hifa*pin '. /MP"SMO (. All of these answers are correct. ). Show +<plaination C $A ' /he *ost co**on location of der*ato6#rosarco*a protu#erans is: 1. /runk $. Fead and neck %. +<tre*ities '. Eone of these answers are correct (. head and neck and e<tre*ities ). Show +<plaination A $A ( ,hich of the followin& is associated with HeiterJs syndro*e: 1. Blcerati3e colitis $. Multiple sclerosis %. F8A"Cw) '. Asy**etric arthritis (. Perioral der*atitis ). Show +<plaination 0 $A ) A $C year old pre3iously healthy *an presents with thick crustin&hyperkeratosis of the pal*s and soles: urethritis: and one *onth of peripheral arthritis. ,hat of the followin& is true re&ardin& his condition 1. 2e*ales and *ales are eLually aMected $. A chronic defor*in& arthritis occurs in $!N %. /E2"alpha inhi#itin& a&ents ha3e no role in the treat*ent of this condition '. Patients *ust ha3e urethritis: con4uncti3itis: and arthritis for dia&nosis (. Cla*ydia cer3icitis is not associated with this condition ). Show +<plaination B $A A Al*ost all patients with S8+ ha3e positi3e AEAs. A patient can ha3e AEA"ne&ati3e S8+ if they only *ake anti#odies to: 1. ss0EA $. S* %. B1HEP '. Ho (. ds0EA ). Show +<plaination A $A C /he *ost co**on associated *ali&nancy with der*ato*yositis is: 1. Colon carcino*a $. Fepatocellular carcino*a %. Henal cell carcino*a '. +sopha&eal carcino*a (. 93arian carcino*a ). Show +<plaination + $A D ,hich of the followin& is a function of Propioni#acteriu* acnes =P. acnes> in contri#utin& to the patho&enesis of acne. 1. 0ownre&ulation of /8H"$ e<pression $. 1nhi#ition of co*ple*ent %. Acti3ation of /8H"$ '. 1nhi#ition of 1l"1 (. Heduction in free fatty acid le3els ). Show +<plaination C $C ! ,hich of the followin& are risk factor=s> for post" transplant C/C8. 1. Henal transplant $. 8i3er transplant %. Cyclosporine therapy '. 2e*ale Se< (. Henal transplant and Cyclosporine therapy ). Show +<plaination + $C 1 ,hich of the followin& *edications is E9/ associated with e<acer#atin& or causin& this eruption. 1. 8ithiu* $. Prednisone %. Phenytoin + D( '. 1sonia;id (. Propranolol ). Show +<plaination $C $ A %$ year"old wo*an presents with *oderate hirsutis*. She has nor*al *enses: nor*al"si;ed o3aries: no e3idence of tu*ors of the adrenal or o3ary: and nor*al adrenal function: #ut does ha3e sli&ht ele3ations of plas*a androstenedione and testosterone. ,hat is the *ost likely dia&nosis. 1. Stein"8e3enthal syndro*e $. Cushin& syndro*e %. 1diopathic hirsutis* '. 9ccult 3irili;in& tu*or (. Krucken#er& tu*or ). Show +<plaination C $C % A *other #rin&s her ' year old #oy with sil3ery #lond hair into your oTce and reports that her childGs hair cannot #e co*#ed -at. Sou notice that it has a spun"&lass appearance. ,hich of the followin& is false re&ardin& this condition. 1. Biotin has shown i*pro3e*ent in a few cases $. /here are usually no syste*ic 6ndin&s %. 8on&itudinal &roo3in& can #e seen on electron *icroscopy '. /he hair a#nor*ality tends to persist with a&e (. /he condition is autoso*al do*inant ). Show +<plaination 0 $C ' Presence of which of the followin& autoanti#odies is dia&nostic of S8+ and not reported in patients with other connecti3e tissue diseases. 1. anti"B1HEP $. anti"ds0EA %. anti"Ho '. anti"8a (. anti"S* ). Show +<plaination + $C ( 2irst line treat*ent of chro*o#lasto*ycosis includes sur&ery and a *edication with which *echanis* of action. 1. 1nhi#its sLualene epo<idase $. 1nhi#its 1'"alpha"de*ethylase %. 0isrupts *icrotu#ule *itotic spindle for*ation '. 1nhi#its sythesis of #eta"1:%"di&lucan and disrupts cell walls (. 1nhi#its fun&al cytochro*e P"'(! *ediated 1' alpha"lanosterol de*ethylation ). Show +<plaination B $C ) AcLuired an&ioede*a is characteri;ed #y: 1. 1nheritance $. 0ecreased le3els of C1 %. Self"li*ited course '. Association with underlyin& *ali&nancy (. 1ncreased C1 esterase inhi#itor ). Show +<plaination 0 $C A /his patient has si*ilar lesions on his distal e<tre*ities. ,hich la#oratory test can #e done in order to *ake a dia&nosis. 1. Fe*o&ra* $. Alkaline phosphatase %. Creatinine '. Potassiu* (. /SF ). Show +<plaination B $C C ,hich for* of sarcoidosis is associated with ca*ptodactyly. 1. 8of&renGs syndro*e $. FeerfordtGs syndro*e %. 0arier"Houssy '. Mikulic; syndro*e (. Blau syndro*e ). Show +<plaination + $C D ,hich F8A is associated with psoriatic arthritis in diseLuili#riu*: especially if spondylitis is present. 1. F8A"B1A $. F8A"B1% %. F8A"Bw(A '. F8A"Cw) (. F8A"B$A ). Show +<plaination + $D ! ,hat is the *ost co**on site of e<tracutaneous in3ol3e*ent in this non"infectious disease. 1. 8un&s $. +yes %. Bone Marrow '. 8i3er (. Easal *ucosa ). Show +<plaination A $D 1 ,hat sur&ical dressin& would you use if you wished to de#ride a wound: reduce pain: and pro3ide a coolin& eMect. 1. Al&inates $. 2oa*s %. Fydrocolloid '. Fydro&el (. 2il*s ). Show +<plaination 0 $D $ Mutations in the M+2? &ene product: pyrin: produce an autoso*al recessi3e syndro*e characteri;ed #y recurrent fe3ers: peritonitis: 1. 2a*ilial Mediterranean 2e3er $. PAPA syndro*e %. /E2 receptor associated periodic syndro*e A D) pleuritis: arthritis and erysipelas"like erythe*a. ,hich of the followin& syndro*es is descri#ed a#o3e. '. Fyper 1&0 syndro*e (. 2a*ilial cold autoin-a**atory syndro*e ). Show +<plaination $D % Pernio or Chil#lains is an in-a**atory skin condition which is tri&&ered #y cold: wet: non" free;in& en3iron*ental conditions. Acral skin has 3iolaceous discoloration acco*panied #y #urnin& or itchin&. ,hile a3oidance and pre3ention is #est: the *ost eMecti3e phar*acolo&ic treat*ent is: 1. Eifedipine $. Eicotina*ide %. Pheno<y#en;a*ine '. PsoralenPB?A (. Aspirin ). Show +<plaination A $D ' Sou suspect that a patient has acLuired an&ioede*a. 8e3els of which of the followin& would you e<pect to #e low. 1. C1L: C% $. C1L: #radykinin %. C': C1L '. #radykinin: C% (. C': C% ). Show +<plaination C $D ( A %( year old *an has a history of intensely pruritic papules and 3esicles on the e<tensor surfaces of his lower e<tre*ities. Anti#odies to epider*al trans&luta*inase are detected. ,hat is this patient at risk for de3elopin&. 1. Eon"Fod&kin ly*pho*a $. Colon cancer %. 8un& cancer '. C88 (. +sopha&eal cancer ). Show +<plaination A $D ) ,hich of the followin& is true re&ardin& this entity. 1. /he su#cutaneous 3ariant is *ost co**on in children $. A(N of locali;ed lesions are still present $ years after dia&nosis %. 8ocali;ed lesions often ulcerate and heal with scarrin& '. /he perforatin& 3ariant classically in3ol3es the lower e<tre*ities (. 0ia#etes is present in the *a4ority of patients with the &enerali;ed 3ariant ). Show +<plaination A $D A ,hat type of colla&en is o3errepresents in this lesion. 1. Colla&en 1 $. Colla&en 111 %. Colla&en 1? '. Colla&en ? (. Colla&en ?11 ). Show +<plaination B $D C /wenty"nail dystrophy: nail plate splittin& and ptery&iu* for*ation are nail chan&es seen in: 1. 8ichen Planus $. 0arierGs disease %. Psoriasis '. Scleroder*a (. 0er*ato*yositis ). Show +<plaination A $D D A patient de3elops pul*onary 6#rosis: HaynaudGs pheno*enon and skin chan&es si*ilar to syste*ic sclerosis. /hese chan&es resol3e followin& discontinuation of which of the followin& *edications. 1. Bleo*ycin $. Penicilla*ine %. Fydrala;ine '. Minocycline (. 1sonia;ide ). Show +<plaination A %! ! /he pri*ary co*ponent of hu*an se#u* is: 1. /ri&lycerides $. 2ree fatty acids %. Cera*ides '. Cholesterol (. SLualene ). Show +<plaination A %! 1 /he *ost co**on non"speci6c cutaneous *anifestation associated with this disease is: 1. 8eukocytoclastic 3asculitis $. +rtythe*a *ultifor*e %. Acne 3ul&aris '. +rythe*a nodosu* (. 8ichen planus ). Show +<plaination 0 %! $ ,hat is the #est *uscle to #iopsy in der*ato*yositis. 1. triceps $. #iceps %. Luadriceps '. &luteus *a<i*us (. deltoid ). Show +<plaination A %! % ,hich aller&en is the *ost likely cause of this eyelid der*atitis. 1. Mercapto#en;othia;ole $. +thyleneurea *ela*ine for*aldehyde %. +thylenedia*ine dihydrochloride 0 DA '. /osylona*ide for*aldehyde resin (. Ben;alkoniu* chloride ). Show +<plaination %! ' ,hich of the followin& is *ost associated with pruritus. 1. Purpura annularis telan&iectodes $. 5ou&erotXagBlu* syndro*e =pi&*ented purpuric lichenoid der*atitis> %. 8ichen aureus '. Scha*#er&hGs disease (. 0ucas and Kapetanakis pi&*ented purpura ). Show +<plaination + %! ( /he protein co*ponent of nodular a*yloidosis is: 1. SAA protein $. A8 protein %. Keratin '. Colla&en (. Bp1C! ). Show +<plaination B %! ) ,hat is the *ost co**on presentation of psoriatic arthritis. 1. Asy**etric oli&o" or polyarthritis $. Sy**etric polyarthritis %. Spondylitis =a<ial> '. 0istal interphalan&eal 4oint =01P> disease (. Arthritis *utilans ). Show +<plaination A %! A ,hich of the followin& is E9/ a feature associated with this condition. 1. Accelerated #lanch response $. ,hite der*ato&raphis* %. Anterior su#capsular cataracts '. Keratoconus (. Pityriasis al#a ). Show +<plaination A %! C ,hich en;y*e can #e a useful ad4unct test to dia&nose ;inc de6ciency where the seru* ;inc le3el is nor*al or near nor*al. 1. Kinc sulfatase $. Alkaline phosphatase %. AS/ '. A8/ (. Creatinine kinase ). Show +<plaination B %! D /he #est dia&nosis for this con&enital *elanopenic lesion without e<tracutaneous associations is 1. Fypo*elanosis of 1to $. Se&*ental 3itili&o %. Ash leaf spots '. Ee3us ane*icus (. Ee3us achro*icus ). Show +<plaination + %1 ! A patient presents with cutaneous lesions su&&esti3e of sarcoidosis. Sou consider orderin& a seru* AC+ le3el to help with the dia&nosis. ,hat is the sensiti3ity and speci6city of checkin& an ace le3el in this pt. 1. Sensiti3ity: )! Speci6city: C! $. Sensiti3ity: D! Speci6city: )! %. Sensiti3ity: D( Speci6city: C! '. Sensiti3ity: )! Speci6city: )! (. Sensiti3ity: D! Speci6city: $( ). Show +<plaination A %1 1 ,hich of the followin& F8A types is associated with psoriasis and predicts earlier onset and *ore se3ere disease. 1. F8A"B1% $. F8A"B1A %. F8A"Bw(A '. F8A"Cw) (. F8A"B$A ). Show +<plaination B %1 $ ,hich F8A type is *ore co**only associated with this clinical 6ndin&. 1. F8A"BA $. F8A"B1( %. F8A"B$A '. F8A"B(1 (. F8A"0H' ). Show +<plaination C %1 % ,hich of the followin& F8A types is associated with early onset psoriasis. 1. F8A"B1% or F8A"B1A $. F8A"B$A %. F8A"B(A: F8A"Cw): or F8A"0HA '. F8A"Cw$ (. F8A"0H% ). Show +<plaination C %1 ' Eeka*Gs 0isease: 1. 1s &enerally responsi3e to topical and intralesional steroids $. Characteristically lacks scale %. Harely in3ol3es the #uttocks '. Presents with a reticulate pattern on the dorsal hands and feet (. Presents with hypopi&*ented: atrophic 0 DC lesions on the e<tre*ities ). Show +<plaination %1 ( /F$ i**une responses: 1. Are associated with cell"*ediated i**unity $. Produce 18") %. Produce 12E"&a**a '. Produce /E2"#eta (. Produce 18"$ ). Show +<plaination B %1 ) Phrynoder*a can #e seen in all of the followin& nutritional de6ciency e<cept 1. ?ita*in A $. ?ita*in B %. ?ita*in C '. ?ita*in 0 (. ?ita*in + ). Show +<plaination 0 %1 A ,hat is the epony* used for this inherited: con&enital condition. 1. Mei&e disease $. Milroy disease %. Proteus syndro*e '. Klippel"/renaunay syndro*e (. Stuart"/re3es syndro*e ). Show +<plaination B %1 C ,hich of the followin& is correct a#out eosinophilic folliculitis. 1. painful $. only seen in adults %. classi6ed as an A10S"de6nin& illness '. *ore co**on in fe*ales (. associated with P. acnes infection of hair follicles ). Show +<plaination C %1 D Macro&lossia can present in all of the followin& disorders e<cept 1. Pri*ary syste*ic a*yloidosis $. 0ownGs syndro*e %. Mucopolysaccharidoses '. Cretinis* (. Behchets disease ). Show +<plaination + DD ). Ben&in @ Mali&nant /u*or # Question Answers An s 1 ,hich of the followin& is true re&ardin& di&ital FP?" associated sLua*ous cell cancers. 1. /he rate of *etastasis approaches 1(N. $. FP?1C is the *ost co**on associated sy#type. %. Mohs *icro&raphic sur&ery yields a $!N recurrence rate. '. ,o*en outnu*#er *en $:1. (. /hese lesions only occur in association with i**unosuppression. C $ ,hat 3irus is *ost closely associated with these lesions in this F1? infected patient. 1. Fu*an herpes 3irus $ $. Fu*an herpes 3irus ) %. Fu*an herpes 3irus C '. Cyto*e&alo3irus (. +#stein Barr 3irus C % All of the followin& are true re&ardin& lenti&o *ali&na e<cept: 1. Fi&h rates of recurrence $. 9ccurs *ostly on head and neck %. Mostly in sun"e<posed areas '. Mar&ins diTcult to e3aluate (. Spares oral *ucosa + ' All of the followin&s can #e used for treate*t of this condition e<cept 1. Cryotherapy $. /opical 1*iLui*od (N crea* %. /opical ("-urouracil '. /opical retinoids (. Sur&ical e<cision + ( /he *ost co**on location of super6cial spreadin& *elano*a in *en is: 1. 8ower le&s $. Back %. Bpper e<tre*ities '. Fead and neck (. 0i&its B ) 9n histolo&ic e<a*ination of the adenocarcino*a of the perineal area: which of the followin& stains would E9/ #e positi3e. 1. PAS $. Mucicar*ine %. C+A '. +MA (. FMB'( + A ,hich is the *ost co**on neoplas* in patients who ha3e had lon&"ter* PB?A therapy. 1. Basal cell carcino*a $. Atypical 6#ro<antho*a %. SLua*ous cell carcino*a '. Cutaneous / cell ly*pho*a (. Melano*a C C ,hich of the followin& are true for the si&n of 8eser" /relat. 1. 8esions are co**only located on the chest and #ack $. Classically descri#ed to appear in a JChrist*as treeJ pattern %. Bsually associated with internal *ali&nancies '. +rupti3e nature (. All of these answers are correct + D All of the followin& state*ents are true re&ardin& an&iosarco*as +OC+P/: 1. /hey occur *ore co**only in Caucasians than in non" Caucasians $. Men are *ore often aMected than wo*en %. /hey are rarely sy*pto*atic '. Hadiation is usually e*ployed after sur&ical e<cision (. Cer3ical ly*ph nodes are a co**on site of *etastases C 1! Merkel cell carcino*a stains positi3ely for: 1. 8eukocyte co**on anti&en $. Eeuron speci6c enolase %. ?i*entin '. S"1!! (. Eone of these answers are correct B 11 /he followin& lesion is the classic presentation of: 1. BCC $. Merkel cell carcino*a %. Melano*a '. C/C8 (. Keratoacantho*a + 1$ ,hich of the followin& are the *ost relia#le pro&nostic factors in *ali&nant *elano*a. 1. BreslowJs depth and ulceration $. BreslowJs depth and Clark le3el %. Clark le3el and ulceration '. BreslowJs depth and Clark le3el (. Eone of these answers are correct A 1% /he *ucin found in this lesion is produced #y: 1. 2i#ro#lasts $. Keratinocyte %. Merkel cells '. Eail *atri< (. 9steo#last A 1' /he incidence of cutaneous sLua*ous cell carcino*a in or&an transplant recipients is increased #y how *uch co*pared with the &eneral population. 1. $ fold $. ( fold %. 1! fold '. $! fold + 1!! (. )( fold 1( An elderly *an presents to your oTce with a telan&iectatic: 3iolaceous 1c* do*e"shaped nodule on the neck. Biopsy re3eals lar&e: solid nests of cells of inter*ediate si;e: with a tra#ecular pattern at the periphery. /hese cells in3ol3e the der*is and spread into the su#cutaneous fat: #ut spare the o3erlyin& epider*is. /he cells are round and unifor* in si;e: with a round to o3al nucleus: s*all nucleoli: and e3enly dispersed chro*atin. Eu*erous *itotic 6&ures and necrotic areas are seen. Eeuron speci6c enolase is positi3e. ,hich of the followin& is true re&ardin& this patientGs dia&nosis. 1. Mohs *icro&raphic sur&ery is contraindicated in treat*ent of this lesion $. ?i*entin and des*in stains *ay #e positi3e %. S"1!! stains should #e positi3e '. /his lesion *ay contain AC/F (. /he neoplas* should not contain &astrin 0 1) 1n which of the followin& ethnic &roups is sLua*ous cell carcino*a the *ost co**on type of skin cancer. 1. Asian 1ndians $. Caucasians %. Fispanics '. 7apanese (. Chinese A 1A /he treat*ent of choice for this lesion shown is: 1. Hadiation therapy $. 1*iLui*od %. ( 2lourouracil '. ,ide e<cision with $c* *ar&ins (. Mohs sur&ery + 1C All of the followin& *ay increase the incidence of SCC: +OC+P/: 1. Sun e<posure $. 1**unosuppression %. 1ncreasin& a&e '. Pro<i*ity to the eLuator (. All of the followin& *ay increase the incidence of SCC ). + 1D ,hich of the followin& is a clinical sta&e of keratoacantho*as. 1. PlaLue $. Mature %. Proliferati3e '. Hesol3in& (. All of these answers are correct e<cept plaLue + $! ,hat is the *ost co**on location for pa&etoid reticulosis. 1. /runk $. Fead and neck %. Fands and feet '. 2le<ural sites of upper and lower e<tre*ities (. 5enitals C $1 A %! year"old &entle*an presents with *ultiple self healin& lesions si*ilar to the one pictured. 9ther fa*ily fa*ilies also ha3e the sa*e disease. ,hat is the *ost likely dia&nosis. 1. +pider*odysplasia 3errucifor*is $. 2er&uson"S*ith syndro*e %. Ee3oid #asal cell carcino*a syndro*e '. 0yskeratosis con&enita (. Base< syndro*e B $$ ,hich of the followin& is not considered a hi&h"risk location of SCC: 1. +yelids $. Eose %. +ar '. 8ips (. Chest + $% '! year"old *ale presented with a slow &rowin& lar&e tu*or on the #ack. /he patient under&oes wide e<cision with ad4uncti3e che*otherapy. ,hich of the che*otherapeutic a&ents was used. 1. 1*atini# $. Cytara#ine %. 0o<oru#icin '. ?incristine (. Cisplatin A $' ,hich of the followin& are features of der*ato6#ro*as: which help diMerentiate it fro* der*ato6#rosarco*a protu#erans. 1. Co**only located on the e<tre*ities $. 2actor O111a ne&ati3e %. 2actor O111a positi3e '. Co**only located on the trunk (. Co**only located on the e<tre*ities and factor O111a positi3e + $( /he risk of a *elano*a de3elopin& in a &iant con&enital *elanocytic ne3us is appro<i*ately: 1. %N $. )N %. 1$N '. (!N (. ?irtually all of these patients will de3elop *elano*a B $) A patient with *ultiple skin nodules has a #iopsy su&&esti3e of cutaneous ly*pho*a. /he ne<t appropriate step in *akin& the dia&nosis is: 1. Poly*erase chain reaction $. Co*plete #lood count %. 2low cyto*etry '. 1**unohistoche*ical stains (. Chest <ray A $A An elderly white *an presents with a slowly enlar&in&: well"de*arcated pink: scaly plaLue on the neck. Biopsy of the lesion re3eals epider*al dysplasia and keratinocytic disor&ani;ation with preser3ation of the #ase*ent *e*#rane. Fyperkeratosis and parakeratosis are also present: and nu*erous atypical keratinocytes are seen throu&hout the epider*is: with loss of polarity: atypia: and *itoses. A chronic in-a**atory in6ltrate is present in the upper der*is. ,hich of the followin& treat*ent=s> should #e considered for this patient. 1. Con3entional e<cision $. Mohs *icro&raphic sur&ery %. 1*iLui*od '. Con3entional e<cision and Mohs *icro&raphic sur&ery (. All of these answers are correct + $C 1nter*ittent sun e<posure with painful sun#urns is a predisposin& factor for the de3elop*ent of: 1. Atypical ne3i $. Se#orrheic keratosis %. Mali&nant *elano*a '. Atypical ne3i and Mali&nant *elano*a (. All of these answers are correct 0 $D /he cure rate of cryotherapy as a treat*ent for actinic keratoses is: 1. C!N $. C(N %. D!N '. D(N (. DDN + 1!1 %! Co*pared with the &eneral population: what is the o3erall risk of de3elopin& cutaneous and syste*ic *ali&nancies in or&an transplant recipient. 1. ' fold $. 1! fold %. $( fold '. (! fold (. 1!! fold A %1 ,hat would #e the characteristic histopatholo&ic 6ndin&s of this lesion. 1. 2ull thickness keratinocytic atypia $. Cornoid la*ella %. Pale stainin& cells '. Forn pseudocysts (. ,ed&e shaped &ranular layer with lichenoid in6ltrate B %$ /he *ost co**on location for pri*ary *ucinous carcino*a is: 1. neck $. eyelid %. areola '. scrotu* (. nose B %% Co**only used i**unohistoche*ical *arkers that are positi3e in Merkel cell carcino*as include all of the followin& +OC+P/: 1. Chro*o&ranin AB $. Synaptophysin %. 5lial 6#rillary acidic protein '. Cytokeratin $! (. Cytokeratin C C %' /he Pinkus BCC is freLuently located on the: 1. 0i&its $. +yelid %. 8ips '. +ar (. 8u*#osacral area + %( /he *ost i*portant *utated &ene associated with a predisposition to de3elop *ali&nant *elano*a is: 1. P/CF $. C0KE$A %. P/CF$ '. Eone of these answers are correct (. All of these answers are correct B %) ,hat te*perature *ust #e achie3ed for adeLuate treat*ent of a s*all super6cial sLua*ous cell carcino*a with cryotherapy. 1. "1! de&rees Celcius $. "$! de&rees Celcius %. "%! de&rees Celcius '. "'! de&rees Celcius (. "(! de&rees Celcius + %A 0enileukin diftito< =9E/AKJ>: 1. is a syste*ic treat*ent option for C/C8 $. is a diphtheria fusion to<in %. tar&ets the interleukin"$ receptor '. Eone of these answers are correct (. All of these answers are correct + %C /he 5r;y#owski type of keratoacantho*a: 1. 1s characteri;ed #y rapid &rowth of a sin&le lesion reachin& a dia*eter of D c* or *ore $. /ypically in3ades underlyin& cartila&e %. 0e*onstrates si*ultaneous central healin& '. Presents in childhood on sun"e<posed surfaces (. Presents with hundreds of disse*inated lesions + %D 0es*oplastic trichoepithelio*a: 1. 1s *ost co**on in *iddle"a&ed patients $. More co**only appears in *ales than in fe*ales %. 0oes not display foci of calci6cation or ossi6cation '. Presents as a well"circu*scri#ed lesions located in the upper der*is (. Eone of these answers are correct 0 '! Se3enty"63e year old: *ale patient: with a %c*s pink: pearly nodule on his chest. Sou notice that the patient has a scar on the sa*e area. /he treat*ent of choice is: 1. Hadiation $. Con3entional e<cision %. Cryotherapy '. Mohs sur&ery (. 1*iLui*od 0 '1 ,hat is the epony* for this *etastasis to the u*#ilicus. 1. /ripe pal* $. Si&n of 8eser"/relat %. Pityriasis rotunda '. /rousseau syndro*e (. Sister Mary 7oseph Eodule + '$ ,hich of the followin& is the *ost co**on initial site of *etastasis fro* a pri*ary BCC. 1. 8un&s $. He&ional ly*ph nodes %. Bone '. 8i3er (. Pleura B '% A youn& wo*an presents with a sin&le s*all: 6r*: u*#ilicated papule on the face. Biopsy re3eals a well"circu*scri#ed lesion located in the upper der*is. Strands of #asaloid cells are seen surrounded #y 6#rotic or des*oplastic stro*a. Forn cysts and foci of se#aceous cells and calci6cation are also noted. Fow should this patient and her lesion #e treated. 1. Heassurance and no further treat*ent $. Close clinical follow"up %. /opical ("-uorouracil '. Cryotherapy (. 8ocal sur&ical e<cision + '' ,hich of the followin& is an i**unhistoche*ical *arker for Merkel Cell Carcino*a.: 1. S"1!! $. ?i*entin %. FMB"'( '. Eeuron speci6c enolase (. All of these answers are correct 0 '( ,hich of the followin& i**unolo&ic dru&s has #een shown to increase sur3i3al of patients with sta&e 111 and sta&e 1? *elano*a. 1. +tanercept $. Adali*u*a# %. Bstekinu*a# '. 1pili*u*a# (. 1n-i<i*a# 0 ') ,hat is the *ost location for this lesion which lacks 1. Back C 1!$ phosphorylase in the epider*al cells. $. Buttock %. 8e& '. Eeck (. Sacru* 'A A (! year"old fe*ale patient presents with a reddish indurated plaLue located on her ri&ht cla3icular area =see i*a&e>. /he #iopsy shows the presence of *ono*orphic spindle cells in a storifor* pattern: and deep su#cutaneous in6ltration. Accordin& to the afore*entioned infor*ation: the *ost likely dia&nosis is: 1. Microcystic adne<al carcino*a $. Se#orrheic keratosis %. 0er*ato6#rosarco*a protu#erans '. Merkel cell carcino*a (. Eone of these answers are correct C 'C /he *ost co**on site for intra oral *elano*a is. 1. #uccal $. soft palate %. hard palate '. &in&i3a (. u3ula C 'D ,hich of the followin& is true re&ardin& actinic keratoses. 1. Salicylic acid: tretinoin: and alpha"hydro<y acids are not useful in treat*ent $. 8ow fat diets *ay decrease the incidence of AKs %. Cure rates for treat*ent with topical ("-uorouracil are hi&her than for treat*ent with cryotherapy '. B?A fro* sunli&ht is *ost responsi#le for AK de3elop*ent (. Male &ender is not a risk factor for AKs. B (! ,hat is the *ean ti*e"fra*e for de3elop*ent of Stewart"/re3es Syndro*e. 1. 8ess than 1 year $. 1 year %. ( years '. 1! years (. `$! years + (1 Fistolo&ically: this lesion is shows plu*p: poly&onal cells arran&ed in nests and fascicles with &ranular cytoplas*. ,hich i**unohistoche*ical stain would #e positi3e. 1. Colloidal iron $. ?on kossa %. ,arthin"starry '. S"1!! (. C0)C 0 ($ ,hat is the *ost co**on location of oral SCC. 1. Soft palate $. Buccal *ucosa %. 5in&i3a '. 0orsal ton&ue (. 8ateral ton&ue + (% ,hich phase of the cell cycle does p(% re&ulate. 1. 51 $. 5$ %. S phase '. Mitosis (. Meiosis A (' All of the followin& state*ents re&ardin& BowenGs disease are true +OC+P/: 1. 8esions arisin& on the lower li*#s are *ore co**on in *en than in wo*en $. /he #ase*ent *e*#rane re*ains intact on histopatholo&y %. (N of patients with BowenGs disease de3elop in3asi3e sLua*ous cell carcino*a '. Chronic sun e<posure is a risk factor for BowenGs disease (. Mucosal BowenGs disease *ay appear as a 3errucous plaLue A (( A (( year"old fe*ale presents with an ulcerated *ali&nant *elano*a with Breslow dept of 1.(**. Sentinel ly*ph node #iopsy is ne&ati3e. ,hich of the followin& is the correct sta&in& classi6cation. 1. Sta&e 1B $. Sta&e 11A %. Sta&e 11B '. Sta&e 111A (. Sta&e 111B B () 0er*oscopic features su&&esti3e of *ali&nant *elano*a include: 1. Presence of $ or *ore colors within the lesion $. Blue"whitish 3eil %. Asy**etric radial strea*in& '. A#rupt interruption of pi&*ent network in the periphery (. All of these answers are correct + (A ,hich su#stance does p(% nor*ally acti3ate to pro*ote apoptosis 3ia inhi#ition of #cl"$. 1. p$1 $. p1) %. Pu*a '. Md*$ (. Akt C (C A )( year"old fe*ale with *ultiple actinic keratosis on the face under treat*ent with ("2B. Accordin& to the i*a&e and afore*entioned infor*ation: you *ay conclude that: 1. /he patient *ust i**ediately stop treat*ent since une<pected side eMects ha3e de3eloped $. /he patient has #een co*pliant with ("2B treat*ent and the appearance of in-a**ation: erythe*a and erosions are e<pected %. /he i*a&e is not rele3ant to ("2B treat*ent '. Eone of these answers are correct (. All of these answers are correct B (D ,hich of the followin& features of thin *elano*as =d1 ** thick> has #een associated with an increased risk of *etastasis. 1. He&ression $. 8ocation %. Si;e of lesion '. 5ender (. P(% e<pression A )! /he risk of *etastasis fro* SCC increases with: 1. /u*or si;e $. 0epth of in3asion %. 0e&ree of diMerentiation '. 1**unosupression (. All of these answers are correct + 1!% )1 ,hich of the followin& is a co**on location of *elano*a in wo*en. 1. Chest $. 8ower le&s %. 5enitals '. 0i&its (. Scalp B )$ Sentinel ly*ph node #iopsy in *ali&nant *elano*a: 1. Fas &ained acceptance for the treat*ent of MM of inter*ediate thickness =1"'**> $. 1s *ainly #ein& used for a *ore accurate sta&in& %. 1s not reco**ended '. Eone of these answers are correct (. Fas &ained acceptance for the treat*ent of MM of inter*ediate thickness =1"'**> and is *ainly #ein& used for a *ore accurate sta&in& + )% /he keratoacantho*a 3ariant characteri;ed #y the sudden appearance durin& childhood or adolescence of *ultiple KAs is called: 1. 5orlin syndro*e $. Oeroder*a pi&*entosa %. 2er&uson"S*ith '. 5r;y#owski (. Eone of these answers are correct C )' A '! year"old fe*ale patient presents with the followin& lesion =see i*a&e>. /he #iopsy report shows duct like structures: tadpole structures within a 6#rotic stro*a. Accordin& to the afore*entioned infor*ation: the *ost likely dia&nosis is: 1. Microcystic adne<al carcino*a $. Se#orrheic keratosis %. 0er*ato6#rosarco*a protu#erans '. Merkel cell carcino*a (. Eone of these answers are correct A )( Mutations of the p(% &ene has #een associated with the de3elop*ent of: 1. M*elano*a $. KaposiJs sarco*a %. Actinic keratosis '. Molluscu* conta&iosu* (. All of these answers are correct C )) ,hich test should #e used to detect *onoclonal &ene rearran&e*ents in cutaneous /"cell ly*pho*a. 1. Eorthern #lot $. Southern #lot %. ,estern #lot '. +81SA (. +lectrophoresis B )A ,hich of the followin& is *ost likely to present with cutaneous *etastases in *en. 1. 8un& cancer $. Colon cancer %. Prostate cancer '. Melano*a (. +sopha&eal Cancer 0 )C ,hen perfor*in& a #iopsy of a suspected keratoacantho*a: 1. 2usifor* incision throu&h the entire KA *ay #e perfor*ed $. 1t is necessary to #iopsy down to su#cutaneous fat %. A full"thickness sha3e #iopsy is accepta#le '. A co*plete e<cisional #iopsy *ay #e perfor*ed (. All of these answers are correct + )D At what location is this tu*or 8+AS/ likely to recur after sur&ical e<cision. 1. Easola#ial fold $. 8ateral canthus %. Mid forehead '. Preauricular (. Scalp C A! Knowin& that the patient in this picture has a #iopsy con6r*ed BCC: which of the followin& would #e the treat*ent of choice: 1. Con3entional e<cision $. 1*iLui*od %. Hadiation '. Mohs *icro&raphic sur&ery (. Cryosur&ery ). 0 A1 ,hich of the followin& *elano*a su#types is *ore consistently seen in dark"skinned indi3iduals. 1. Acral lenti&inous *elano*a $. Super6cial spreadin& *elano*a %. 8enti&o *ali&na *elano*a '. Eodular *elano*a (. Super6cial spreadin& *elano*a and 8enti&o *ali&na *elano*a A A$ ,hich one of the followin& *ali&nancies is associated with FP? infection. 1. ?errucous carcino*a $. Metastatic *elano*a %. Basal cell carcino*a '. Se#aceous carcino*a (. Atypical 6#ro<antho*a A A% ,hich of the followin& is co**ony seen in se#orrheic keratoses when e<a*ined with der*oscopy. 1. Maroon la&oons $. Eetwork %. Pi&*ent &lo#ules '. Milia like cysts (. Blue"&ray 3eil 0 A' ,hich one of the followin& a&ents has de*onstrated potential #ene6t as a che*opre3enti3e to B?" induced skin cancer. 1. Prosta&landin +$ $. ?ita*in 0 %. Arachidonic acid '. Celeco<i# (. ?ita*in + 0 A( /he *ost co**on location for an&iosarco*a is: 1. 8e&s $. Ar*s %. Fead and neck '. /runk (. 0i&its C A) A ()"year old wo*an with a history si&ni6cant for chronic ly*phede*a after radical *astecto*y twel3e years a&o presents with this &rowth on her ar*. ,hat is the dia&nosis. 1. An&iosarco*a $. Bacillary an&io*atosis %. Castle*anGs syndro*e '. KaposiGs sarco*a (. Metastatic #reast carcino*a A 1!' AA ,hich of the followin& *elano*a scenarios ha3e the #est pro&nosis. 1. /wenty"one year old fe*ale with pri*ary lesion located on the ri&ht lower le& $. /wenty"one year old *ale with pri*ary lesion located on the chest %. /hirty"si< year old *ale with pri*ary lesion located on the #ack '. /wenty"one year old fe*ale with pri*ary: ulcerated lesion located on the ri&ht lower le& (. /hirty"si< year old *ale with pri*ary lesion located on his left lower le& with palpa#le in&uinal ly*ph nodes A AC Ba;e< syndro*e can #e diMerentiated clinically fro* Ho*#o syndro*e #y presence of 1. Multiple #asal cell carcino*as $. /richiepithelio*as %. Milia '. Bollicular atrophoder*a (. Fypohidrosis + AD 1f left untreated: which of the followin& is not at risk for *ali&nant transfor*ation. 1. Bowenoid papulosis $. Cutaneous horn %. Actinic cheilitis '. 8eukoplakia (. Stucco keratosis + C! ,hat kind of /"ly*phocyte is the *ost co**on neoplastic cell in Cutaneous /"Cell 8y*pho*a: 1. C0' $. C0C %. Eatural killer '. Eone of these answers are correct (. All of these answers are correct A C1 All of the followin& state*ents re&ardin& the patient pictured are true +OC+P/: 1. Spina #i6da *ay #e an associated 6ndin& $. /he patient likely has a *utation in the P/CF &ene %. A*elo#lasto*a is a tu*or associated with this disease '. /his patient likely had a si*ilarly aMected parent (. /hese lesions appeared in childhood C C$ A patient has a *ali&nant *elano*a 1.)** thick with ulceration and a *icro*etastasis in 1 node. /he patientGs sta&in& accordin& to the A*erican 7oint Co**ittee on Cancer Sta&in& Syste* is: 1. 11C $. 111A %. 111B '. 111C (. 1? C C% ,hat is the *ost co**on location for an epitheloid sarco*a. 1. head and neck $. pro<i*al e<tre*ities %. hands and forear*s '. lower le&s (. &roin and #uttocks C C' Mutations in which &ene would likely #e found in the neoplastic cells of this lesion. 1. PA/CF $. p(% %. 2u*arate hydratase '. CH+BBP (. p)% B C( ,hich of the followin& is the *ost i*portant pro&nostic indicator in a patient with cutaneous ly*pho*a. 1. A&e $. Pri*ary 3s secondary cutaneous %. 5ender '. +<tent of cutaneous in3ol3e*ent (. Su#type of ly*pho*a B C) /he patient is a '( year old *ale co*plainin& of red: chapped lower lip. ,hich of the followin& lasers is the *ost appropriate to treat this condition. 1. Pulsed 0ye 8aser $. Ed:SA5 laser %. C9$ laser '. 0iode laser (. 8aser treat*ent is not an option C CA ,hat is the *ost co**on site of *etastasis for this der*al tu*or. 1. 8un& $. Brain %. Kidney '. 8i3er (. Bone A CC ,hich of the followin& state*ents re&ardin& pro&nosis for *ali&nant *elano*a is true. 1. 1ncreasin& a&e has a positi3e eMect on sur3i3al. $. Patients with pri*ary lesions located on the e<tre*ities =e<cept acral lesions> ha3e a worse pro&nosis than those with tu*ors located on the trunk. %. /here is a hi&her sur3i3al rate for patients with palpa#le *etastatic nodes co*pared to those with *icro*etastatic nodal disease. '. 2or sta&e 1? disease: patients with non"3isceral *etastases =e& skin: su#cutis: distant ly*ph nodes> ha3e a #etter pro&nosis co*pared with those with 3isceral *etastases. (. Male &ender has a positi3e eMect on sur3i3al. 0 CD ,hich of the followin& are features of *icrocystic adne<al carcino*a that help distin&uish it fro* des*oplastic trichoepithelio*as. 1. 0eep su#cutaneous in6ltration $. Perineural in3asion %. C+A positi3e stainin& '. Co**only located on the face (. 0eep su#cutaneous in6ltration:perineural in3asion: and C+A positi3e stainin& + D! ,hich of the followin& neoplas*s has de*onstrated an association with F/8?"1 infection. 1. Mycosis fun&oides $. Adult / cell ly*pho*a %. 2ollicular ly*pho*a '. Multiple *yelo*a (. Fod&kinJs disease B D1 Chloro*a is a characteristic cutaneous *anifestation of: 1. /u#erous sclerosis $. SweetJs syndro*e %. Eeuro6#ro*atosis 0 1!( '. 8euke*ia (. Pseudo*onas sepsis D$ ,hat is the dia&nosis. 1. Condylo*a acu*inata $. Molloscu* conta&iosu* %. Pearly penile papules '. 8ichen planus (. Psoriasis C D% Periun&ual SLua*ous cell carcino*a has #een linked to which FP? type=s>. 1. ): 11 $. $: ' %. 1) '. 1% (. C C D' ,hat would you e<pect to see under der*oscopy of this 3ascular neoplas*. 1. Hed sacculae $. Ar#ori;in& #lood 3essels %. Fair pin telan&ectasia '. Milky red &lo#ules (. Blue"&rey o3oid nests A D( ,hich FP? serotype has #een associated with periun&ual SCC. 1. ) $. 1) %. 11 '. All of these answers are correct (. FP? is not associated with SCC B D) /he 5orlin syndro*e is characteri;ed #y: 1. Multiple BCCs durin& childhood $. Macrocephaly %. 9donto&enic keratocysts of the 4aw '. Autoso*al"recessi3e inheritance pattern (. All of these answers are correct e<cept Autoso*al" recessi3e inheritance pattern + DA 1**unohistoche*istry of der*ato6#rosarco*a protu#erans typically re3eals: 1. C0%' ne&ati3e and factor O111a positi3e $. C0%' ne&ati3e and factor O111a ne&ati3e %. C0%' positi3e and factor O111a positi3e '. C0%' positi3e and factor O111a ne&ati3e (. Eone of these answers are correct 0 DC /he *ost co**on locations of *icrocystic adne<al carcino*a include all of the followin&: e<cept: 1. Perioral $. Easola#ial %. /runk '. Perior#ital (. Perioral:nasola#ial: and perior#ital C DD Maple leaf"like structures seen on der*oscopy are characteristic of which lesion. 1. Se#orrheic keratoses $. Pi&*ented #asal cell carcino*a %. 0er*al ne3i '. Melano*a (. Fe*an&io*a B 1! ! /opical ("2luorouracil: 1. 1nterferes with the synthesis of 0EA and HEA $. 1s an alternati3e for the treat*ent of actinic keratosis %. May cause pruritus and #urnin& at the site of application '. All of these answers are correct (. Eone of these answers are correct ). 0 1! 1 B?B induced *utations on the P/CF &ene is associated with the de3elop*ent of: 1. BCC $. Merkel cell carcino*a %. An&iosarco*a '. BCC and Merkel cell carcino*a (. Merkel cell carcino*a and An&iosarco*a ). A 1! $ Mohs *icro&raphic sur&ery the treat*ent of choice for all of the followin&: e<cept: 1. 1 c* SCC located on the chest $. $ c*s BCC on lower e<tre*ities %. 1 c* BCC on the eyelid '. Morpheafor* BCC on the cheek (. Hecurrent BCC on the chest A 1! % Fistopatholo&ical e3idence of epider*otropis* *ay #e seen in which of the followin& conditions: 1. Merkel cell carcino*a $. SCC %. C/C8 '. Eone of these answers are correct (. All of these answers are correct C 1! ' Currently: the sur&ical *ar&in for *elano*as that *easure less than $ ** in thickness is: 1. 1 ** $. !.( c* %. 1 c* '. $ c*s (. % c*s C 1! ( ,hich of the followin& i**unohistoche*istry *arker is ne&ati3e in an&iosarco*as. 1. C0%1 $. C+A %. Cytokeratin '. C0%' (. 2actor ?111 B 1! ) /he hu*an papillo*a 3irus type associated with red #rown s*ooth and warty papules is 1. FP? 1 $. FP? ( %. FP? A '. FP? 1% (. FP? 1) + 1! A All of the followin& disorders *ay *anifest as e<foliati3e der*atitis e<cept 1. Behchets disease $. Psoriasis %. Pe*phi&us foliaceus '. 0ru& reaction A 1!) (. Se;ary syndro*e 1! C 0er*oscopy would #e the least helpful in e3aluatin& with lesion. 1. Pi&*ented #asal cell carcino*a $. Con&enital *elanocytic ner3us %. Se#orrheic keratosis '. A*elanotic *elano*a (. Fe*an&io*a 0 1! D /his tu*or: also called a Sha&reen patch: is characteristic of which of the followin& &enoder*atoses. 1. Eeuro6#ro*atosis"1 $. Eeuro6#ro*atosis"$ %. /u#erous sclerosis '. EAM+ syndro*e (. Buschke"9llendorf C 11 ! Keratoacantho*as ha3e #een linked etiolo&ically to: 1. Bltra3iolet e<posure $. Fu*an papillo*a 3irus %. Che*ical carcino&ens such as tar and pitch '. S*okin& (. All of these answers are correct + 11 1 A*pli6cation of which of the followin& &enes is associated with Merkel cell carcino*a. 1. 8"Myc $. C"Myc %. 5811 '. C0KE$A (. P/+E A 11 $ ,hat cancer in wo*en *ost co**only *etastasi;es to the skin. 1. Breast cancer $. Medullary thyroid carcino*a %. 5lio#lasto*a *ultifor*e '. Colon adenocarcino*a (. Cer3ical cancer A 11 % ,hich of the followin& is true re&ardin& poikiloder*atous *ycosis fun&oides. 1. Ma4ority of cases are predo*inantly C0C=P> $. Patients typically ha3e a later a&e of onset co*pared to classic *ycosis fun&iodes %. Can #e associated with 8yP '. More wo*en than *en aMected (. Patients typically do not respond well to phototherapy C 11 ' All of the followin& state*ents re&ardin& Basal Cell Ee3us Syndro*e are true +OC+P/: 1. Associated tu*ors include *edullo#lasto*a $. 1t is inherited in an autoso*al recessi3e *anner %. Fyperteloris* is a feature '. /he aMected &ene is *utated in %!"'!N of sporadic #asal cell carcino*as (. All of these answers are correct B 11 ( ,hat is the *ost co**on location for this rapidly &rowin& tu*or. 1. Fead and neck $. Chest %. Back '. Ar*s (. 8e&s ). A 11 ) A patient with a innu*era#le disse*inated keratoacantho*as: includin& lesions on the laryn< and oral *ucosa: 1. 1s unlikely to ha3e pal*oplantar in3ol3e*ent $. 8ikely has an underlyin& i**une de6ciency %. 1s at hi&h risk for *yelodysplasia '. 8ikely inherited their condition in an autoso*al do*inant *anner (. 8ikely de3eloped the* durin& adulthood ). + 11 A /he *ost co**on location of Merkel cell carcino*a is: 1. 0i&its $. /runk %. 5enitalia '. +<tre*ities (. Fead and neck + 11 C /his lesion is associated with which of the followin&: 1. FP? ( $. FP? 11 %. FP? ' '. FP? A (. FP? 1 B 11 D Characteristics indicati3e of a hi&h risk of *etastasis with SCC include: 1. Mitotic rate $. +<tre*ity location %. ,ell diMerentiated '. 0eep in3asion (. Papillo*a3irus infection 0 1$ ! ,hich of the followin& would you not e<pect to see under der*oscopy. 1. Maple leaf pattern $. Ar#ori;in& #lood 3essels %. Blue"&rey o3oid nests '. 9ran&e crust (. Milky red &lo#ules + 1$ 1 ,hich of the followin& ethnic &roups are co**only dia&nosed with der*atosis papulosa ni&ra: 1. Asians $. Fispanics %. African"A*ericans @ Fispanic patients '. Caucasians (. Eo diMerence #etween ethnic &roups C 1$ $ A child presents with a &iant con&enital ne3us o3erlyin& the #ack of the skull: e<tendin& onto the shoulders. 1t is _1(N #ody surface area: sparin& the face and anterior neck. ,hich test should #e ordered. 1. A head C/ $. A head MH1 %. A skull plain 6l* O"ray '. A skin #iopsy (. A P+/ scan B 1$ % ,hich area of the face recei3es the *ost cu*ulati3e e<posure to B? radiation. 1. 0orsu* of nose $. 9r#ital re&ion %. Base of the nose '. Chin C 1!A (. Central cheek 1$ ' 0es*oplastic trichoepithelio*as are co**only located on: 1. 8e&s $. Chest %. Back '. 2ace (. Scalp 0 1$ ( Pseudorosettes in Merkel cell carcino*a: 1. Are seen in the tra#ecular 3ariant $. Are seen in the inter*ediate"cell type %. Are seen in the s*all"cell type '. All of these answers are correct (. Eone of these answers are correct A 1$ ) Se;ary syndro*e: 1. Fas characteristic Se;ary cells in peripheral #lood $. 1s the leuke*ic 3ariant of Mycosis fun&oides %. 1s characteri;ed #y the triad of pruritic erythroder*a: &enerali;ed ly*phadenopathy: and presence of Se;ary cells in peripheral #lood '. 1s associated with a poor pro&nosis (. All of these answers are correct + 1$ A A patient has a '.( ** ulcerated *elano*a without re&ional or distant *etastases. Accordin& to the new A7CC criteria: what *elano*a sta&e does this correspond to. 1. 1n situ $. Sta&e 1 %. Sta&e 11 '. Sta&e 111 (. Sta&e 1? C 1$ C ,hich of the followin& 3ariants of *ycosis fun&oides is #est dia&nosed usin& a punch #iopsy instead of a #road super6cial sha3e #iopsy. 1. ,orin&er"Kolopp pa&etoid reticulosis $. Syrin&otropic *ycosis fun&oides %. Ketron"5ood*an pa&etoid reticulosis '. Poikiloder*atous *ycosis fun&oides (. Se;ary syndro*e B 1$ D ,hich of the followin& #est descri#es the incidence of skin cancer in transplant recipients fro* &reatest to least. 1. BCC`*elano*a`SCC`Merkel cell $. SCC`BCC`*elano*a`Merkel cell %. BCC`SCC`*elano*a`Merkel cell '. SCC`BCC`Merkel cell`*elano*a (. Merkel cell`SCC`*elano*a`BCC B 1% ! ,hich of the followin& lesions are J#etter felt than seenJ: 1. Se#orrheic keratosis $. SCC in situ %. Actinic keratosis '. Se#aceous hyperplasia (. All of these answers are correct C 1% 1 0er*ato6#rosarco*a protu#erans is: 1. Cyto&enetically characteri;ed #y reciprocal translocation t=1Ac$$>=L$$cL1%> $. 2actor O111a positi3e %. C+A positi3e '. C0 %' positi3e (. Cyto&enetically characteri;ed #y reciprocal translocation t=1Ac$$>=L$$cL1%> and C0 %' positi3e + 1% $ A )! year"old fe*ale presents with a well" de*arcated: scaly: erythe*atous plaLue on her ri&ht shin. /he #iopsy shows full thickness epider*al atypia with scattered *itotic 6&ures and o3erlyin& parakeratosis. Fower3er: the #ase*ent *e*#rane re*ains intact. Accordin& to the afore*entioned infor*ation: the dia&nosis is: 1. BCC $. SCC %. BowenGs disease '. An&iosarco*a (. C/C8 C 1% % A %"year"old &irl presents with *ultiple s*all: an&ulated: 6r* nodules. /here is a positi3e Itent si&nI and #iopsy re3eals &host cells and &er*inati3e cells. 9f the followin& conditions associated with *ultiple pilo*atrico*as: which is thou&ht to #e *ost closely linked. 1. /urnerGs $. Myotonic dystrophy %. Hu#enstein"/ay#i '. Sarcoidosis (. 5ardnerGs syndro*e B 1% ' All of the followin& are risk factors for *etastasis fro* a pri*ary sLua*ous cell carcino*a +OC+P/: 1. 1ncreasin& tu*or si;e $. 8ocation on the ear %. Hecurrent tu*or '. /u*or within ulcer (. All of these answers are correct + 1% ( Si<ty"year old: *ale patient that presents with a Jstuck onJ: wa<y: hyperkeratotic and hyperpi&*ented plaLue on his #ack. /he *ost likely dia&nosis is: 1. Actinic keratosis $. SCC in situ %. An&iosarco*a '. Se#orrheic keratosis (. Eone of these answers are correct 0 1% ) Merkel cell carcino*a has #een found to #e associated with which of the followin& 3iruses. 1. Ferpes 3irus $. Polyo*a3irus %. Para*y<o3irus '. 2la3i3irus (. +ntero3irus B 1% A A patient presents with tender papules with a pseudo"0arierGs si&n. She has other fa*ily *e*#ers with the sa*e condition. Screenin& should #e perfor*ed to rule out which *ali&nancy. 1. 5astric carcino*a $. 93arian carcino*a %. Henal cancer '. /esticular cancer (. 8un& cancer C 1% C ,hat is the *ost likely dia&nosis. 1. AcLuired di&ital 6#rokerato*a $. Supernu*ery di&it %. ?erruca '. 1nfantile di&ital 6#ro*a (. Acrochordon A 1% D ,hich of the followin& i**unohistoche*ical *arkers *ay #e indicati3e of *etastatic potential for *erkel cell carcino*a. 1. Chro*o&ranin $. Synaptophysin %. C0'' C 1!C '. CK$! (. //2"1 1' ! All of the followin& are true re&ardin& curetta&e of &iant con&enital *elanocytic ne3i e<cept: 1. /reat*ent re*ains a contro3ersial topic $. Best perfor*ed durin& the 6rst $ weeks of life %. 0ecreases the risk of *elano*a '. 9Mers an adeLuate alternati3e to sur&ical e<cision (. Mandates careful lon&"ter* follow"up C 1' 1 A patient ha3in& Mohs sur&ery for a sLua*ous cell carcino*a on the ear has tu*or in3adin& the cartila&e. ,hat sta&e disease does the patient ha3e. 1. /! $. /1 %. /$ '. /% (. /' + 1' $ All of the followin& cytokines ha3e de*onstrated therapeutic #ene6t in the treat*ent of *elano*a +OC+P/: 1. 12E"alpha $. 18"$ %. /E2"alpha '. 18"1! (. 5M"CS2 + 1' % All of the followin& are true of thick *elano*as =`% **> e<cept: 1. Predo*inantly nodular type $. ,o*en aMected *ore than *en %. Predilection for the head and neck '. Mainly in older patients =`(! years> (. Associated with fewer ne3i B 1' ' All of the followin& are true of *elano*a"associated leukoder*a e<cept: 1. 8esions rese*#le 3itili&o $. Portends a worse pro&nosis %. Seen in patients with *etastatic disease #ut no pri*ary lesion '. Fistolo&y rese*#les that of a halo ne3us (. 8esions de3elop distant to *elano*a B 1' ( ,hich of the followin& *arkers do not stain *elanocytic lesions: 1. Eeuron speci6c enolase $. ?i*entin %. S"1!! '. FMB"'( (. All of these answers are correct A 1' ) Merkel cell carcino*a should #e treated with what si;e sur&ical *ar&ins. 1. $** $. (** %. 1c* '. %c* (. (c* 0 1' A 1*iLui*od is an i**une response *odi6er that sti*ulates innate and cell *ediated i**une pathways. 1t induces all of the followin& cytokines +OC+P/: 1. 18"1 $. 18"' %. 18"( '. 18") (. 18"C B 1' C ,hich neoplas* is associated with the Stewart" /re3es syndro*e. 1. Henal leio*yo*as $. Basal cell carcino*a %. An&iosarco*a '. Keratoacantho*a (. / cell ly*pho*a C 1!D A. Medical Mycolo&y # Question Answers An s 1 /he *a4or ende*ic area for Fistoplas*osis 1. Africa $. Central A*erica %. Southwest Bnited States '. South A*erica (. +astern Bnited States ). + $ Eonder*atophytes &rowin& on 0er*atophyte /est Media cause the *edia to turn what color. 1. A*#er $. Hed %. Sellow '. Black (. 5reen ). C % All of the followin& para*eters are used to distin&uish der*atophytes e<cept: 1. Eutritional reLuire*ents $. Colony *orpholo&y %. Sucrose hydrolysis '. Fair -uorescence (. 5rowth te*perature ). C ' /his or&anis* is often considered a conta*inant #ut has #een reported to cause onycho*ycosis: 1. Sepedoniu* $. Cur3ularia %. Scopulariopsis '. Penicilliu* (. Phialophora ). C ( /richophyton tonsurans sporulates 3ia thick"walled round cells resistant to the en3iron*ent known as: 1. Arthroconidia $. Blastoconidia %. Chla*ydoconidia '. Sporan&ia (. Mycelia ). C ) /richosporon o3oides is a cause of: 1. Black piedra $. ,hite piedra %. /inea ni&ra pal*aris '. +ctothri< tinea capitis (. +ndothri< tinea capitis ). B A A patient with white nodules on the hair shaft has a K9F which shows hyphae and arthroconidia. /he etiolo&y is: 1. Coryne#acteriu* *inutissi*u* $. +<ophiala wernickii %. Piedraia hortae '. /richosporon #ei&elii (. Coryne#acteriu* tenuis ). 0 C A fun&al culture de*onstrates a suedelike crea*"colored colony of teardrop and #alloon"shaped *icroconidia which produce a red"#rown pi&*ent. ,hich of the followin& is true of this or&anis*. 1. Causes ectothri< infection $. HeLuires partial thia*ine for &rowth %. 0oes not ha3e arthroconidia B 11! '. Causes -uorescent hair infection (. 1s not a cause of tinea un&ui* ). D Mycelia can for* structures with a co*#" lke appearance called: 1. Hacket for*s $. 2a3ic chandeliers %. Pectinate #odies '. Spiral hyphae (. Eodular #odies ). C 1! 5rowth of which of the followin& is not inhi#ited on Mycosel *edia. 1. Cryptococcus neofor*ans $. Seast for*s of Fistoplas*a %. Seast for*s of Blasto*yces '. Microsporu* &ypseu* (. Scytalidiu* species ). 0 11 ,hich of the followin& or&anis*s would you e<pect to reco3er fro* a -uorescent tinea capitis. 1. /. tonsurans $. M. &ypseu* %. M. canis and M. &ypseu* '. /. 3iolaceu* (. /. ru#ru* ). C 1$ ,hich of the followin& fun&i can cause an endothri< tinea capitis and fa3us. 1. /. tonsurans $. M. canis %. /. schoenleinii '. M. audouinii (. /. ru#ru* ). C 1% All of the followin& state*ents a#out paracoccidioido*ycosis are true +OC+P/. 1. 1t is *ost co**on in *ale a&ricultural workers $. 1t has a characteristic J*arinerJs wheelJ appearance on histopatholo&y %. 0isease is al*ost always con6ned to the skin '. 1t is ende*ic to Bra;il (. 1t *ay cause *ucocutaneous lesions ). C 1' ,hich of the followin& state*ents re&ardin& super6cial *ycotic infections is /HB+. 1. Malasse;ia species do not -uoresce under a ,oodJs la*p $. Phaeoannello*yces wernecki causes tinea ni&ra %. /richophyton #ei&elii causes white piedra '. /richosporon hortae causes #lack piedra (. Micrococcus sedentarius causes tinea ni&ra ). B 1( ,hich of the followin& usually does not 1. M. audouinii C 111 -uoresce #ri&ht &reen upon ,oods la*p e<a*ination. $. M. canis %. /. 3iolaceu* '. /. schoenleinii (. M. distortu* ). 1) ,hich of the followin& 09+S E9/ typically cause white super6cial onycho*ycosis. 1. Asper&illus species $. 2usariu* species %. /richophyton *enta&rophytes '. Scopulariopsis species (. /richophyton ru#ru* ). + 1A Sa#ouraud 0e<trose A&ar =S0A> +**ons Modi6cation contains which of the followin&. 1. Peptones $. Phenol red %. Chlora*phenicol '. 5enta*icin (. Cyclohe<i*ide ). A 1C /his 1) year"old patient was recently dia&nosed with F1?: the dia&nosis is: 1. 0istal 9nycho*ycosis $. Pro<i*al Su#un&ual 9nycho*ycosis %. Pro<i*al ,hite Su#un&ual 9nycho*ycosis '. ,hite Super6cial 9nycho*ycosis (. Paronychia with Candida 9nycho*ycosis ). C 1D ,hich two characteristics co*#ined for* a si&ni6cant risk for acLuirin& sporotrichosis. 1. Ani*al handler $. Male &ender %. Alcoholis* '. 2ilipino or African decent (. 5enetic predisposition 1. 1: $ $. 1: % %. %: ' '. ': ( (. %: ' ). B $! Septate true hyphae are characteri;ed #y: 1. /rans3erse cross walls for*in& within the hyphae $. 0iscrete unicellular #odies %. Constrictions at septations '. Branchin& occurin& at septations (. A ter*inal cell that is s*aller than the others ). A $1 A 1$ year old #oy has a pruritic #ullous eruption on his feet. A K9F is positi3e and a fun&al culture shows *icroconidia in &rape"like clusters. /he etiolo&y is: 1. /richophyton 3errucosu* $. Microsporu* canus %. /richophyton *enta&rophytes '. Microsporu* &ypseu* (. /richophyton tonsurans ). C $$ /he *ost relia#le *ethod for distin&uishin& #etween /richophyton ru#ru* and /. *enta&rophytes is: 1. Morpholo&y of *icroconidia $. Morpholo&y of *acroconidia %. Pi&*entation studies 0 11$ '. Fair perforation test (. Colony *orpholo&y ). $% Culturin& /. ru#ru* fro* a white opacity on the 6n&ernail plate should pro*pt testin& for what. 1. 0ia#etes *ellitus $. Fypothyroidis* %. Fyperthyroidis* '. F1? (. Cirrhosis ). 0 $' ,hich actino*ycotic or&anis* has red &rains. 1. Strepto*yces so*aliensis $. Eocardia asteroides %. Actino*adura *adurae '. Actino*adura pelletieri (. Eocardia #rasiliensis ). 0 $( /his der*atophyte has a &rowth reLuire*ent for inositol and thia*ine 1. /richophyton eLuinu* $. /richophyton 3iolaceu* %. /richophyton concentricu* '. /richophyton tonsurans (. /richophyton 3errucosu* ). + $) ,hich der*atophyte is *ost co**only responsi#le for tinea corporis &ladiotoru*. 1. /. *enta& $. /. tonsurans %. /. schonlenleinii '. /. ru#ru* (. +. -occosu* ). B $A ,hich of the followin& state*ents re&ardin& *yceliu* is E9/ true. 1. A *ass of hyphae is *yceliu* $. Myceliu* has reproducti3e capa#ility %. Fyphae for*in& corkscrew"like turns are spiral hyphae '. Pectinate #odies are hyphae rese*#lin& a co*# (. Hacket for*s are clu#" shaped cells ). B $C ,hich of the followin& is a -uorescent ectothri< der*atophyte. 1. /richophyton ru#ru* $. /richophyton *enta&rophytes %. Microsporu* ferru&ineu* '. /richophyton 3iolaceu* (. Microsporu* nanu* ). C $D ,hich of the followin& is E9/ used for histolo&ic e<a*ination for fun&al infection. 1. Chlora;ol Black"+ $. 5or*ori Methana*ine Sil3er %. Periodic Acid SchiM '. 2ontana"Masson (. MayerGs *ucicar*ine ). A %! ,hich of the followin& state*ents re&ardin& histoplas*osis is /HB+. 1. /er#ina6ne is the treat*ent of choice C 11% $. Fistoplas*a capsulatu* is a yeast at $(JC %. /he or&anis*s are intracellular on histolo&ical e<a*ination '. /he disease is *ost co**on in the San 7oaLuin ?alley (. /he disease is pri*arily a *ucocutaneous infection ). %1 ,hich patho&en is not inhi#ited #y cyclohe<i*ide. 1. Cryptococcus $. Candida al#icans %. Prototheca '. Scopuloniopsos #re3icaulis (. Scytalidiu* species ). B %$ A landscape worker co*plained of se3eral tender nodules on the ri&ht dorsal hand and forear*. /he #iopsy of the lesion showed asteroid #odies #ut no or&anis*s. /he likely dia&nosis is: 1. Eocardiosis $. Blasto*ycosis %. Sporotrichosis '. Candidiasis (. Coccidioido*ycosis ). C %% All of the followin& state*ents re&ardin& super6cial *ycotic infections are true +OC+P/: 1. /richosporon species can cause syste*ic disease in i**unoco*pro*ised patients $. Malasse;ia furfur is the *ost co**on cause of tinea 3ersicolor %. /richosporon #ei&elii causes white piedra '. Malasse;ia species *ay #e associated with neonatal cephalic pustulosis (. Piedraia hortae causes #lack piedra ). B %' ,hich of the followin& state*ents a#out arthroconidia is correct. 1. Arthroconidia are for*ed #y #uddin& $. Arthroconidia are for*ed #y fra&*entation of hyphae %. Arthroconidia are thick" walled round cells '. Arthroconidia are spores that are produced in a sac (. Arthroconidia are yeast for*s of di*orphic fun&i ). B %( All of the followin& are features of *yceto*a e<cept: 1. Swellin& $. Self"li*ited %. 5ranules '. 2ascia and #one in3ol3e*ent B 11' (. 0rainin& sinuses ). %) ,hich of the followin& state*ents re&ardin& der*atophyte infection 2A8S+. 1. /richophyton schoenleinii is a co**on cause of fa3us $. Microsporu* canis is associated with ectothri< tinea capitis %. /richophyton 3iolaceu* is nor*ally associated with endothri< tinea capitis '. Microsporu* audouinii displays yellow -uorescence with ,oodJs la*p e<a*ination (. /richophyton ru#ru* is always an ectothri< infection ). + %A /he outstandin& characteristics of Asper&illus species on #iopsy include: 1. Blastoconidia $. Fyaline: septate dichoto*ously #ranchin& hyphae %. Copper pennies '. Hi##on"like 6la*ents that *ay #e twisted and distorted #ranchin& at ri&ht an&les (. Stain with 2ontana"Mason stain ). B %C ,hich one of the followin& is not caused #y a Candida infection. 1. An&ular cheilitis $. Balanitis %. Median rho*#oid &lossitis '. 8eukoplakia (. Myceto*a ). + %D Althou&h this or&anis* is not a fun&us: it stains with PAS and 5MS and produces spherules in tissue. /his or&anis* can #e identi6ed as: 1. Hhinosporidiu* see#eri $. Coccidioides i**itis %. Penicilliu* *arneMei '. Prototheca wickerha*i (. 8eish*ania *e<icana ). 0 '! A #iopsy shows #road"#ased #uddin& thick walled yeast cells: 1!"1( u* with a dou#le contoured appearance. 1. /his yeast has a yeast phase at roo* te*perature $. 0oes not &row at %AJ C %. Bsually produces a se3ere characteristic pul*onary disease '. May #e found in do&s (. 1s trans*itted #y *osLuitoes ). 0 '1 A ()"year"old *ale with F1? presents with 1. Cryptococcus + 11( *ultiple u*#ilicated #rown papules on the face. ,hich of the followin& is the least likely cause of his lesions. $. Fistoplas*osis %. Coccidio*ycosis '. Penicilliosis (. Blasto*ycosis ). '$ ,hich of the followin& causes tinea i*#ricata. 1. +pider*ophyton -occosu* $. /richophyton concentricu* %. /richophyton ru#ru* '. /richophyton *enta&rophytes (. /richophyton tonsurans ). B '% ,hich of the followin& stains is speci6c for chitin. 1. K9F $. K9F with 0MS9 %. Swart; 8a*kins '. Chlora;ol Black + (. Calco-uor ,hite ). 0 '' ,hich of the followin& *ethods of direct *icroscopic e<a*ination for fun&i is &lucan speci6c. 1. K9F $. Swart; 8a*kins %. Chlora;ol #lack + '. Calco-uor white (. MayerGs *ucicar*ine ). 0 '( /he or&anis* that causes white piedra: 1. 0oes not cause onycho*ycosis $. 1s known as Piedraia hortae %. May also cause post" operati3e wound infections '. 5rows as s*all: co*pact: #lack"&reenish 3el3ety colonies (. Can only #e treated #y cuttin& oM aMected hairs ). C ') Penicilliu* *arneMei is an infection ende*ic to which part of the world. 1. Me<ico $. South A*erica %. Africa '. Southeast Asia (. /he Cari##ean ). 0 'A Black &ranules are found in *yceto*a caused all the followin& or&anis*s e<cept: 1. Madurella &risea $. M. *yceto*atis %. +<ophiala 4eansel*ei '. Cur3ularia (. Scedosporiu* apiosper*u* ). + 'C A neutropenic patient has erythe*atous nodules for se3eral days. A #iopsy shows #ranchin& septate hyaline hyphae. ,hich of the followin& conditions should #e included in the diMerential. 1. Coccidioido*ycosis $. 2usariosis %. 1. 1 $. 1: $ %. $: % '. %: ' (. $: %: ( ). C 11) Asper&illosis '. Ky&o*ycosis (. Phaeohypho*ycosis 'D A patient returns fro* a 3acation in Bra;il with keloidal"like nodules on the face and ar*s. /he patient denies any sick contacts or e<posures and reports only lyin& on the #each and swi**in& with dolphins. She likely has: 1. Actino*ycosis $. 8o#o*ycosis %. Chro*o#lasto*ycosis '. Mucor*ycosis (. Sporotrichosis ). B (! /his or&anis* is the *ost co**on cause of eu*ycotic *yceto*a in the BS: 1. Madurella *yceto*atis $. Eocardia asteriodes %. Scedosporiu* apiosper*u* '. Phialophora 3errucosa (. ,an&iella der*atitis ). C (1 ,hich of the followin& or&anis*s will cause infections of skin: nails and endothri< hair. 1. +pider*ophyton -occosu* $. Microsporu* audouinii %. /richophyton schoenlinii '. Microsporu* canis (. /richophyton *enta&rophytes ). C ($ An or&anis* producin& an apricot colored colony and re-e<i3e #ranchin& with few conidia was cultured fro* a patient fro* Africa. ,hich of the followin& isare true. 1. /his *ostly likely is /. 3errucosu* $. /his *ost likely is /. soudanense %. /his *ost likely is /. 3iolaceu* '. /his or&anis* causes endothri< tinea capitis (. /his or&anis* is ;oophilic 1. 1:( $. $:' %. $:':( '. %: ' (. %: ': ( ). B (% Clinically: actino*ycotic *yceto*a and eu*ycotic *yceto*a appear identical. /he i*portance of identifyin& the etiolo&ic a&ent is 1. Acade*ic $. Selectin& the appropriate therapy %. 9rderin& appropriate stains '. 0eter*ine if the infection is conta&ious (. 0eter*ine if a*putation is necessary ). B (' ,hich of the followin& stat*ents re&ardin& pseudohyphae is incorrect. 1. Pseudohyphae are seen in yeasts $. Are constricted at septations %. Branchin& occurs at septations '. /he ter*inal cell is s*aller than the others (. Are not septated ). + (( ,hich of the followin& or&anis*s is *ost likely to cause a sporotrichoid nodule on the ar*: 1. Eocardia #rasiliensis $. Phialophora 3errucosa %. Hhinospiridiu* see#erii '. 2usariu* A 11A (. 2onsecaea pedrosoi ). () ,hich of the followin& der*atophytes causes endothri< tinea capitis. 1. Microsporu* &ypseu* $. Microsporu* audouinii %. /richophyton tonsurans '. Microsporu* canis (. Microsporu* ferru&ineu* ). C (A A crea*y white colony *i&ht #e any of these or&anis*s e<cept: 1. Candida al#icans $. Prototheca wickerha*ii %. Cur3ularia '. Sporothri< schenckii at %AJ C (. Cryptococcus neofor*ans ). C (C /his or&anis* &rew on Sa#ouraud 0e<trose a&ar without any anti*icro#ials. /he patient presented with onycho*ycosis of #oth &reat toenails. /he nails were dark and dystrophic: and thick scale was apparent on #oth feet. 1dentify the fun&us. 1. /richophyton ru#ru* $. Microsporu* distortu* %. Scytalidiu* di*idiatu* '. Coccidioides i**itis (. /richophyton 3iolaceu* ). C (D ,hat or&anis* produces round thick walled spiny *acroconidia and pear shaped *icroconidia. 1. Sporothri< schenckii $. Blasto*yces der*atitidis %. Paracoccidioides #rasiliensis '. Cryptococcus neofor*ans (. Fistoplas*a capsulatu* ). + )! Cutaneous lesions of Cryptococcosis *ay #e 1. Eodular $. Papular %. 5ranulo*atous"ulcerati3e '. Ferpetifor* (. Cellulitis"like: 1. $: %: ' $. %: ': ( %. $: %: ' '. 1: %: ': ( (. All of these answers are correct ). + )1 ,hat is the *ost freLuently reported cause of pri*ary cutaneous asper&illosis. 1. Asper&illus -a3us $. Asper&illus fu*i&atus %. Asper&illus ni&er '. Asper&illus solani (. Asper&illus *arneMei ). A )$ ,hich of the followin& state*ents re&ardin& candidal infection is 2A8S+. 1. Candida species displays true hyphae on potassiu* hydro<ide e<a*ination $. Predisposin& factors for candidal infection include dia#etes *ellitus: hyperhidrosis and #road spectru* anti#iotics %. Candida al#icans is the nu*#er one cause of *ucocutaneous infections '. Candidal infections typically do not spare the scrotu* A 11C (. Candida species *ay #e associated with &ranulo*a &luteale infantu* ). )% ,hich of the followin& is the *ost useful *orpholo&ic feature in identifyin& the *ycelial phase of Fistoplas*a capsulatu*. 1. Arthroconidia in e3ery other cell $. +ncapsulated spores $"( u* %. /u#erculate *acroconidia C"1' u* '. S*all o3al conidia on lon& thin conidiophores (. Microconidia laterally alon& the hyphae strand ). C )' A 'D"year"old *an has painless su#cutaneous nodules on his feet with sinus tracts and a#scesses. Patholo&y shows I&rainI in sinus tract draina&e. A dia&nosis of eu*ycotic *yceto*a is *ade. ,hat is the *ost co**on cause in the Bnited States. 1. Acre*oniu* $. Cur3alaria %. +<ophilia 4eansel*ei '. Pseudallescheria #oydii (. Eocardia ). 0 )( 9f the followin& Candida species: which is likely to cause disse*inated Candidiasis with cutaneous lesions. 1. Candida al#icans $. C. parapsilosis %. C. tropicalis '. C. krusei (. C. neofor*ans ). C )) A %A year old wo*an fro* Eew Me<ico: now %! weeks pre&nant: presents with -u" like sy*pto*s. Chest <"ray re3ealed diMuse *iliary in6ltrates: with #lood cultures &rowin& Coccidioidis i**itis. ,hat is the treat*ent of choice. 1. A*photericin B $. /er#ina6ne %. 1tracona;ole '. 5riseoful3in (. Eo therapy ). A )A A pet store owner co*es into your oTce with pruritic scaly plaLues on his ar*s. A fun&al culture de*onstrates a yellow colony with spindle"shaped *acroconidia which &rows on polished rice &rains. /he or&anis* is: 1. Microsporu* canis $. +pider*ophyton -occosu* %. /richophyton ru#ru* '. Microsporu* &ypseu* (. /richophyton tonsurans ). A )C ,hich of the followin& is true re&ardin& culture of the or&anis*s that cause Pityriasis 3ersicolor. 1. 0iTcult to &row: reLuires oli3e oil o3erlay $. 5row easily on Mycosel *edia %. Cannot #e cultured '. 5row easily on 0er*atophyte /est Media (. 5row easily on Sa#ouraud 0e<trose A&ar ). A )D ,hich of the followin& is *ost likely to #e a pri*ary cutaneous infection. 1. Eorth A*erican Blasto*ycosis $. Fistoplas*osis %. Paracoccidioido*ycosis '. Cryptococcosis A 11D (. Penilliosis ). A! A %'"year"old *ale patient presents with a 3errucous lesion of the nasal *ucosac the #iopsy showed spherules ran&in& in si;e fro* $!! to %$( u*. /he *ature endospores ha3e a rou&h appearance. /he dia&nosis is: 1. Coccidioido*ycosis $. Protothecosis %. Hhinosporidiosis '. Pseudallescheriosis (. Cryptococcosis ). C A1 ,hich of the followin& is 2A8S+ with re&ards to ase<ual reproduction of fun&i. 1. Arthroconidia are for*ed #y the fra&*entation of hyphae $. Sporan&ia are spores that are produced in a sac %. Chla*ydoconidia are thin" walled and are suscepti#le to en3iron*ental in4ury '. Conidia are cells produced on the sides or ends of hyphae (. 0e*atophytes produce conidia ). C A$ /his a&ent causes a super6cial: asy*pto*atic infection usually on the pal*s of the hands. /he lesions are -at: nonscaly and appear as irre&ularly shaped #rown *acules: 1. Piedra hortae $. Cladosporiu* carrionii %. Aureo#asidiu* pullulans '. Phaeoannello*yces werneckii (. +<ophiala 4eansel*ei ). 0 A% ,hich of the followin& is true re&ardin& 0er*atophyte /est Media =0/M>. 1. 0/M contains chlortetracycline and *inocycline $. Ali;arin red is the indicator present in 0/M %. Eon"der*atophytes cause the *edia to turn yellow due to acid #yproducts '. 0/M is useful for culturin& der*atophytes fro* skin and nails: #ut not hair. (. 0er*atophytes utili;e &lucose as a car#on source: producin& alkaline #y"products. ). C A' Choose the correct state*ent re&ardin& Coccidioido*ycosis: 1. /he *ost co**on for* of pri*ary inoculation is cutaneous $. 0isse*ination *ay in3ol3e the #ones: 4oints: 3iscera: #rain and skin %. Causati3e or&anis*: C. i**itits: is a thick"walled spherule with a polysaccharide capsule B 1$! de*onstrated with Alcain #lue '. +rythe*a nodosu* is a poor pro¬ic si&n (. 9ut#reaks occur in the Mississippi and 9hio Hi3er ?alley ). A( ,hich one of the followin& a&ents accounts for the depi&*entation seen in pityriasis 3ersicolor. 1. /hy*idine kinase $. Ketocona;ole %. Acetone '. 0icar#ocyclic acid (. Postin-a**atory eMect ). 0 A) ,hich of the followin& is not true re&ardin& fun&al culture *edia containin& cyclohe<i*ide. 1. Cyclohe<i*ide is not found in Sa#ouraud 0e<trose A&ar =S0A> +**ons Modi6cation $. Cyclohe<i*ide is found in Mycosel %. Cyclohe<i*ide is found in 0er*atophyte /est Media '. Cyclohe<i*ide is found in Myco#iotic (. Cyclohe<i*ide inhi#its #acterial -ora ). + AA Septate hyphae with D!J #ranchin& and thick walled #arrel shaped arthroconidia alternatin& with e*pty cells #est descri#es the *icroscopic *orpholo&y of: 1. Fistoplas*a capsulatu* $. Sporothri< schenckii %. Microsporu* &ypseu* '. /richophyton tonsurans (. Coccidioides i**itis ). + AC /inea i*#ricata is *ost freLuently caused #y: 1. /. *enta&rophytes $. M. Audouinii %. /. concentricu* '. +. -occosu* (. M. furfur ). C AD ,hat is the *ost likely cause of this infection. 1. /. tonsurans $. /. schoenleinii %. M. canis '. /. *enta& (. /. ru#ru* ). C C! A patient has a positi3e ,oodGs li&ht e<a* that is caused #y pteridine. ,hat is the causati3e condition. 1. Microsporu* ferru&ineu* $. Coryne#acteriu* %. /richophyton 3iolaceu* '. Pseudo*onas (. /richophyton tonsurans ). A C1 ,hich of the followin& stains is E9/ used to stain fun&al ele*ents. 1. Mucicar*ine $. Periodic Acid SchiM %. 2ontana"Masson '. ?on Kossa 0 1$1 (. 5or*ori *ethena*ine sil3er ). C$ A *ass of hyphae is known as a: 1. Sporan&ia $. Conidia %. Myceliu* '. Chla*ydoconidia (. Pseudohyphae ). C C% /he *ost sensiti3e *icroscopic test for fun&al infection is: 1. Potassiu* Fydro<ide $. Potassiu* Fydro<ide with 0MS9 %. Chlora;ol Black + '. Calco-uor white (. Swart; 8a*kins stain ). 0 C' ,hich of the followin& state*ents re&ardin& lo#o*ycosis is 2A8S+. 1. 1tracona;ole is the treat*ent of choice $. 1t rese*#les a Jchain of coinsJ on histopatholo&y %. 1t is also known as Jkeloidal #lasto*ycosisJ '. 8aca;ia =for*erly 8o#oa> lo#oi is the nu*#er one cause (. /he infection also occurs in dolphins ). A C( +rythe*atous to 3iolaceous papules that *ay pro&ress to nodular and necroti;in& skin lesions in neutropenic patients *ay #e caused #y which of the followin& or&anis*s. 1. /richosporon asahii $. /richosporon #ei&elii %. /richosporon o3oides '. /richosporon inkin (. /richophyton ru#ru* ). A C) A child presents with se3eral yellowish: cup"shaped crusts on the scalp: so*e with sin&le hairs piercin& throu&h the center. K9F prep re3eals arthroconidia and airspaces within the hair shaft. A likely cause is: 1. M. &ypseu* $. M. canis %. /. tonsurans '. /. 3errucosu* (. /. *enta&rophytes ). A CA /his or&anis* produces an endothri< tinea capitis: 1. /richophyton *enta&rophytes $. Microsporu* &ypseu* %. Microsporu* nanu* '. /richophyton 3errucosu* (. /richophyton soudanense ). + CC ,hich structure is found in a #iopsy of Candida tropicalis: it is #ranchin& and pinchin& in at the points of septations: 1. Septate hyphae $. Arthroconidia %. Mosaic fun&us '. Pseudohyphae (. Pectinate #odies ). 0 CD Cryptococcus neofor*ans resides in: 1. /he Mississippi ?alley re&ion C 1$$ $. Chicken roosts %. Pi&eon droppin&s '. Sandy soil (. Eone of these answers are correct ). D! A #lood culture fro* a neutropenic patient with onycho*ycosis &rew which of the followin& or&anis*: 1. Scopulariopsis sp $. Asper&illus sp %. 2usariu* sp '. Acre*oniu* sp (. /. ru#ru* ). C D1 A ) year"old Fispanic &irl ca*e into the clinic with a history of alopecia and scaly scalp for % weeks. /he ,oodGs la*p e<a*ination was ne&ati3e and her cer3ical ly*ph nodes were not enlar&ed. 1t was noted that the hairs were #roken oM at the surface of the scalp. /he *ost likely or&anis* to #e isolated would #e: 1. M. audouinii $. M. canis %. M. &ypseu* '. /. tonsurans (. /. ru#ru* ). 0 D$ /his or&anis* does not produce *icroconidia. /he *acroconidia are clu# shaped: and s*ooth walled: they &row sin&ly or in clusters. 1. /richophyton ru#ru* $. +pider*ophyton -occosu* %. Microsporu* &ypseu* '. Microsporu* canis (. /richophyton *enta&rophytes ). B D% A solitary chancre"like lesion appeared on the ar* of a -orist who has a pet do&. Eo or&anis*s were seen on #iopsy: howe3er Sporothri< schenckii was cultured fro* the tissue. /his infection is known as J6<ed cutaneous sporotrichosisJ. 1t re*ains 6<ed #ecause: 1. /here is a resistance due to a prior e<posure $. /here is a decreased resistance due to A10S %. /he infection was acLuired fro* the do& rather than fro* a plant '. /he infection occurred in the a#sence of tissue in4ury (. /his species of Sporothri< has li*ited infecti3ity ). A D' /he *ost co**on eu*ycotic or&anis* in the BS produces which color &rains in its *icrocolony. 1. ,hite $. Black %. Hed '. Sellow (. 5reen ). A D( ,hich of the followin& *ethods of direct *icroscopic e<a*ination is chitin speci6c. 1. Chlora;ol #lack + $. Calco-uor white %. 5o*ori Methena*ine Sil3er '. 2ontana"Masson (. K9F ). A D) /he dia&nosis is phaeohypho*ycosis: what is the or&anis* 1. Alternaria sp. $. 1. 1:$:% $. $:%:' C 1$% Bipolaris sp. %. 2usariu* sp. '. +<ophiala sp. %. 1:$:' '. 1:%:' (. All of these answers are correct ). DA A patient with a pul*onary infection and cer3ical adenopathy has 3esicles: papules and ulcerations on the oral and nasal *ucosa. /issue culture re3eals yeast with *ultiple #uds &i3in& a J*arinerJs wheelJ appearance. /he or&anis* is: 1. Penicilliu* *arneMei $. Coccidioides i**itis %. Paracoccidioides #rasiliensis '. Blasto*yces der*atitidis (. 8eish*ania *e<icana ). C DC ,hich of the followin& *ost accurately descri#e conidia. 1. Cells produced on the end or sides of hypha or conidiophore $. Spores that are produced in a sac %. /hick"walled round cell '. 2or*ed #y #uddin& (. 2or*ed #y fra&*entation of hyphae ). A DD ,hich of the followin& state*ents a#out di*orphic fun&i is 1EC9HH+C/. 1. Are in the *ould for* in the en3iron*ent $. Are in yeastspherule for* in tissue %. Are in *ould for* at $( de&rees C '. Are in yeastspherule for* at %A de&rees C (. Are in *ould for* in tissue ). + 1! ! A '( year"old a&ricultural worker fro* Bra;il presented with ulcers of the #uccal *ucosa and ton&ue. Cer3ical ly*ph nodes were tender and enlar&ed. /he #iopsy would *ost likely re3eal: 1. Seast cells in chains and a lar&e thick walled round central yeast cell surrounded #y se3eral thinly attached #uddin& s*aller yeast cells $. Seast cells with lar&e capsules %. S*all #uddin& yeast cells '. Seast cells with pseudohyphae (. Copper pennies ). A 1! 1 /he *ost co**on cause of tinea capitis in the Bnited States today is: 1. /richophyton schoenleinii $. /. tonsurans %. /. *enta&rophytes '. Microsporu* audouinii (. M. canis ). B 1! $ /he etiolo&ic a&ent responsi#le for ,hite Piedra is: 1. Candida al#icans $. Pityrosporu* o#iculare %. Coryne#acteriu* tenuis '. Piedra hortai + 1$' (. /richosporon o3oides ). 1! % An elderly lady with *occasin"type tinea pedis has a fun&al culture which de*onstrates s*ooth: teardrop"shaped *icroconidia which produce a Jport" wineJ pi&*ent. /he or&anis* is: 1. /richophyton 3errucosu* $. Microsporu* canus %. /richophyton ru#ru* '. Microsporu* &ypseu* (. /richophyton *enta&rophytes ). C 1! ' A sli*y: *ucoid colony &rowin& on Sa#ouraudGs a&ar at %A de&rees Celsius is o#ser3ed to produce urease. /he or&anis* is: 1. Candida al#icans $. Cryptococcus neofor*ans %. Candida &la#rata '. Candida tropicalis (. Asper&ilus -a3us ). B 1! ( Eu*erous u*#ilicated nodules rese*#lin& *olluscu* conta&iosu* de3eloped in a patient with recently dia&nosed F1? infection. ,hich of the followin& fun&i *i&ht #e the causati3e a&ent=s>. 1. Candida tropicalis $. Cryptococcus neofor*ans %. Asper&illus fu*i&atus '. Fistoplas*a capsulatu* (. Penicilliu* *arneMei 1. $ $. % %. $: ' '. ': ( (. $: ': ( ). + 1! ) /wo weeks after a #icycle accident a $(" year"old fe*ale dia#etic patient co*plained of a swollen tender ri&ht cheek /he PAS #iopsy de*onstrated rin& for*s and distorted wide hyphae with few septations and ri&ht an&le #ranchin&. /he dia&nosis is: 1. Asper&illosis $. Actino*ycosis %. Mucor*ycosis '. 0er*atophytosis (. 2usariosis ). C 1! A /his or&anis* causes a resistant tinea pedis indistin&uisha#le fro* der*atophytosis: 1. Asper&illus -a3us $. Scytalidiu* di*idiatu* %. Cur3ularia sp. '. Scopulariopsis (. 2onsecaea sp ). B 1! C ,hich of the followin& is /HB+ re&ardin& coccidio*ycosis. 1. 2irst line treat*ent in pre&nancy is itracona;ole $. 0roplet trans*ission is the *ost co**on *ethod of acLuisition of disease %. Southeast Asians are at a hi&her risk of disse*inated disease '. +rythe*a nodosu* is associated with a poor pro&nosis (. Appro<i*ately A(N of those contractin& the disease will #e sy*pto*atic C 1! D A horticulturist of spha&nu* *oss topiaries co*es in with a nodular eruption with ly*phan&itic spread and treat*ent 1. 5astrointestinal distress $. Shortness of #reath %. 2lushin& A 1$( with oral potassiu* iodide is initiated. ,hat is the *ost well reco∋ed side eMect of this treat*ent. '. An&ioede*a (. Pruritus 11 ! A whitish: heaped and con3oluted colony with &rowth su#*er&ed into the a&ar and a colorless re3erse was isolated fro* the scalp of a %("year"old *ale. /he or&anis* did not produce any conidia. /he dia&nosis *ost likely is: 1. Microsporu* canis $. Microsporu* ferru&ineu* %. /richophyton ru#ru* '. /richophyton tonsurans (. /richophyton schoenleinii + 11 1 A patient with scaly feet has a positi3e K9F. 2un&al culture re3eals s*ooth: clu#" shaped *acroconidia attached to hyphae in &roups. Eo *icroconidida are seen. /he or&anis* is: 1. Microsporu* canis $. +pider*ophyton -occosu* %. /richophyton ru#ru* '. Microsporu* &ypseu* (. /richophyton tonsurans B 11 $ Seast: 1. Are 6la*entous fun&i $. Are characteri;ed #y tu#ular #ranchin& cells %. 2or* fu;;y colonies '. 2or* s*ooth colonies (. Are unicellular o3al to round cells that reproduce #y #uddin& or 6ssion ). + 11 % A youn& &irl presented with a scaly annular facial rash and alopecia of her lower eyelashes. ,hich of the followin& state*ents isare true. 1. A K9F prep and fun&al culture *i&ht con6r* the dia&nosis. $. /he etiolo&ic a&ent *i&ht #e Microsporu* canis. %. /he fa*ily puppy *i&ht #e infected. '. 5riseoful3in would #e the dru& of choice. (. A topical a;ole crea* would #e the dru& of choice. 1. 1:$:' $. 1:$:%:' %. 1:%:' '. 1:$:( (. 1:$:%:( B 11 ' /his der*atophyte is not an anthropophilic or&anis*: 1. /richophyton ru#ru* $. +pider*ophyton -occosu* %. Microsporu* &ypseu* '. Microsporu* audouinii (. /richophyton soudanense C 11 ( A %! year"old *ale li3in& in the Chica&o su#ur#s co*plained of a slowly &rowin& 3errucous plaLue with sharp #orders on his left wrist. A #iopsy re3ealed yeast cells 1!"1' u*. Mucicar*ine was ne&ati3e. A -uMy white colony &rew at roo* te*perature ha3in& s*all round conidia on thin conidiophores. /he dia&nosis is: 1. Blasto*ycosis $. Cryptococcosis %. Candidiasis '. South A*erican Blasto*ycosis (. Fistoplas*osis A 11 ) Medlar #odies are dia&nostic of infection with which or&anis*. 1. Blasto*ycosis $. Chro*o*ycosis %. Coccidio*ycosis '. Fistoplas*osis (. Sporotrichosis B 11 A ,hich of the followin& causes I#lack dot rin&wor*I. 1. M. canis $. /. 3iolaceu* %. /. 3errucosu* '. M. &ypseu* B 1$) (. M. auddouinii 11 C All of the followin& are co**on causes of chro*o#lasto*ycosis +OC+P/: 1. Cladosporiu* carrionii $. Hhinocladiella aLuaspera %. Phialophora 3errucosa '. Pseudallescheria #oydii (. 2onsecaea pedrosi 0 11 D ,hich of the followin& or&anis*s causes fa3us. 1. /richophyton schoenleinii $. /richophyton *enta&rophytes %. Microsporu* canis '. /richophyton ru#ru* (. Microsporu* distortu* A D. Pediatric 0er*atolo&y # Question Answers Correc t Answe r 1 ,hich treat*ent choice would #e contraindicated in a one"year old child who presents with *ono*orphous: nonpruritic -at" topped papules on the face: #uttocks: e<tre*ities: pal*s and soles. 1. Ad3il $. Aceta*inophen %. Fydration '. Corticosteroids (. 9#ser3ation ). Show +<plaination 0 $ ,hat is the *ost likely dia&nosis. 1. Psoriasis $. Pityriasis rosea %. Cutaneous /"cell ly*pho*a '. Contact der*atitis (. 8ichen planus ). Show +<plaination 0 % An infant who presents with this a#nor*ality will often ha3e the followin&: 1. /he lesion will #e on the left" side in a se&*ental distri#ution $. Airway restriction %. Fa3e *ultiple li3er he*an&io*as '. Most likely #e a *ale infant (. An anterior fossa *alfor*ation ). Show +<plaination A ' 8ate onset su#un&ual keratotic tu*ors are associated with: 1. 1ncontinentia pi&*enti $. Eeuro6#ro*atosis /ype 1 %. Carney co*ple< '. Cowden syndro*e (. Basal cell ne3us syndro*e ). Show +<plaination A ( PastiaJs lines are characteristic for which eruption: 1. Measles $. Scarlet fe3er %. Hu#ella '. KawasakiJs disease (. Mu*ps B 1$A ). Show +<plaination ) All four su#types of Phako*atosis Pi&*ento3ascularis ha3e which feature in co**on: 1. Ee3us spilus $. +pider*al ne3i %. Ee3us -a**eus '. Ee3us ane*icus (. 0er*al Melanocytosis. ). Show +<plaination C A ,hat is the #est treat*ent option. 1. 9ral cephale<in $. 9ral acyclo3ir %. 9#ser3ation '. /opical tretinoin (. /opical ketocona;ole ). Show +<plaination 0 C ?ariants of <eroder*a pi&*entosu* are due to all of the followin& defects e<cept: 1. Eucleotide e<cision repair $. Felicase %. +ndonuclease '. /hy*idine kinase (. Postrepliction repair ). Show +<plaination 0 D ,hich of the followin& can present as collodion #a#y. 1. 1chthyosis 3ul&aris $. O"linked ichthyosis %. 8a*ellar ichthyosis '. Bullous con&enital ichthyosifor* erythroder*a (. S4o&ren"8arsson syndro*e ). Show +<plaination C 1! A ) *onth"old presents with oran&e" #rown crusted plaLues around the *outh and &roin. Se3eral #ullae are present on the 6n&ers and toes. ,hich of the followin& la#oratory 3alues is likely to #e a#nor*al. 1. Fe*atocrit $. Calciu* %. Platelet count '. A8/ (. Alkaline phosphatase ). Show +<plaination + 11 Psa**o*atous *elanotic schwanno*as are associated with: 1. Bloo* syndro*e $. Carney co*ple< %. Eeuro6#ro*atosis /ype 1 '. Eeuro6#ro*atosis /ype $ (. /u#erous sclerosis ). Show +<plaination B 1$ ,hat is the #est test to con6r* a dia&nosis. 1. 0s0EA anti#ody $. Anti Ho anti#ody %. Biopsy '. K9F (. 5ra* stain ). Show +<plaination 0 1% 7u3enile self"healin& papular *ucinosis is associated with which of the 6ndin&s. 1. Arthral&ias $. Cataracts %. /ype 1 dia#etes '. Calcinosis cutis (. Oeropthal*ia ). Show +<plaination A 1' ,hich of the followin& clinical si&ns is *ost likely associated. 1. Clitoral hypertrophy $. Eeuro6#ro*as %. A<illary frecklin& '. Fypertrichosis (. Alopecia ). Show +<plaination A 1( A full ter* neonate is noted to ha3e s*all pustules with no underlyin& erythe*a present at deli3ery. /he 1. Miliaria $. +rythe*a to<icu* neonatoru* C 1$C pustules are easily re*o3ed with clearin& of the 3erni< and a collarette appears. A &ra* stain is done showin& predo*inately neutrophils without #acteria. ,hat is the *ost likely dia&nosis. %. /ransient neonatal pustular *elanosis '. Con&enital candidiasis (. Brticaria pi&*entosa ). Show +<plaination 1) ,hat syndro*e is the disorder shown in the photo associated with. 1. /urner syndro*e $. 0own syndro*e %. Eoonan syndro*e '. 5riscelli syndro*e (. Bloo* syndro*e ). Show +<plaination C 1A A #oy is noted at #irth to ha3e coarse scales o3er his trunk and e<tre*ities. /he face: pal*s: soles and -e<ures are spared. ,hat is the least likely association. 1. Corneal opacities $. Cryptorchidis* %. +ctropion '. Prolon&ed *aternal la#or (. Eeurolo&ic a#nor*ality ). Show +<plaination C 1C ,hat is the *ost likely dia&nosis. 1. Psoriasis $. 8an&erhans cell histiocytosis %. 5ranulo*a &luteale infantu* '. Contact der*atitis (. Perianal streptococcal disease ). Show +<plaination + 1D ,hat is the *ost co**on tu*or associated with this condition. 1. /richo#lasto*a $. SLua*ous Cell carcino*a %. Se#aceous carcino*a '. Basal cell carcino*a (. /richoadeno*a ). Show +<plaination A $! JSlapped cheeksJ followed #y a lacy eruption on e<tre*ities: 1. ss0EA 3irus $. ds0EA 3irus %. ssHEA 3irus '. dsHEA 3irus (. Streptococcus ). Show +<plaination A $1 A patient presents with an epider*al ne3us of the lower a#do*en. Sou take a skin #iopsy to con6r* the dia&nosis. /he patholo&y report indicates that there were 6ndin&s of epider*olytic hyperkeratosis. ,hich of the followin& defects is her oMsprin& at risk for #ased on these 6ndin&s. 1. /rans&luta*inase $. Keratin )a1) %. Keratin )#1A '. Keratin 11! (. Keratin $e ). Show +<plaination 0 $$ Koplik spots typically appear: 1. Before the e<anthe* $. At the sa*e ti*e as the e<anthe* %. 1 week after the e<anthe* '. ) weeks after the e<anthe* (. C weeks after the e<anthe* ). Show +<plaination A $% /his condition *ay #e associated with which of the followin&: 1. Fypercalce*ia $. Fyperlipide*ia %. ?itili&o '. +pider*olysis #ullosa dystrophica (. Perinatal trau*a ). Show +<plaination 0 1$D $' An infant presents with the lesion depicted in the photo. ,hich of the followin& is least likely. 1. Sternal cleftin& $. Suprau*#ilical raphe %. 0andy",alker *alfor*ation '. Sei;ure disorder (. Con&enital cataracts ). Show +<plaination 0 $( An C year"old #oy presents with pink: -ushed cheeks and a low"&rade fe3er. 9n week later: the followin& lacy eruption appeared. ,hat is the *ost likely etiolo&y. 1. Para*y<o3irus $. /o&a3irus %. 5roup A streptococcus '. FF?) (. Par3o3irus ). Show +<plaination + $) ,hat is the *ost likely dia&nosis is this 1) year old patient who de3eloped &enerali;ed eruption $ weeks after onset of sore throat due to strep infection. 1. Psoriasis $. Pityriasis rosea %. Cutaneous /"cell ly*pho*a '. Contact der*atitis (. 8ichen planus ). Show +<plaination A $A A youn& &irl presents with enlar&ed ton&ue: e<o*phal*os: and or&ano*e&aly and has history of ,il*Gs tu*or. ,hat cutaneous 6ndin& is *ost likely on physical e<a*. 1. Pal*oplantar keratoder*a $. 8y*phatic *alfor*ation %. Acral ede*a '. Midline capillary *alfor*ation (. An&iokerato*a ). Show +<plaination 0 $C ,hat is the *ost likely dia&nosis. 1. Psoriasis $. 8an&erhans cell histiocytosis %. Se#orrheic der*atitis '. Contact der*atitis (. Perianal streptococcal disease ). Show +<plaination A $D P9+MS syndro*e is associated with which of the followin&: 1. Pre*ature a&in& $. 9donto&enic cysts %. +ye a#nor*alities '. M protein (. Saddle nose defor*ity ). Show +<plaination 0 %! ,hich presentation of psoriasis is *ore co**on in children: 1. Pustular psoriasis $. Acroder*atitis continua of Fallopeau %. Keratoder*a #lennorra&ica '. +rythroder*ic psoriasis (. 5uttate psoriasis ). Show +<plaination + %1 ,hat is the *ost likely dia&nosis. 1. 1ncontinentia pi&*enti $. Bullous pe*phi&oid of infancy %. Ferpes ;oster '. 0isse*inated herpes si*ple< (. +pider*olysis #ullosa si*ple< ). Show +<plaination A %$ A % *onth"old &irl with *ultiple he*an&io*as alon& her ri&ht 4aw is at increased risk for: 1. Bleedin& co*plications $. Bnderlyin& #one a#nor*alities %. Su#&lottic he*an&io*a '. 9ral o#struction (. Fearin& defecits ). Show +<plaination C 1%! %% ,hich of the followin& *ay #e associated. 1. Paronychia $. Cleft palate %. A?M '. Sei;ure disorder (. Atrial septal defect ). Show +<plaination 0 %' ,hat is the #est therapeutic option. 1. 9ral cephale<in $. /opical i**une *odulator %. 9ral acyclo3ir '. /opical *upirocin (. 9ral prednisone ). Show +<plaination C %( Happ"Fod&kin is caused #y a defect in the followin& &ene: 1. Plakophilin $. 0es*o&lein 1 %. +ctodysplasin A '. P)% (. Conne<in %! ). Show +<plaination 0 %) An ei&ht year"old #oy presents with pink: -ushed cheeks and a low"&rade fe3er. +ruptions then appeared. ,hat is the *ost likely etiolo&y. 1. Para*y<o3irus $. /o&a3irus %. 5roup A streptococcus '. FF?) (. Par3o3irus ). Show +<plaination + %A ,hat de6ciency is responsi#le for this condition. 1. /hia*ine $. Eiacin %. Kinc '. ?ita*in C (. ?ita*in A ). Show +<plaination C %C A new#orn presents with a pustular rash: 4oint swellin&: oral *ucosal lesions: and pain with *o3e*ent. 93er ti*e: cutaneous pustulosis: ran&in& fro* discrete crops of pustules to &enerali;ed se3ere pustulosis and ichthyosifor* lesions de3elop. 1n addition the new#orn de3elops sterile *ultifocal osteo*yelitis and periostitis. ,hat is this condition. 1. Muckle",ells syndro*e $. 0e6ciency of the interleukin" 1"receptor anta&onist =01HA> %. 2a*ilial Mediterranean fe3er '. Fyper"1&0 syndro*e (. PAPA syndro*e ). Show +<plaination B %D ,hat is the dia&nosis. 1. Bnilateral laterothoracic e<anthe* $. Hoseola %. Hu#ella '. Brticaria (. Contact der*atitis ). Show +<plaination A '! Pachyonychia con&enita type $ is *ost co**only associated with which of the followin&: 1. 1ncreased risk of *ali&nancy $. Poikiloder*a %. Eatal teeth '. Aplastic nails (. 0eafness ). Show +<plaination C '1 ,hat is the #est therapeutic option. 1. 9ral cephale<in $. /opical i**une *odulator %. 9ral acyclo3ir '. /opical *upirocin (. 9ral prednisone ). Show +<plaination C 1%1 '$ Posterior auricular adenopathy is a co**on feature of which e<anthe*: 1. Measles $. Mu*ps %. Scarlet fe3er '. Hu#ella (. +rythe*a infectiosu* ). Show +<plaination 0 '% C1) acti3atin& *utation in c"kit are found *ost often in which su#set of patients with *astocytosis. 1. Adults with syste*ic disease refractory to i*antini# $. Adults with syste*ic disease associated with eosinophilia %. Patients with fa*ilial history of *astocytosis '. Adults with /elan&iectasia Macularis +rupti3a Perstans (. As a *osaic *utation in children with solitary cutaneous *astocyto*a ). Show +<plaination A '' A de6ciency of the surface &lycoprotein sialophorin is seen in which i**unede6cient disease. 1. ,iskott"Aldrich syndro*e $. Chronic &ranulo*atous disease %. 7o# syndro*e '. Se3ere co*#ined i**unode6ciency syndro*e (. 8einerJs disease ). Show +<plaination A '( A healthy: full"ter* infant de3elops a pustular: erythe*atous eruption on her face and trunk on the third day of life. A s*ear taken fro* one of these pustules would show: 1. 5ra*"positi3e #acteria $. Predo*inantly neutrophils %. Multi"nucleated &iant cells '. Predo*inantly eosinophils (. Fyphae ). Show +<plaination 0 ') ,hat is the *ost co**on tu*or associated with this condition. 1. /richo#lasto*a $. Syrin&ocystadeno*a papilliferu* %. Se#aceous carcino*a '. Basal cell carcino*a (. /richoadeno*a ). Show +<plaination A 'A Multiple cylindro*as are associated with: 1. Myotonic dystrophy $. Cowden syndro*e %. Carney co*ple< '. /richoepithelio*as (. Pilo*atricho*as ). Show +<plaination 0 'C ,hat is the *ost likely dia&nosis. 1. Papular acroder*atitis of childhood $. Mucocutaneous ly*ph node syndro*e %. 5er*an *easles '. 8etterer"Siwe disease (. +<anthe* su#itu* ). Show +<plaination A 'D 1n a child with ;inc de6ciency: yet nor*al or near nor*al ;inc le3els: which test could #e a 3alua#le ad4uncti3e test. 1. Ma&nesiu* $. Eiacin %. Man&anese '. Alkaline phosphatase (. 1ron ). Show +<plaination 0 (! A 1! year old &irl presents with 1. Henal ultrasound B 1%$ desLua*ation of the 6n&ertips. ,hich e<a* should #e ordered. $. AS9 titer %. ++5 '. +ye e<a* (. Chest <"ray ). Show +<plaination (1 A )"year"old #oy presents with osteo*a cutis on his face. ,hich of the followin& &enes is *ost likely defecti3e. 1. 5EAS $. ES0F8 %. P/+E '. SP1EK( (. S8BHP1 ). Show +<plaination A ($ 9steopathia striata is found in which disorder. 1. McCune"Al#ri&ht syndro*e $. Eeuro6#ro*atosis 1 %. Buschke"9llendorM syndro*e '. 5orlinGs syndro*e (. 2ocal der*al hypoplasia ). Show +<plaination + (% /his patient had si&ni6cantly ele3ated seru* CPK. /he likely dia&nosis is: 1. 0er*ato*yositis $. 8upus erythe*atosus %. Psoriasis '. Atopic der*atitis (. 8ichen planus ). Show +<plaination A (' /he causati3e a&ent of Hoseola is: 1. A ss0EA 3irus $. A ds0EA 3irus %. A ssHEA 3irus '. A dsHEA 3irus (. Streptococcus ). Show +<plaination B (( Eeonatal acne is associated with species of which or&anis*: 1. Staphylococcus $. Propioni#acteriu* %. Candida '. Malasse;ia (. Streptococcus ). Show +<plaination 0 () A patient presents with *ultiple 4u3enile <antho&ranulo*as: a<illary frecklin&: *ultiple cafJ"au"lait *acules: three neuro6#ro*as and a fa*ily history of E2"1. ,hat other condition is this patient at increased risk for. 1. AM8 $. CM8 %. C88 '. Medullo#lasto*a (. Pancreatic carcino*a ). Show +<plaination B (A An infant presents with the lesion depicted in the photo. ,hat infor*ation do you pro3ide the parents re&ardin& this condition. 1. /he lesion will &o throu&h rapid &rowth followed #y sta#ili;ation and re&ression $. /he lesion will respond to laser therapy %. /he lesion will persist and *ay &row further '. /he lesion will resol3e with antifun&al therapy (. /he lesion is associated with a O9 karyotype ). Show +<plaination C (C /he lesion depicted is *ost often associated with: 1. Polyostotic 6#rous dysplasia $. Chondrodysplasia punctata %. Sphenoid win& hypoplasia '. Cleft palate (. 9steopoikilosis ). Show +<plaination A 1%% (D ,hich of the followin& #ullous disorders is due to a tar&et anti&en that is a DA k0 protein which is a *e*#er of BPA5"$. 1. Bullous 0er*olysis of the Eew#orn $. Eeonatal Pe*phi&us %. 8inear 1&A Bullous 0er*atosis '. 7unctional epider*olysis #ullosa =Ferlit;> (. +pider*olysis #ullosa si*ple< ). Show +<plaination C )! ,hat is the *ost co**on cause of neonatal purpura ful*inans. 1. Strep $. Staph %. ?aricella '. Protein C de6ciency (. 2actor ? 8eiden de6ciency ). Show +<plaination 0 )1 A new#orn infant presents with rin& of lon&: dark: coarse hair surroundin& a *idline scalp patch of alopecia. ,hat is associated with this 6ndin&. 1. Alopecia areata $. +ctopic #rain tissue %. /hyroid disease '. Ee3us se#aceus of 7adassohn (. 0eafness ). Show +<plaination B )$ Schi**elpennin&"2euerstein"Mi*s syndro*e *ay #e associated with which of the followin&: 1. 9steopokilosis $. Polyostotic 6#rous dysplasia %. 9steopathia striata '. Chondrodysplasia punctata (. Fypophosphate*ic rickets ). Show +<plaination + )% /he risk of fetal death with intrauterine par3o3irus infection *ay occur with infection in which tri*ester: 1. 2irst $. Second %. /hird '. 2irst: Second and /hird (. Eone of these answers are correct ). Show +<plaination 0 )' /he standard of care of patients with acute KawasakiJs disease is: 1. Prednisone $. Supporti3e care %. Aspirin and 1?15 '. Penicillin (. Aceta*inophen and 1?15 ). Show +<plaination C )( A neonate presents with a lar&e se&*ental he*an&io*a of the ?1 distri#ution. ,hat are features *ay #e associated with this 6ndin&. 1. /ra*"track calci6cations $. Anterior fossa defect %. Posterior fossa defect '. M"paraproteine*ia (. Muscular dystrophy ). Show +<plaination C )) A child presents with hi&h fe3er: straw#erry ton&ue: ly*phadenopathy: and a poly*orphous e<anthe*. Appro<i*ately how *any of these patients will ha3e coronary artery aneurys*s if left untreated. 1. /wo percent $. 2i3e percent %. /en percent '. /wenty"63e percent (. Si<ty percent ). Show +<plaination 0 )A ,hat syndro*e can accessory tra&i #e associated with. 1. 5oldenhar syndro*e $. /urner syndro*e %. Eeuro6#ro*atosis '. 1chthyosis (. Birt Fo&& 0u#e ). Show +<plaination A 1%' )C ,hich of the followin& disorders is *ore likely to occur in children with chronic fecal incontinence. 1. 8an&erhans cell histiocytosis $. Perianal pseudo3errucous papules and nodules %. Perianal streptococcal disease '. 5ranulo*a &luteale infantu* (. Se#orrheic der*atitis ). Show +<plaination B )D ,hat the *ost likely dia&nosis. 1. Atopic der*atitis $. 8a*ellar ichthyosis %. Hud syndro*e '. ,iskott"Aldrich syndro*e (. Keratosis pilaris ). Show +<plaination A A! Menkes kinky hair syndro*e is associated *ost co**only with: 1. /richorrhe<sis in3a&inata $. /richostasis spinulosa %. Pili *ulti&e*ini '. Pili torti (. Plica neuropathica ). Show +<plaination 0 A1 ,hat is the *ost likely dia&nosis. 1. /raction alopecia $. /inea capitis %. Aplasia cutis con&enita '. Ee3us se#aceous (. Alopecia areata ). Show +<plaination C A$ ,hich of the followin& should #e the ne<t step in the *ana&e*ent of this patient. 1. Bariu* swallow $. spine <"ray %. Cardiac e3aluation '. Ad*inistration of oral anti#iotics (. Pul*onary function studies ). Show +<plaination C A% A $ day"old full ter* neonate de3elops #lotchy erythe*atous *acules with s*all central pustules o3er the upper trunk and e<tre*ities. A &ra* stain re3eals predo*inantly eosinophils. ,hat is the *ost likely dia&nosis. 1. +rythe*a to<icu* neonatoru* $. 1ncontinentia pi&*enti %. Brticaria pi&*entosa '. /ransient neonatal pustular *elanosis (. Miliaria ). Show +<plaination A A' A youn& #oy presents with a port" wine stain: a Mon&olian spot: and a ne3us spilus. ,hich phako*atosis pi&*ento3ascularis does he ha3e. 1. /ype 1 $. /ype 11 %. /ype 111 '. /ype 1? (. /ype ? ). Show +<plaination 0 A( A new#orn has a nodule o3er his lu*#ar spine. Skin #iopsy re3eals a lipo*a. /he ne<t appropriate step is: 1. 9#ser3ation $. +<cision of the lesion %. 5enetic testin& '. 1*a&in& study (. Mali&nancy work up ). Show +<plaination 0 A) ,hich 6ndin& is associated with Pachyder*operiostosis: 1. Cutis 3erticis &yrata $. Pal*oplantar hyperkeratosis %. Mucosal keratoses '. 9steopathia striata (. Cutis *ar*orata ). Show +<plaination A 1%( AA Most co**on location of cutaneous lesions in neonatal lupus erythre*atosus is 1. Cheeks $. Eose %. Scalp '. Perioral (. Perior#ital ). Show +<plaination + AC ,hich of the followin& is *ost likely associated. 1. Eo additional a#nor*ality $. 0eafness %. Coarctation of the aorta '. Bi6d ri# (. Alopecia ). Show +<plaination A AD ,hat disorder is associated with a defect in 8MO1B. 1. Eail patella syndro*e $. Steatocysto*a %. Monilithri< '. 8her*itte"0uclos syndro*e (. Chediak"Fi&ashi syndro*e ). Show +<plaination A C! ,hich of the followin& is characteristic of ,ooly Fair Ee3us: 1. /ypically occurs in the (th decade $. 9cular a#nor*alities *ay #e associated %. /here are no ne3i associated with this hair a#nor*ality '. Sponteneous i*pro3e*ent ne3er occurs (. 1t is hereditary ). Show +<plaination + C1 ,hich of followin&s syndro*es characteri;ed #y Se#orrheic"like or e<foliati3e der*atitis 1. 8einerGs disease $. Fyperi**uno&lo#uline*ia + syndro*e %. ,iskott"Aldrich syndro*e '. Chronic &ranulo*atous disease (. O"linked a&a**a&lo#uline*ia ). Show +<plaination A C$ ,hich of the followin& is not a *a4or criterion for KawasakiJs disease: 1. 2e3er `( days $. Pal*oplantar erythe*a ` desLua*ation %. Cardiac aneurys* '. Straw#erry ton&ue red lips (. Cer3ical adenopathy ). Show +<plaination C C% /richothiodystrophy classically includes which of the followin& 6ndin&s: 1. /richostasis $. /richoschisis %. Pili torti '. /richorrhe<is in3a&inata (. /richorrhe<is nodosa ). Show +<plaination B C' /he *ost co**on a&e &roup for papular"purpuric &lo3es and socks syndro*e is: 1. Eew#orns $. /oddlers %. )"1! year olds '. Adolescents (. +lderly ). Show +<plaination 0 C( ,hich of the followin& is the *ost co**on co*plication associated with cutis *ar*orata telan&ectatica con&enita. 1. Sei;ure disorder $. Atrial septal defect %. Syste*ic lupus erythe*atosus + 1%) '. Fypercalce*ia (. 8i*# hypertrophy or atrophy ). Show +<plaination C) A ' *onth"old with diMuse #listers and erosions has a skin #iopsy dia&nostic of &enerali;ed *astocytosis. ,hich topical dressin& should #e a3oided in this patient. 1. Mupirocin oint*ent $. Poly*y<in B oint*ent %. Petrolatu* '. Eeo*ycin oint*ent (. Sil3er sulfadia;ine ). Show +<plaination B CA /he Carney co*ple< is associated with a defect in: 1. PHKAH1A $. 8SS/ %. P/+E '. MASF$ (. M8F1 ). Show +<plaination A CC /he association of Port"wine stains on a li*# with soft tissue swellin& with or without #ony o3er &rowth is: 1. Bannayan"Hiley"Hu3alca#a syndro*e $. 5oldenharGs syndro*e %. Stur&e",e#er syndro*e '. Klippel"/renaunay syndro*e (. Proteous syndro*e ). Show +<plaination 0 CD A $ year"old has a hi&h fe3er for three days: as the fe3er #reaks: a &enerali;ed rash appears on the trunk. /he *ost likely dia&nosis is: 1. Measles $. Mu*ps %. Hu#ella '. +rythe*a infectiosu* (. Hoseola ). Show +<plaination + D! An infant with failure to thri3e has *ultiple <antho*as on skin e<a* and foa*y histiocytes on #one *arrow #iopsy. Sour dia&nosis is: 1. 5aucherJs disease $. /ay"SachJs disease %. Eie*ann"Pick disease '. 2a#ryJs disease (. FunterJs syndro*e ). Show +<plaination C D1 ,hat is the function of the &ene which is defecti3e in ata<ia" telan&iectasia. 1. 5ap 4unction protein $. Cross"linkin& of structural proteins in the protein and lipid en3elope of the upper epider*is %. Pathway of cholesterol #iosynthesis '. 0EA repair protein (. /u*or supressor protein ). Show +<plaination 0 D$ A $' *onth"old infant presents with yellowish"#rown: crusted papules with petechiae in a se#orrheic distri#ution. A #iopsy is done to con6r* a dia&nosis. ,hich histolo&ic picture is *ost likely. 1. C01": S1!!" cells with renifor* nuclei $. 2oa*y histiocytes with /outon &iant cells %. C01P: S1!!P cells with renifor* nuclei '. Mi<ed cellular in6ltrate in a J#all and clawJ pattern (. Super6cial peri3ascular in6ltrate with *ild spon&iosis and neutrophil containin& scale crust ). Show +<plaination C D% ,hich of the followin& is the *ost co**on lon& ter* seLuelae fro* con&enital ru#ella syndro*e. 1. Macrocephaly $. Sa#er shins %. 0eafness C 1%A '. Eysta&*us (. Microcephaly ). Show +<plaination D' ,hich of the followin& is not true a#out 5ianotti"Crosti. 1. 1t is associated with 3iral infections like enterocirus: +B?: and CM? $. 1t typically aMects children #etween the a&e of % *onths and 1( years %. 1t is characteri;ed #y *ono*orphic sy**etric -at topped papules in acral areas '. 1t *ay ha3e associated fe3ers: ly*phadenopathy: and diarrhea (. Corticosteroids should #e &i3en to alle3iate the pruritus of the lesions ). Show +<plaination + D( 58B/"1 stains placental tissue. ,hat other tissue shows positi3e 58B/"1 stainin&. 1. capillary 3ascular *alfor*ation $. sal*on patch %. &lo*us tu*or '. infantile he*an&io*a (. an&elGs kiss ). Show +<plaination 0 D) ,hat is the *ost likely dia&nosis: 1. +pider*olysis #ullosa si*ple< $. +pider*olysis #ullosa dystrophica %. Pe*phi&us 3ul&aris '. 8inear #ullous 1&A disease (. 2acticial der*atosis ). Show +<plaination A DA An infant presents with yellowish" #rown: crusted papules with petechiae in a se#orrheic distri#ution. ,hich of the followin& state*ents a#out this entity is likely to #e true. 1. /his presentation is consistent with 7acLuetGs der*atitis $. C01P: S1!!P cells with co**a"shaped nuclei should #e seen on #iopsy %. /his presentation occurs when the infant is weaned oM of #reast *ilk '. An autoso*al recessi3e defect in holocar#o<ylase synthetase is the cause (. Maternal"fetal trans*ission *ost likely occured in the peripartu* period ). Show +<plaination B DC Su#cutaneous fat necrosis of the new#orn is usually treated with: 1. Aspirin $. /opical calcipitriol %. +<cision '. 9#ser3ation (. Hetinoids ). Show +<plaination 0 DD A $ week"old infant is #rou&ht to the +H with a rash on her face. She is found to ha3e a %rd de&ree heart #lock. ,hat is the risk that a second 1. (N $. 1!N %. $(N '. (!N C 1%C child #orn to this *other will ha3e the sa*e dia&nosis. (. 1!!N ). Show +<plaination 1! ! A % *onth old #a#y &irl is #rou&ht to your oTce for consultation. Sou note an infantile he*an&io*a on the tip of the nose. 1*a&in& re3eals no underlyin& structural ano*alies in the cere#ro"3asculature. Sou discuss startin& propranolol. ,hat side eMect do you need to *onitor for. 1. Fypertension $. Fypercalce*ia %. Fypo&lyce*ia '. /achycardia (. Fypernatre*ia ). Show +<plaination C 1! 1 1ndi3iduals with which of the followin& syndro*es characteristically present with photosensiti3ity: *ental retardation: a Iwi;enedI appearance: I#ird" headedI facies: and IMickey MouseI ears. 1. /ay Syndro*e $. Hoth*und"/ho*son Syndro*e %. Futchinson"5ilford Pro&eria Syndro*e '. Cockayne Syndro*e (. ,erner Syndro*e ). Show +<plaination 0 1! $ ,hich of the followin& is a potential co*plication of su#cutaneous fat necrosis of the new#orn. 1. Cholestrol clefts in fat cells $. Fypercalce*ia %. Acute renal failure '. Fepatitis (. +le3ated uric acid le3els ). Show +<plaination B 1! % /he &enetic disorder depicted in this photo is caused #y a *utation in: 1. MASF$ $. S/K11 %. P/+E '. Patched (. /SC1 ). Show +<plaination B 1! ' ,hich of the followin& is a ss0EA 3irus: 1. Ferpes3irus $. Par3o3irus %. Picorno3irus '. Adeno3irus (. Parapo< ). Show +<plaination B 1! ( ,hat is the dia&nosis. 1. /ransient neonatal pustular *elanosis $. Acropustulosis of infancy %. Eeonatal cphalic pustulosis '. Miliaria (. +rythe*a to<icu* neonatoru* ). Show +<plaination C 1! ) A full ter* new#orn de3elops erythe*atous: indurated plaLues on the upper #ack. ,hich of the followin& tests should #e perfor*ed. 1. Calciu* $. Co*plete #lood count %. Blood culture '. /hyroid function test (. Alkaline phosphatase ). Show +<plaination A 1! A /he diMerential dia&nosis of ;inc de6ciency is least likely to include: 1. 5ranulo*a &luteale infantu* $. Biotin de6ciency %. Multiple car#o<ylase de6ciency '. Cystic 6#rosis (. Folocar#o<ylase synthetase de6ciency ). Show +<plaination A 1! C ,hich of the followin& is a si&n of +AH8S postnatal con&enital syphilis. 1. CluttonGs 4oints $. Fi&ou*enakiGs si&n + 1%D %. FutchinsonGs teeth '. Sa#er shins (. ,i*#er&erGs si&n ). Show +<plaination 1! D /he *ost likely etiolo&y of 7acLuetJs diaper der*atitis is: 1. Candida $. /richophyton ru#ru* %. 5roup A #eta"he*olytic streptococcus '. Ferpes si*ple< 3irus: /ype $ (. Multifactorial ). Show +<plaination + 11 ! Most co**on *ali&nancy associated with *ultiple lesions si*ilar to the attached i*a&e is: 1. Acute *yelo&enous leuke*ia $. Chronic *yelo&enous leuke*ia %. Acute ly*phocytic leuke*ia '. Chronic ly*phocytic leuke*ia (. Melano*a ). Show +<plaination B 11 1 ,hich of the followin& diseases with i**unode6ciency has an increased risk of ly*phoreticular *ali&nancy. 1. ,iskott"Aldrich syndro*e $. Chronic &ranulo*atous disease %. 7o# syndro*e '. Se3ere co*#ined i**unode6ciency syndro*e (. 8einerJs disease ). Show +<plaination A 11 $ Ankylo#lepharon 6lifor*e adnatu* is seen with a defect in: 1. Plakophilin $. Plako&lo#in %. 0es*o&lein '. C"kit (. p)% ). Show +<plaination + 11 % An infant presents with red"purple: &ranulo*atous nodules occurrin& in the diaper area. /he etiolo&y is secondary to local irritation: *aceration and Candida al#icans. ,hat is the *ost likely dia&nosis. 1. 5ranulo*a &luteale infantu* $. 8an&erhans cell histiocytosisc. %. Se#orrheic der*atitis '. Biotin de6ciency (. Psoriasis ). Show +<plaination A 11 ' A new#orn presents with a well" de6ned: shiny patch with co*plete alopecia on the 3erte< of the scalp alon& the suture lines. ,hich of the followin& is the 6rst step in dia&nosis. 1. MH1 $. Calciu* %. Skin #iopsy '. Skull <"ray (. 2un&al culture ). Show +<plaination 0 11 ( Hhinorrhea: condylo*ata lata: and *ucous patches are all seen with which con&enital disorder. 1. Hu#ella $. /o<oplas*osis %. Ferpes si*ple< 3irus '. Syphilis (. Fu*an papillo*a3irus infection ). Show +<plaination 0 11 ) ,hich enanthe* is *ost co**only seen in association with +<anthe* su#itu*. 1. Koplik spots $. Hed straw#erry ton&ue %. Chapped lipsc dry: red *ucosa '. Hed *acules and streaks on the soft palate (. Palatal erosions 0 1'! ). Show +<plaination 11 A /he disorder caused #y a defect in intestinal ;inc"speci6c transporter S8C%DA' is *ost likely to present: 1. At #irth $. Bpon weanin& fro* #reast *ilk %. Bpon weanin& fro* for*ula '. 1n childhood (. 1n adulthood ). Show +<plaination B 1!. Cutaneous Manifestations of Syste*ic 0isease # Question Answers Corre ct Answ er 1 /he patient de*onstrated in 6&ure ) 1. 808 receptor de6ciency C 1'1 would ha3e: $. 0ecreased chylo*icrons %. +le3ated tri&lycerides '. 0ecreased 808 (. 0ecreased tri&lycerides ). $ ,hich of the followin& is true re&ardin& pie#aldis*. 1. 1t is caused #y a *utation in the 57B$ &ene $. 1t is caused #y defecti3e *eta#olis* of phytanic acid %. 1t is caused #y a defect in a protein su#unit of a kinase that acti3ates E2kappaB '. 1t is caused #y a de6ciency of fatty aldehyde dehydro&enase (. 1t is caused #y a *utation in the proto"onco&ene c"K1/ ). + % ,hat is the *ost co**on internal cause of intracta#le pruritus. 1. Fepatitis C $. Fypothyroidis* %. Fyperthyroidis* '. Chronic renal failure (. 1nternal *ali&nancy ). 0 ' Porphyria cutanea tarda *ay #e associated with all of the followin& e<cept: 1. Fepatitis C 3irus infection $. Alcohol %. +stro&ens '. Polyhalo&enated hydrocar#ons (. 1nherited de6ciency of uroporphyrino&en 111 synthase ). + ( ,hich of the followin& state*ents a#out *ultiple endocrine neoplasia =M+E> syndro*es is 2A8S+. 1. M+E 1 is associated with an&io6#ro*as and colla&eno*as $. M+E 11a is related to a defect in H+/ proto"onco&ene %. M+E 11a and 11# are #oth associated with *edullary thyroid carcino*a '. M+E 1: 11a and 11# are all inherited in an autoso*al do*inant fashion (. M+E 11a is associated with *ultiple *ucosal neuro*as ). + ) A (!"year old wo*an with a history of spontaneous pneu*othora< de3elops *ultiple 6r*: skin colored lesions on her face and neck o3er a period of se3eral years. /his patient should ha3e periodic sur3eillance for the de3elop*ent of: 1. Henal Cell Carcino*a $. 5astric Carcino*a %. Breast Carcino*a '. 93arian Carcino*a (. 8un& Carcino*a ). A A ,hich of the followin& is true re&ardin& cutaneous associations with hepatitis C 3irus =FC?> infection. 1. +rosi3e *ucosal lichen planus has a weaker association with FC? than does cutaneous lichen planus $. Pruritus in the settin& of chronic FC? infection is &enerally correlated with ele3ated #ile salt le3els in the settin& of li3er failure %. Polyarteritis nodosa is associated with FC? infection #ut not hepatitis B 3irus infection 0 1'$ '. FC?"related porphyria cutanea tarda *ay #e caused #y deco*part*entali;ation of iron stores and resultant o<idation of uroporphyrino&en decar#o<ylase (. Cutaneous reactions to FC? treat*ent are less co**on with interferonri#a3irin co*#ination treat*ent than with treat*ent with interferon alone ). C ,hich of the followin& state*ents re&ardin& porphyrias is /HB+. 1. +le3ated uroporphyrins are found in the red #lood cells of hepatoerythropoietic porphyria $. 0elta a*inole3ulenic acid is the only o<idi;ed porphyrin %. Acute inter*ittent porphyria is the *ost co**on for* of porphyria '. 5riseoful3in is safe for those with 3arie&ate porphyria (. Plas*a -uoresces at '1! n* in patients with 3arie&ate porphyria ). B D ,hich of the followin& la#oratory a#nor*alities is *ost co**on in patients with cholesterol e*#oli. 1. Eeutrophilia $. +le3ated a*ylase %. Fypercalce*ia '. +osinophilia (. Ane*ia ). 0 1! A patient presents with episodic -ushin& of the face and neck: a#do*inal pain: whee;in&: cou&h: and diarrhea. Carcinoid syndro*e is suspected. ,hich of the followin& tests would con6r* the dia&nosis. 1. Chan&e of urine to &reen color upon addition of nitrosonaphthol $. +le3ated urinary (" hydro<yindolacetic acid %. +le3ated $'"hour urine norepinephrine '. 0ecreased $'"hour urine 3anilyl*andelic acid (. +le3ated seru* tryptase ). B 11 A youn& African A*erican patient presents with ane*ia and spontaneously appearin& le& ulcers o3er #oth lateral and *edial *alleoli. /he *ost likely dia&nosis is: 1. Atherosclerotic disease $. Sickle cell ane*ia %. 2actitial der*atitis '. 8upus erythe*atosus (. /rau*a ). B 1$ ,hen *etastases to the skin occur fro* a thyroid *ali&nancy: they are usually due to: 1. Medullary carcino*a $. Papillary adenocarcino*a %. 2ollicular carcino*a '. Anaplastic carcino*a (. Cutaneous *etastases ha3e not #een reported in association with thyroid *ali&nancies ). B 1% /he or&anis* that causes this infection shown in the 6&ure is: 1. /. tonsuran $. C. i**itis %. C. al#icans '. P. aeru&inoas (. /. 3errucosu* C 1'% ). 1' ,hich of the followin& is true a#out #lue ru##er #le# ne3us syndro*e. 1. typically appears in adulthood $. the 3enous *alfor*ations re&ress with ti*e %. characteri;ed #y co*pressi#le #lue nodules !.1 to ( c* in si;e. '. the nodules are pruritic (. the &astrointestinal lesions are typically located in the sto*ach ). C 1( Fypothyroidis* *ay result in all of the followin& cutaneous 6ndin&s e<cept: 1. 1ncrease in the percenta&e of telo&en hairs $. Sellowish hue %. Purpura '. Madarosis (. All of these answers are correct ). + 1) +ach of the followin& de*onstrates a 3asculitis e<cept: 1. 5ranulo*a faciale $. Fenoch"Schoenlein purpura %. /ype 1 cryo&lo#uline*ia '. ,e&enerJs &ranulo*atosis (. 8eukocytoclastic 3asculitis ). C 1A ,hich of the followin& state*ents a#out necrolytic *i&ratory erythe*a is /HB+. 1. /here is a low incidence of *etastasis of the oMendin& tu*or at the ti*e of dia&nosis $. Acanthosis and parakeratosis are found on routine histolo&y %. Seru* &luca&on le3els are usually nor*al '. ?acuolar chan&es are nor*ally found on routine histolo&y (. /he oMendin& tu*or ori&inates fro* endocrine ar&entaTn cells ). B 1C ,hich of the followin& syndro*es necessitates a work"up for colon cancer. 1. /urcot $. Birt"Fo&&"0u#e %. 8AMB '. Muckle ,ells (. ,ells ). A 1D A $!"year"old *ale de3elops an eruption of 1!!Gs of red"#rown yellowish papules with in3ol3e*ent of the *ucous *e*#ranes. Fe has no ly*phadenopathy. /he *ost likely dia&nosis is: 1. Mont&o*eryGs syndro*e $. Beni&n cephalic histiocytosis %. Hosai"0orf*an disease '. Eecro#iotic <antho&ranulo*a (. Multicentric reticulohistiocytosis ). A $! All of the followin& are true re&ardin& Fenoch"Schonlein Purpura +OC+P/: 1. 1t is an 1&5 *ediated s*all 3essel 3asculitis $. 0irect i**uno-uorescence of lesional and peri"lesional skin will de*onstrate C% and 6#rin deposits in s*all 3essel wall %. 1t is often preceded #y an upper respiratory infection '. 1t *ay #e co*plicated #y intussusception (. 1t is self"resol3in& A 1'' ). $1 ,hich of the followin& is E9/ nor*ally associated with der*ato*yositis. 1. 93arian cancer in wo*en $. Psoriasifor* der*atitis of the scalp %. +le3ated aldolase le3els '. Cuticular dystrophy (. /esticular cancer in *en ). + $$ 9ne *i&ht see all of the followin& la#oratory and clinical a#nor*alities in cryo&lo#uline*ia associated with hepatitis C 3irus infection +OC+P/: 1. +le3ated li3er function tests $. Positi3e rheu*atoid factor %. +le3ated C% le3els '. Acrocyanosis (. Brticarial plaLues ). C $% He&ardin& carcinoid syndro*e: which of the followin& is /HB+. 1. Sy*pto*s are caused #y *etastases to the skin $. /he *ost co**on location for the tu*or is the rectu* %. So*atostatin is a preferred treat*ent '. ?MA is ele3ated in the urine (. /he tu*ors should not #e re*o3ed ). C $' A patient de3elops cold"e<acer#ated dusky acral plaLues consistent with pernio. /he syste*ic condition *ost likely to *anifest such lesions is: 1. 8upus +rythe*atosus $. 0er*ato*yositis %. AcLuired 1**unode6ciency Syndro*e '. Fepatitis C (. 0ia#etes Mellitus ). A $( All of the followin& disorders ha3e an increased risk of syste*ic *ali&nancy e<cept 1. Hoth*und /ho*pson syndro*e $. Cockayne syndro*e %. Bloo* syndro*e '. ,erner syndro*e (. Oeroder*a pi&*entosu* ). B $) An unco**on co*plication of treat*ent with potassiu* iodide is: 1. Acute &enerali;ed e<anthe*atous pustulosis $. +rythe*a nodosu* %. ,olM"ChiakoM eMect '. +<acer#ation of lichen planus (. Fyperhidrosis ). C $A All of the followin& are true re&ardin& the condition pictured +OC+P/: 1. Perilesional direct i**uno-uroescence shows &ranular 1&A in the der*al papillae and at the der*oepider*al 4unction $. 9nly $!N of patients ha3e a &luten"sensiti3e enteropathy %. 1t is associated with F8A"0Q$: F8A"0H%: and F8A"BC '. 1t is associated with Fashi*otoGs thyroiditis (. Cutaneous 6ndin&s are due to autoanti#odies to epider*al trans&luta*inase ). B 1'( $C ,hat is the *ost co**on pri*ary site of a carcinoid tu*or. 1. 8i3er $. Sto*ach %. Appendi< '. 0uodenu* (. 1leu* ). C $D All of the followin& state*ents are true re&ardin& this condition +OC+P/: 1. 0ia#etes or &lucose intolerance is found in $!N of these patients $. /his condition *ay #e associated with cutaneous anesthesia: hypohidrosis: and partial alopecia %. /here is no i*pact of ti&ht &lucose control on the likelihood of de3elopin& this condition '. !.%"%N of dia#etics ha3e this skin condition (. /his condition is associated with increased der*al *ucin ). + %! A patient with &astric cancer de3elops acanthosis ni&ricans and a sudden eruption of nu*erous warty stuck"on papules on the trunk. ,hat other 6ndin& *ay #e seen. 1. Eu*erous wart"like lesions on the dorsal hands and wrists $. 2ollicular spicules on the nose %. Carpal tunnel syndro*e '. Perior#ital pupura (. Mi&ratory thro*#ophle#itis ). A %1 A patient presents with a co*plaint of facial -ushin& that spreads to the neck and upper trunk. He3iew of syste*s re3eals that the patient has occassional #outs of diarrhea: and inter*ittent #ronchospas*. 8a#oratory testin& re3eals an ele3ated urine ("hydro<yindole"acetic acid le3el. ,hich of the state*ents re&ardin& this condition is E9/ correct. 1. Chest and a#do*inalpel3ic C/ scannin& should #e the ne<t step in this patientGs e3aluation $. /he patient *ay ha3e an associated scleroder*oid"like eruption on e<a*ination %. /he patient likely has a neoplas* ori&inatin& in the endocrine ar&entaTn cells '. /reat*ent with cyproheptadine would #e contraindicated (. /he patient likely has a neoplas* located in the 51 tract ). 0 %$ A patient presents with planepal*ar <antho*as. /he *ost likely &enetic disorder would #e: 1. fa*ilial lipoprotein lipase de6ciency $. 2a*ilial hypertri&lyceride*ia %. 2a*ilial hypercholesterole*ia '. 2a*ilial dys#etalipoproteine*ia (. Cere#rotendinous <anto*atosis ). 0 %% Mutations in the S/K11 &ene encodin& a serine threonine kinase are seen in: 1. Muir"/orre Syndro*e $. Bannayan"Hiley"Hu3alca#a Syndro*e %. Birt"Fo&&"0u##e Syndro*e '. Cronkhite"Canada Syndro*e (. Peut;"7e&hers Syndro*e ). + %' A patient presents with *ild *ental 1. /his syndro*e is inherited in + 1') retardation: infertility: 4oint contractures: short stature: ichthyosis: and sparse hair with trichoschisis. All of the followin& are true re&ardin& this patientGs condition +OC+P/: an autoso*al recessi3e *anner $. 1f photosensiti3ity is a feature: &onad si;e *ay #e nor*al %. Patients *ay ha3e associated cataracts '. /he syndro*e is caused #y i*paired nucleotide e<cision repair (. Perifo3eal &listenin& white dots are a feature ). %( ,hich of the followin& is E9/ associated with Cronkhite"Canada syndro*e. 1. 8enti&ines $. Adeno*atous &astrointestinal polyps %. 2i#rocystic #reast disease '. 9nycholysis (. ,ei&ht loss ). C %) ,hich of the followin& cutaneous 6ndin&s are characteristic for ?ohwinkles Syndro*e. 1. Foneyco*#ed diMuse pal*oplantar keratoder*a: pseudoainhu* with autoa*putation: star"shaped keratosis o3er knuckles: nail dystrophy: and alopecia $. 0ense depi&*ented lusterless hair: pili torti: dou&hy skin: diMuse cutaneous hypopi&*entation %. /ransient erythroder*a at #irth: pal*oplantar keratoder*a: follicular hyperkeratosis: scarrin& alopecia: dystrophic nails '. Hapidly pro&ressi3e alopecia of all hair"#earin& areas: onycholysis: onychoschi;ia: onycho*adesis: hyperpi&*ented *acules on e<tre*ities (. 9ral papillo*atosis: pal*oplantar keratoses: acral keratoses: lipo*as: he*an&io*as: scrotal ton&ue ). A %A A $ year old child is e3aluated for suspected dia&nosis of neuro6#ro*atosis. ,hich of the followin& dia&nostic 6ndin&s is typically a#sent on e<a* in this a&e &roup. 1. neuro6#ro*as $. *acrocephaly %. pi&*ented iris ha*arto*as '. sei;ures (. cafe au lait *acules ). A %C Fyperkeratotic follicular nasal papules ha3e #een descri#ed as a paraneoplastic si&n in the settin& of which neoplas*. 1. Multiple *yelo*a $. Castle*anGs tu*or %. AM8 '. Adenocarcino*a of the lun& (. Henal cell carcino*a ). A %D /his syndro*e is cause #y defects in the &enes that code for tu*or suppressor proteins ha*artin and tu#erin. 1. Bannayan"Hiley"Hu#alca#a Syndro*e $. Blue Hu##er Ble# Ee3us Syndro*e %. Cronkhite"Canada Syndro*e '. Bourne3illeGs 0isease (. CowdenGs Syndro*e ). 0 1'A '! ,hich of the followin& is an autoso*al recessi3e disorder characteri;ed #y pili torti and deafness. 1. ,aarden#ur& Syndro*e $. S4or&en"8arsson Syndro*e %. Hefsu* 0isease '. B4ornstadGs Syndro*e (. Cockayne Syndro*e ). 0 '1 ,hich type of porphyria is the autoso*al recessi3e for* of porphyria cutanea tarda. 1. +rythropoietic protoporphyria =+PP> $. Con&enital erythropoietic porphyria =C+P> %. Fepatoerythropoietic porphyria =F+P> '. ?arie&ate Porphyria =?P> (. Acute 1nter*itent Porphyria =A1P> ). C '$ All of the followin& are true re&ardin& Cockayne Syndro*e +OC+P/: 1. 1nheritance is autoso*al recessi3e $. 1t is caused #y an ina#ility to repair cyclo#utane di*ers %. 1t is associated with #asal &an&lia calci6cations '. 1t is associated with retinal pi&*ent de&eneration with a Isalt and pepperI appearance (. Eone of these answers are incorrect ). + '% ,hich of the followin& is *ore co**only associated with ulcerati3e colitis as co*pared to CrohnJs disease. 1. Pyoder*a &an&renosu* $. 9ral lesions %. Polyarteritis nodosa '. 2istulae (. Metastatic lesions ). A '' A ) *onth"old has a 3errucous plaLue on the *ucosal surface of the lower lip. Skin #iopsy is consistent with Hi&a"2ede disease. Sou should refer the patient to: 1. An ophthal*olo&ist $. A &astroenterolo&ist %. A neurolo&ist '. A he*atolo&ist (. An otolarn&olo&ist ). C '( Carotene*ia can #e a *anifestation of: 1. Porphyria $. Fypothyroidis* %. Preti#ial *y<ede*a '. 5ra3es disease (. A*yloidosis ). B ') Patients with ple<ifor* neuro*a and E2 1 who also ha3e 7O5 are at increased risk for de3elopin&: 1. 7u3enile chronic *yelo&enous leuke*ia $. Eon"Fod&kinGs 8y*pho*a %. +sopha&eal cancer '. Breast cancer (. Medullary carcino*a of the thyroid &land ). A 'A /he *ost co**on autoi**une disease associated with hepatitis C is: 1. Autoi**une thyroiditis $. Myasthenia &ra3is %. Aplastic ane*ia '. 8y*phocytic sialadenitis (. Hheu*atoid arthritis ). A 1'C 'C Patients with ,ernerJs syndro*e typically e<perience which of the followin& types of cardiac disease. 1. Fypertrophic cardio*yopathy $. Aortic aneurys*s %. Pre*ature atherosclerosis '. Cardio*e&aly (. Mitral 3al3e prolapse ). C 'D He&ardin& paraneoplastic pe*phi&us: which of the followin& is /HB+. 1. 0es*oplakin: one of the *olecular anti&ens: has a *olecular wei&ht of 1D!kd $. Metastatic sLua*ous cell carcino*a of the skin is a co**on cause %. 5ranular C% deposition is found at the der*oepider*al 4unction on direct i**uno-uorescence '. Monkey esopha&us is the preferred su#strate for indirect i**uno-uorescence (. Eon"Fod&kins ly*pho*a is rarely associated ). C (! He&ardin& erupti3e <antho*ata: which of the followin& is /HB+. 1. /hey are associated with type 11 and 111 hyperlipide*ias $. /hey are associated with calciu* channel #lockers %. /here is no association with ethanol consu*ption '. /hey are associated with type 1: 1?: and ? hyperlipide*ias (. /hey fa3or the -e<or surfaces of the e<tre*ities ). 0 (1 All of the followin& are seen *ore co**only in CrohnGs disease than in ulcerati3e colitis +OC+P/: 1. 9ral co##lestonin& $. Polyarteritis nodosa %. Pyosto*atitis 3e&etans '. Perineal 6stulas (. Perineal 6ssures ). C ($ 1n patients with *i<ed cryo&lo#uline*a associated with hepatitis C: the *ost likely la#oratory a#nor*ality is: 1. +le3ated rheu*atoid factor $. P AEA %. P p"AECA '. 0ecreased cryo&lo#ulins (. +le3ated he*atocrit ). A (% ,hat is the *ost co**on *ali&nancy associated with this condition at this location. 1. Breast cancer $. 5astric cancer %. /hyroid cancer '. Pancreatic cancer (. Melano*a ). B (' All of the followin& are true re&ardin& the condition pictured +OC+P/: 1. Men with this condition *ay #e at increased risk for ly*pho*a $. Associated calcinosis cutis *ay #e seen in pediatric patients %. A&e"appropriate cancer screenin& is reco**ended '. May #e associated with a psoriasifor* scalp der*atitis (. +le3ated aldolase is *ore + 1'D speci6c than CPK ). (( A )'"year old *an de3elops yellowish perior#ital plaLues that occasionally ulcerate and heal with scarrin&. ,hat is the *ost likely associated la# 6ndin&. 1. Monoclonal &a**opathy $. Positi3e antinuclear anti#odies %. +le3ated creatinine '. +le3ated tri&lycerides (. 1ncreased thyroid sti*ulatin& hor*one ). A () ,hich of the followin& is true re&ardin& treat*ent of in-a**atory der*atoses with potassiu* iodide. 1. /he ,olM"ChaikoM eMect *ust #e considered $. Bindin& of e<cess or&anic iodide in the thryoid &land *ay occur %. /hyroid hor*one synthesis *ay #e inhi#ited '. Eone of the answers are correct (. All of these answers are correct ). + (A ,hich of the followin& is characteristic of dia#etic skin. 1. Appro<i*ately $!N of dia#etics ha3e necro#iosis lipoidica dia#eticoru* =E80> $. /he le3el of clea3a&e in #ullous dia#eticoru* is su#corneal %. Candida tropicalis is the *ost co**on cause of an&ular cheilitis '. /here is a well"esta#lished association #etween deep &ranulo*a annulare =5A> and dia#etes (. Sellow skin *ay occur in up to 1!N of dia#etics ). + (C ,hich of the followin& diseases is caused #y an en;y*atic defect that occurs in the *itochondria. 1. Porphyria Cutanea /arda $. Acute 1nter*ittent Porphyria %. Con&enital +rythropoeitic Porphyria '. +rythropoeitic Protoporphyria (. Fepatoerythropoetic Porphyria ). 0 (D SLua*ous cell carcino*a is seen in which syndro*e. 1. 5orlin syndro*e $. Ho*#o syndro*e %. Eicolau"Balus syndro*e '. Has*ussen syndro*e (. Ba;e< syndro*e =Acrokeratosis paraneoplastica> ). + )! /ripe pal*s are a cutaneous *anifestation associated with which of the followin& *ali&nancies. 1. Henal carcino*a $. 8un& carcino*a %. Prostate carcino*a '. Colon carcino*a (. Pancreatic carcino*a ). B )1 2eatures of Cushin& disease include all of the followin& +OC+P/. 1. 2acial plethora $. Striae %. Fypertension '. Suppression of corticotropin occurs with ad*inistration of 0 1(! de<a*ethasone (. Firsutis* ). )$ A A!"year old *ale de3elops hyperpi&*ented 3el3ety plaLues on his lips: dorsal hands and feet: and in his a<illa. ,hat underlyin& *ali&nancy is *ost co**only associated with this 6ndin&. 1. 5astric Carcino*a $. 8un& Carcino*a %. 93arian Carcino*a '. 8y*pho*a (. Breast Carcino*a ). A )% ,hich of the followin& is a paraneoplastic disease *ost often associated with lun& carcino*a. 1. Fypertrichosis lanu&inosa acLuisita $. 0er*ato*yositis %. Acanthosis ni&ricans '. Paraneoplastic pe*phi&us (. +rythroder*a ). A )' A )%"year"old *ale de3elops s*all: non"tender: 3iolaceous papules on his dorsal hands: face: ears: and trunk. /he patient also de3elops a destructi3e arthropathy with 6n&er defor*ities. ,hat percenta&e of patients with this condition has an associated *ali&nancy. 1. $!"$(N $. ("1!N %. )("A!N '. (!"((N (. C("D!N ). A )( Fepatitis C infection is associated with: 1. 5ianotti"Crosti syndro*e $. Mi<ed cryo&lo#uline*ia %. Pityriasis rosea '. KapsiJs sarco*a (. 9ral hairy leukoplakia ). B )) A patient presents with diMuse wa<y keratoder*a of the pal*s and soles as well as oral hairy leukoplakia. ,hich of the followin& state*ents re&ardin& this condition is E9/ true. 1. /his condition *ay #e associated with esopha&eal carcino*a $. /his condition is autoso*al recessi3e %. 2a*ily *e*#ers should #e ad3ised to under&o cancer screenin& '. 2eatures *ay include sLua*ous cell carcino*as arisin& fro* keratoder*ic skin (. Eone of these answers are correct =all are true> ). B )A ,hich of the followin& *ay #e associated with 5ra3esJ disease. 1. 0er*atitis herpetifor*is $. 5eo&raphic ton&ue %. Fypohidrosis '. Madarosis (. /hick: pale lips ). A )C /his autoso*al do*inant condition is characteri;ed #y trichodisco*as: 6#roepithelial polyps: and 6#rofolliculo*as: 1. P9+MS syndro*e $. Ho*#o syndro*e %. Birt"Fo&&"0u#e syndro*e '. Proteus syndro*e (. CowdenGs disease ). C )D ,hich of the followin& skin 6ndin&s is *ost closely linked to hepatocellular carcino*a as a paraneoplastic syndro*e. 1. Pityriasis lichenoides $. Pityriasis al#a %. Pityriasis a*iantacea '. Pityriasis rotunda 0 1(1 (. Pityriasis rosea ). A! A patient with &luten"sensiti3e enteropathy presents with 3esicles on the e<tensor surfaces of the e<tre*ities. ,hat 6ndin&s are *ost likely on a perilesional #iopsy. 1. 5ranular 1&A at the der*oepider*al 4unction on direct i**uno-uorescence $. 8inear C% and 1&5 at the der*oepider*al 4unction on direct i**uno-uorescence %. 8inear 1&A surroundin& 3essels on direct i**uno-uorescence '. PautrierJs *icro"a#scesses in the epider*is on F @ + (. 8eukocytoclastic 3asculitis ). A A1 An end sta&e A10S patient with tu#erculosis presents with diMuse hyperpi&*entation of #oth sun" e<posed and une<posed areas. /he pal*er creases are *arkedly hyperpi&*ented and the patient is 3ery ill. /he *ost likely dia&nosis is: 1. Ar&yria $. 8y*pho*a %. /inea 3ersicolor '. KaposiGs sarco*a (. Addison disease ). + A$ /erryGs nails can *ost likely #e seen in which of the followin& conditions. 1. Cirrhosis $. CrohnGs disease %. +nd"sta&e renal disease '. Fidradenitis suppurati3a (. Fyperthyroidis* ). A A% /he peak sensiti3ity to prophyrins occurs at which wa3elen&ths. 1. $$!"$D!n* $. $D!"%$!n* %. %$!"'!!n* '. '!!"'1!n* (. '1!"'(!n* ). 0 A' Patients with this syndro*e are at increased risk for de3elopin& 8her*ite"0uclos disease: 1. Bourne3illeGs 0isease $. Eail"Patella Syndro*e %. M+E /ype 11A '. 2a#ryGs 0isease (. CowdenGs Syndro*e ). + A( ,hich of the followin& state*ents re&ardin& *ultiple endocrine neoplasia syndro*es is true. 1. M+E /ype 11B is also known as SippleGs Syndro*e $. M+E /ype 11A is also known as ,er*erGs Syndro*e %. Multiple *ucosal neuro*as are seen in association with M+E /ype 11A '. 8ichen or *acular a*yloidosis is seen in association with M+E /ype 11A (. Patients with M+E /ype 11B are at increased risk for de3elopin& follicular thyroid carcino*a ). 0 A) ,hich of the followin& is E9/ true re&ardin& Cushin&Gs Syndro*e. 1. Brine cortisol le3els are ele3ated $. Corticotropin is suppressed with the ad*inistration of de<a*ethasone %. 1t *ay #e associated with an underlyin& oat cell lun& carcino*a B 1($ '. 1t *ay #e associated with hypertension and hypokale*ia (. May present with hyperpi&*entation and facial plethora ). AA ,hich of the followin& is not a feature of Cronkhite"Canada syndro*e. 1. 0iarrhea $. Alopecia %. 8un& carcino*a '. 0ystrophic nails (. Fyperpi&*ented *acules ). C AC ,hich of the followin& is E9/ true re&ardin& calciphyla<is. 1. Patients with pro<i*ally" located lesions ha3e a #etter pro&nosis than those with acral lesions $. May #e treated with parathyroidecto*y %. May #e present with retifor* purpura '. Fistolo&ic 6ndin&s include *edial calci6cation and inti*al hyperplasia of s*all arteries and arterioles (. Fas an associated *ortality of )!"C!N ). A AD ,ith re&ards to patients with dia#etes *ellitus and skin: which of the followin& is *ost accurate. 1. 0ia#etic der*opathy is *ost co**only e<pressed on the forear*s and feet $. Sclerede*a *ost often presents on the ti#ial surfaces %. Sclerede*a is the *ost co**on skin *anifestation of dia#etes *ellitus '. 8ess than (N of patients with dia#etes *ellitus ha3e necro#iosis lipoidica (. 8ess than (N of patients with necro#iosis lipoidica ha3e dia#etes *ellitus ). 0 C! ,hich of the followin& is 2A8S+ re&ardin& 2a#ryJs disease. 1. 1t is inherited in an O"linked recessi3e fashion $. 1t is associated with acral parasthesias %. 1t is inherited in an O"linked do*inant fashion '. 1t *ay #e associated with renal failure (. /he etiolo&y is a defect in alpha"&alactosidase A ). C C1 ,hich one of the followin& clinical 6ndin&s su&&ests the dia&nosis of *ultiple *yelo*a. 1. Sudden eruption of se#orrheic keratoses $. Fyperkeratotic follicular papules on the nose %. +rythe*a: 3esicles: and erosions in periori6cal and acral areas B 1(% '. Concentric erythe*atous rin&s with trailin& scale on trunk and e<tre*ities (. /ripe pal*s ). C$ A co*plication seen in wo*en of child#earin& a&e with the condition in the 6&ure is: 1. Atrial septal defect $. Pul*onary ly*phan&ioleio*yo*a %. 5astrointestinal #leedin& '. 8isch nodules (. Pul*onary e*#oli ). B C% ,hich of the followin& is =are> characteristic of #asal cell ne3us syndro*e. 1. Autoso*al recessi3e inheritance $. E+M9 &ene *utation %. /ra*"track calci6cations '. Colo#o*as (. /elan&iectasias ). 0 C' ,hat is the uniLue la#oratory 6ndin& in stool in porphyria cutanea tarda =PC/> and hepatoerythropoietic porphyria =F+P>. 1. ele3ated coproporphyrins $. ele3ated isocoproporphyrins %. decreased isocoproporphyrins '. ele3ated protoporphyrins (. ele3ated uroporphyrins ). B C( A A!"year old fe*ale de3elops erythe*a with 6ne adherent scale on acral skin that pro&resses to keratoder*a and e3entually a *ore &enerali;ed psoriasifor* der*atitis. ,hat is the *ost likely underlyin& *ali&nancy. 1. SLua*ous cell carcino*a of the laryn< $. Adenocarcino*a of the colon %. Adenocarcino*a of the #reast '. SLua*ous cell carcino*a of the 3a&ina (. Medullary thyroid carcino*a ). A C) ,hich of the followin& is /HB+ a#out dia#etic"related skin disease. 1. Bullae are co**on on the thi&hs $. Sellow skin aMects the *a4ority of dia#etic patients %. 0ia#etic der*opathy aMects the upper #ack '. Eecro#iosis lipoidica aMects appro<i*ately $!N of dia#etics (. 0irect i**uno-uorenscence is usually ne&ati3e in #ullous dia#eticoru* ). + CA /he shoulder pad si&n has #een descri#ed in which disease. 1. 0er*ato*yositis $. Cushin&s disease %. Syste*ic a*yloidosis '. Eephro&enic 6#osin& der*opathy (. Syste*ic lupus erythe*atosis ). C CC He&ardin& erupti3e <antho*as: which of the followin& is true. 1. /hey occur in the settin& of fa*ilial hyperlipide*ia types 1: 1?: and ? $. /hey occur in the settin& of fa*ilial hyperlipide*ia types 11 and 111 %. /ri&lyceride le3els are usually #elow (!!*&dl A 1(' '. /hey are *ost co**only found on the eyelids (. /hey are not related to alcohol consu*ption ). CD /he presence of anti#odies to c"AECA is characteristically seen in patients with which disease. 1. Bl3erati3e colitis $. Chur&"Strauss disease %. Polyarteritis nodosa '. ,e&enerJs &ranulornatosis (. Eodular 3asculitis ). 0 D! All of the followin& are true re&ardin& incontinentia pi&*enti +OC+P/: 1. 1t is caused #y a *utation in the E+M9 &ene $. 1t is an O"linked recessi3e disorder %. 1n-a**ation and #listerin& *ay #e followed #y hyperkeratotic: 3errucous lesions '. 1t is associated with cere#ellar ata<ia (. 1t is associated with colo#o*a and retinal detach*ent ). B D1 Paraneoplastic pe*phi&us is associated with all of the followin& underlyin& *ali&nancies +OC+P/: 1. Eon"Fod&kins ly*pho*a $. 8un& carcino*a %. Chronic ly*phocytic leuke*ia '. /hy*o*a (. Castle*anGs tu*or ). B D$ A patient infected with the hepatitis C 3irus is at hi&hest risk for which of the followin& cutaneous *anifestations. 1. 1ncreased hyaluronic acid in der*is of lower and upper e<tre*ities $. 8indsayJs nails %. Con&enital hyperpi&*entation of the retinal pi&*ent =CFHP+> '. Periori6cial lenti&ines (. Positi3e rheu*atoid factor and decreased co*ple*ent =C%> ). + D% A patient on he*odialysis presents with indurated plaLues ha3in& a peau dJoran&e te<ture on the #ilateral lower le&s. ,hich of the followin& state*ents is /HB+. 1. /he dia&nostic histopatholo&ical 6ndin&s include acanthosis and hyperkeratosis $. Seru* protein electrophoresis should #e perfor*ed %. 0apsone will likely #e eMecti3e treat*ent '. /he face is usually is aMected (. /he pal*s and soles are usually aMected ). B D' /he *ost co**on location of the lesions in nephro&enic 6#rosin& der*opathy is: 1. 2ace $. Pal*s and soles %. 8ower e<tre*ities '. Back (. Chest ). C D( A (( year old patient presents with new onset #rown *acules on ar*s: le&s: face and pal*s. She &i3es a % *onth history of diarrhea: a#do*inal 1. Peut;"7e&hers syndro*e $. Cowden disease %. Blcerati3e colitis '. Cronkhite"Canada syndro*e 0 1(( cra*ps: wei&ht loss and protein" losin& enteropathy. /he *ost likely dia&nosis is: (. Plu**er"?inson syndro*e ). D) All of the followin& are true re&ardin& calciphyla<is e<cept: 1. Seen in end"sta&e renal disease $. ?ascular *ural calci6cation occurs late in the process %. Fi&h *orality rate is due to sepsis '. Association with hyperparathyroidis* (. Association with an ele3ated calciu*: phosphate product ). B DA All of the followin& state*ents re&ardin& cryo&lo#uline*ia are true +OC+P/: 1. /ype 1 is co*posed of *onoclonal 1&5 and polyclonal 1&M $. /ype 11 is co*posed of polyclonal 1&5 and *onoclonal 1&M %. /ype 111 is co*posed of polyclonal 1&5 and polyclonal 1&M '. C!N of cases of *i<ed cryo&lo#uline*ia are associated with Fepatitis C infection (. Eone of these answers are correct =all state*ents are true> ). A DC ,hich of the followin& porphyria cutanea tarda associations has a direct relationship to the le3el of urine uroporphyrins. 1. 0ystrophic calci6cations $. +stro&en le3els %. Scleroder*oid chan&es '. HBC -uorescence (. Fypertrichosis ). C DD ,hich of the followin& is E9/ associated with Fepatitis C disease. 1. Mi<ed cryo&lo#uline*ia $. Broporphyrino&en decar#o<ylase de6ciency %. Sin&le"stranded HEA 3iridae '. 8eukocytoclastic 3asculitis (. Sin&le"stranded 0EA 3iridae ). + 1! ! ,hat is the treat*ent of choice for porphyria cutanea tarda. 1. Phle#oto*y $. Anti*alarials %. +rythropoietin '. 9ral iron supple*entation (. Eapro<en ). A 1! 1 A patient with con&enital hypertrophy of retinal epitheliu* is *ost likely to ha3e: 1. An autoso*al do*inant *utation in the MSF$ &ene $. Pheochro*ocyto*a %. Adeno*atous polyposis '. /ra*"track calci6cations on head radio&raph (. Pe&"shaped teeth ). C 1! $ /he e<pected histolo&y of a #iopsy take fro* the lesion shown in the i*a&e would 1. 1ncreased *ucin $. Schu*ann #odies %. 5ranulo*atous in6ltrate locali;ed to the papillary der*is '. +lastic 6#er de&eneration (. Colla&en de&eneration ). + 1() 1! % +rythe*a &yratu* repens is known to #e associated with all of the followin& *ali&nancies e<cept: 1. 8un& carcino*a $. Breast carcino*a %. Cer3ical carcino*a '. Bladder carcino*a (. 5astric carcino*a ). + 1! ' ,hich of the followin& is true re&ardin& nephro&enic 6#rosin& der*opathy. 1. Fas a rapidly pro&ressi3e #ut re3ersi#le course $. 1s associated with a paraproteine*ia %. 1s associated with peripheral eosinophilia '. May #e associated with antiphospholipid anti#odies (. 1s associated with a dra*atic increase in der*al *ucin ). 0 1! ( ,hich of the followin& state*ents re&ardin& porphyrias is 2A8S+. 1. 2ecal coproporphyrino&en is increased in 3arie&ate porphyria $. Fepatoerythropoietic porphyria is the ho*o;y&ous for* of porphyria cutanea tarda %. Acute inter*ittent porphyria has no skin 6ndin&s '. Coproporphyrino&en is ele3ated *ore than uroporphyrino&en in $' hour urine sa*ples in porphyria cutanea tarda (. 1n erythropoietic protoporphyria: protoporphyrin 1O a#sor#s in the Soret #and ). 0 1! ) /he clinical 6ndin&s in 6&ure % are associated with which disorder. 1. 5ra3es disease $. Myasthenia &ra3is %. 0er*ato*yositis '. Sarcoidosis (. Hheu*atoid Arthritis ). A 1! A ,hich step is rate"li*itin& in the synthesis of protoporphyrino&en. 1. A*inole3ulinic acid synthase $. A*inole3ulinic acid dehydratase %. Porpho#illino&en dea*inase '. Coproporphyrino&en o<idase (. 2errochelatase ). A 1(A 11 . 0isorders of the Fair and Eails # Question Answers Corre ct Answ er 1 Koilonychia is *ost co**only seen in association with: 1. Henal disease $. Pro&ressi3e syste*ic sclerosis %. Alopecia areata '. 1*paired iron *eta#olis* (. Pul*onary disease ). Show +<plaination 0 $ /he syndro*e includin& *ental and physical retardation: con3ulsions: episodic unconsciousness: li3er enlar&e*ent: skin lesions: and dry and #rittle hair showin& trichorrhe<is nodosa *icroscopically and -uorescin& red. is: 1. Ba;e< 2ollicular Atrophoder*a $. CrandallGs syndro*e %. Citrulline*ia '. Ar&ininosuccinic aciduria (. B4ornstadGs syndro*e ). Show +<plaination 0 % B4Jrnstad syndro*e is the co*#ination of pili torti and which of the followin& features. 1. 8enti&ines $. ,hite forelock %. Fypo&onadis* '. 0eafness (. Sei;ures ). Show +<plaination 0 ' A ' year"old #oy has a sin&le circular alopecic atrophic patch on the 3erte< of the scalp. /his *ost likely is due to which of the followin& conditions. 1. Aplasia cutis con&enita $. Con&enital syphilis %. Keratosis 2ollicularis Spinulosa 0ecal3ans '. Morphea en coup de sa#re (. /rian&ular Alopecia ). Show +<plaination A ( Fi&hest &raft sur3i3al in hair transplantation is achie3ed throu&h the use of: 1. '"( ** plu&s $. Mini&rafts %. Sin&le hairs '. 2ollicular units (. Micro&rafts ). Show +<plaination 0 ) Ar&ininosuccinic aciduria is characteristically associated with this hair 6ndin& Jna*e the condition pictured #elow: 1. Pili trian&uli et canaliculi $. /richoschisis %. Monilethri< '. /richorrhe<is nodosa (. /richorrhe<is in3a&inata ). Show +<plaination 0 A Acute paronychia is *ost co**only caused #y which of the followin& or&anis*s. 1. Candida al#icans $. Candida tropicalis %. Pseudo*onas aeru&inosa '. Staphylococcus aureus (. /richophyton ru#ru* ). Show +<plaination 0 C A patient with diMuse se3ere sudden hair loss de3elopin& % *onths after hospitali;ation for septice*ia likely has which of the followin& conditions. 1. Ana&en eRu3iu* $. Cata&en eRu3iu* %. /elo&en eRu3iu* '. 8oose ana&en syndro*e (. Bnco*#a#le hair syndro*e ). Show +<plaination C D ,hich &enoder*atosis characteri;ed #y #road thu*#s and this nail disorder pictured #elow. 1. Hoth*und"/ho*pson $. /u#erous sclerosis %. Pachyonychia con&enita '. Proteus syndro*e (. Hu#enstein"/ay#i syndro*e ). Show +<plaination + 1! /he #est dia&nosis for this scarrin& for* of alopecia is 1. Chronic cutaneous lupus erythre*atosus $. 8ichen planopilaris 0 1(C %. Peseudopelade of BroL '. Perifolliculitis capitis a#scedens et suModiens (. Central centrifu&al cicatricial alopecia ). Show +<plaination 11 +ndony< onycho*ycosis is due to: 1. /richophyton ru#ru* $. /richophyton *enta&rophytes %. /richophyton soudanense '. Scytalidiu* di*idiatu* (. Scopuliaropsis ). Show +<plaination C 1$ A patient with diMuse hair loss de3elopin& after a thalliu* scan likely has which of the followin& conditions. 1. Ana&en eRu3iu* $. Cata&en eRu3iu* %. /elo&en eRu3iu* '. 8oose ana&en syndro*e (. Bnco*#a#le hair syndro*e ). Show +<plaination A 1% Air spaces in the hair shaft lead to this condition pictured #elow J na*e the condition: 1. Pili trian&uli et canaliculi $. Pili annulati %. /richoschisis '. Pohl Pinkus constriction (. ,ooly hair ). Show +<plaination B 1' /he defect associated with the hair 6ndin& shown in the i*a&e is: 1. hFB)hFB1 $. hFBAhFB$ %. hFBChFB% '. hFBDhFB' (. hFB1!hFB( ). Show +<plaination A 1( /erryGs nails are seen in which of the followin& conditions. 1. Henal failure $. Pul*onary 6#rosis %. 0ia#etes '. Bladder carcino*a (. Aerodi&esti3e carcino*a ). Show +<plaination C 1) A patient with curly hair that strai&htened after pu#erty: ena*el hypoplasia: dental pits: and increased #one density likely has a *utation in which of the followin& &enes. 1. 0istal"less ho*eo#o<"% &ene =08O%> $. ?ascular"endothelial &rowth factor receptor % %. Bone *orpho&enetic protein type $ '. S8BHP 1 (. SP1EK ( ). Show +<plaination A 1A Pro<i*al white su#un&ual onycho*ycosis with polydactylous in3ol3e*ent needs at least one la#oratory test: 1. Blood count $. Sedi*entation rate %. /"helper C0' ly*phocytes '. 8i3er en;y*es (. F1? ). Show +<plaination + 1C A patient has hyperkeratotic tissue on the lateral and pro<i*al nails folds. ,hat is the dia&nosis. 1. 9nychophosis $. 9nycho*adesis %. Fapalonychia '. 9nychocryptosis (. 9nychopha&ia ). Show +<plaination A 1D Black nail is caused #y: 1. /richophyton *enta&rophytes $. Proteus *ira#ilis %. Pseudo*onas spp. '. Staphylococcus aureus (. Fortaea werneckii ). Show +<plaination B $! A child presents with sparse: 6ne hair: thin nails and loose skin in infancy. Fe has a pear" shaped: #road nose and on O"ray has cone" shaped epiphyses. ,hich of the followin& 1. P)% $. 08O% %. HMHP '. /HPS1 0 1(D &enes is *utated in this syndro*e. (. ABCC) ). Show +<plaination $1 ,hich of the followin& syndro*es does E9/ ha3e the 6ndin& shown in the i*a&e as part of the spectru*. 1. Crandall $. Ar&ininosuccinic aciduria %. Citrulline*ia '. MenkeGs Kinky Fair (. Eetherton ). Show +<plaination A $$ ,hich part of the nail co*ple< results in nail pits. 1. Pro<i*al nail fold $. Pro<i*al nail *atri< %. 0orsal nail *atri< '. Eail plate (. Eail #ed ). Show +<plaination B $% ,hat is the &ene defect that causes red hair. 1. /yrosinase $. P protein %. Melanocortin 1 receptor '. /yrosine a*inotransferase (. /yrosinase"related protein 1 ). Show +<plaination C $' Si&ns of 3irili;ation include all of the followin& +OC+P/: 1. Centripetal o#esity $. /e*poral #aldin& %. Clitoro*e&aly '. 1ncreased *uscle *ass in the li*# &irdles (. Acne ). Show +<plaination A $( /rian&ular lunulae are: 1. Part of the syndro*e caused #y a 8MO1# *utation $. Associated with hyperplastic patellae %. Associated with a renal cell carcino*a '. Associated with a *utation of A/P$C1 (. Associated with a *utation of S+HCA$ ). Show +<plaination A $) A patient with wooly hair: diMuse pal*oplantar keratoder*a: and ri&ht 3entricular arrhyth*o&enic cardio*yopathy likely has a *utation in which of the followin& &enes. 1. FFB) $. Plako&lo#in %. Plakophilin '. SP1EK ( (. S8BHP 1 ). Show +<plaination B $A A co**only known cause of splinter he*orrha&es in the nail is endocarditis. ,hich of the followin& would #e unlikely to cause splinter he*orrha&es. 1. /richinosis $. /rau*a %. Psoriasis '. ?asculitis (. Hheu*atoid arthritis ). Show +<plaination + $C A si&ni6cantly increased nu*#er of Iclu# hairsI on a pull test is indicati3e of: 1. Ana&en eRu3iu* $. /elo&en eRu3iu* %. Eor*al hair anato*y '. Alopecia areata (. An&ro&enetic alopecia ). Show +<plaination B $D ,hich of the followin& is an e<a*ple of an apparent leukonychia. 1. /erryGs lines $. MeeGs lines %. MuehrckeGs lines '. FutchinsonGs lines (. 9nychoschi;ia ). Show +<plaination C %! A si< year old &irl presents after #ein& sent ho*e fro* school for IliceI. 9n e<a*ination: there are structures on the hair which are freely slide alon& the hair shaft. ,hat is the 1. Pediculosis $. Fair casts %. /richoptilosis '. Monilithri< B 1)! *ost likely dia&nosis. (. Pili annulati ). Show +<plaination %1 /he #est test for Cushin& syndro*e is: 1. Plas*a 8F $. Plas*a testosterone %. Plas*a prolactin '. Plas*a 0F+A (. 93erni&ht de<a*ethasone suppresion test ). Show +<plaination + %$ Sellow nails can #e seen in each of the followin& syndro*es e<cept: 1. Bronchiectasis $. Fyperlipide*ia %. /o#acco use '. /hyroid disease (. Penicilla*ine ). Show +<plaination B %% A patient with synophrys: dystopia canthoru*: and heterochro*ia iridu* irides likely has which of the followin& hair a#nor*alities. 1. 0iMuse thinnin& $. Scarrin& alopecia %. /richorrhe<is in3a&inata '. /richorrhe<is nodosa (. ,hite forelock ). Show +<plaination + %' /his hair 6ndin& is caused #y *utations in what &ene. 1. SP1EK ( $. E+M9 %. S+HCA$ '. 0yskerin (. Keratin 1) ). Show +<plaination A %( A su#un&ual he*ato*a co3erin& (!N of the nail#ed should #e *ana&ed #y which of the followin&. 1. Eo treat*ent $. He*o3al of the nail plate %. /rephination of the nail plate '. Eeedle aspiration of the he*ato*a (. Pressure dressin& ). Show +<plaination B %) AcLuired pro&ressi3e kinkin& of hair is a co*plication of which of the followin& *edications. 1. Anti*eta#olites $. Anti*alarials %. Hetinoids '. Slfona*ides (. 0apsone ). Show +<plaination C %A ,hich of the followin& syndro*es does not ha3e 0orsal ptery&iu* as a feature: 1. 8ichen planus $. 8esch"Eyhan syndro*e %. Cicatricial pe*phi&oid '. Chronic 5?F0 (. 8a*ellar ichthyosis ). Show +<plaination + %C MuehrckeGs nails are *ost often associated with: 1. Fyperthyroidis* $. Fypothyroidis* %. Fypoal#u*ine*ia '. Fyperal#u*ine*ia (. Eone of these answers are correct ). Show +<plaination C %D Choose the correct state*ent re&ardin& white piedra. 1. Co**only occurs in the tropics $. Consists of crea*"colored colonies attached to hairs %. /reat*ent consists of cuttin& the hair '. 1s caused #y piedra hortae (. Both Consists of crea*"colored colonies attached to hairs and /reat*ent consists of cuttin& the hair ). Show +<plaination + '! /he *ost co**on en;y*e a#nor*ality in con&enital adrenal hyperplasia is: 1. %"#eta"hydro<ysteroid dehydro&enase iso*erase $. 11"#eta"hydro<ylase %. $1"hydro<ylase '. 1("hydro<ylase C 1)1 (. 1A"hydro<ylase ). Show +<plaination '1 Pri*ary total dystrophic onycho*ycosis is due to: 1. Candida spp $. 2usariu* %. /richophyton ru#ru* '. Scytalidiu* di*idiatu* (. Scopulariopsis #re3icaulis ). Show +<plaination A '$ Spotted red lunulae are a#sent in which of the followin& conditions. 1. Alopecia areata $. Keratosis follicularis %. Syste*ic lupus erythe*atosus '. Hheu*atoid arthritis (. 8ichen planus ). Show +<plaination B '% 2rontal #ossin&: saddle nose: hypoplastic *idface: pe& shaped or conical teeth and hypopi&*ented short sparse scalp and #ody hair are pro*inent features of which of the followin& 1. Christ Sie*ens /ouraine syndro*e $. Fidrotic ectoder*al dysplasia %. Ar&inosuccinic aciduria '. Monilethri< (. Pachyonychia con&enita ). Show +<plaination A '' /he Ira&&ed cuticleI seen in der*ato*yositis is also known as: 1. Sa*it; si&n $. ?entral ptery&iu* %. Plu**ers si&n '. Candy"cane nails (. +*peripolesis ). Show +<plaination A '( /he co*#ination of poliosis: u3eitis: deafness: and 3itili&o are characteristic of which of the followin& conditions. 1. Pie#aldis* $. ,aardern#ur& syndro*e %. Cronkhite"Canada syndro*e '. ?o&t"Koyana&i"Farada syndro*e (. Cornelia de 8an&e Syndro*e ). Show +<plaination 0 ') 0eposition of *ucin in the hair follicle can #e associated with which disease. 1. Keratosis 2ollicularis Spinulosa 0ecal3ans $. Ada*s"9li3er Syndro*e %. Mycosis fun&oides '. Acne Keloidalis Euchae (. Perifolliculitis Capitis A#scedens et SuModiens ). Show +<plaination C 'A Con&enital &enerali;ed hypertrichosis is associated with which of the followin& sy*pto*s. 1. Andro&en"secretin& o3arian tu*ors $. Pituitary insuTciency %. 0istichiasis '. Methi*a;ole terato&enicity (. 5in&i3al 6#ro*atosis ). Show +<plaination + 'C ,hich of the followin& is E9/ a cause of red lunulae. 1. +ndocarditis $. Psoriasis %. Car#on *ono<ide poisonin& '. 1*uran (. Hheu*atoid arthritis ). Show +<plaination A 'D Pili trian&uli et canaliculi is the characteristic hair 6din& in which of the followin& disoders. 1. Monilethri< $. Pili annulati %. Ea<os syndro*e '. Anhidrotic ectoder*al dysplasia (. Bnco*#a#le hair syndro*e ). Show +<plaination + (! MeeGs lines are: 1. 0ou#le white trans3erse lines fro* a#nor*al 3ascular #ed $. Brownish *acules #eneath the nail plate %. 8ocal or diMuse hyperkeratotic tissue that de3elops on the lateral or pro<i*al nail folds 0 1)$ '. /rans3erse white lines that aMect all nails: &row out with nail &rowth (. ?ertical #lack lines on a sin&le or *ultiple nails ). Show +<plaination (1 0istal su#un&ual onycholysis associated with paronychia is due to: 1. Candida spp $. 2usariu* spp %. Scytalidiu* di*idiatu* '. /richophyton ru#ru* (. /richophyton *enta&rophytes ). Show +<plaination C ($ Psoriatic onycholysis is caused #y psoriasis in which of the followin& nail su#units. 1. Eail *atri< $. Eail plate %. Eail #ed '. Pro<i*al nail fold (. Fyponychiu* ). Show +<plaination C (% ,hich of the followin& *edication is a cause of hirsutis* without 3irili;ation. 1. 0apsone $. 0ia;o<ide %. 0ia;epa* '. 0initrochloro#en;ene (. 0antrolene ). Show +<plaination B (' /he 3isi#le portion of the nail *atri< is called the: 1. +ponychiu* $. 8unula %. Cuticle '. Fyponychiu* (. Eail plate ). Show +<plaination B (( /he co*#ination of a low"set hairline and synophrys is seen in which of the followin& conditions. 1. ,erner syndro*e $. /urner Syndro*e %. Eoonan Syndro*e '. Cornelia de 8an&e Syndro*e (. ,aarden#ur&Gs syndro*e ). Show +<plaination 0 () /he distal nail *atri< is 3isi#le at the followin& portion of the nail unit: 1. 9nychoder*al #and $. Fyponychiu* %. 8unula '. Cuticle (. 8ateral nailfold ). Show +<plaination C (A ,hich of the followin& are 6ndin&s associated with Eail"Patella Syndro*e. 1. 5lo*erulonephritis $. Alopecia %. Bladder di3erticuli '. Photosensiti3ity (. Fip dysplasia ). Show +<plaination A (C ,hich autoanti#ody is associated with these cuticular chan&es: shawl si&n: and o3erall fa3ora#le pro&nosis. 1. Mi"$ $. 7o"1 %. Fi&h titered AEA with speckled pattern '. Scl"A! (. Ho ). Show +<plaination A (D /richo#e;oar is is a potentially life"threatenin& co*plication of: 1. Alopecia areata $. Anhidrotic ectoder*al dysplasia %. /richotillo*ania '. 0iscoid lupus (. 8ichen planopilaris ). Show +<plaination C )! Plu**erGs nails are associated with which of the followin& disorders. 1. 0er*ato*yositis $. Syste*ic sclerosis %. 8upus erythe*atosus '. Fyperthyroidis* (. 8ead poisonin& 0 1)% ). Show +<plaination )1 /ayJs syndro*e is characteri;ed #y this hair condition pictured #elow J identify the condition: 1. /richorrhe<is nodosa $. /richorrhe<is in3a&inata %. /richoschisis '. Monilethri< (. Pili trian&uli et canaliculi ). Show +<plaination C )$ He&ardin& andro&ens in wo*en: which of the followin& state*ents is E9/ correct. 1. /estosterone #inds the andro&en receptor $. +ye#rows: eyelashes and 3ellus hairs are andro&en"dependent %. /he hair follicle reLuires con3ersion of testosterone to dihydrotestosterone for e<pression of andro&en action '. 0ihydrotestosterone #inds the andro&en receptor (. /here are no diMerences in eyelashes: eye#rows and 3ellus hair" #earin& areas in *en and wo*en ). Show +<plaination B )% A $' year"old wo*an presents with the co*plaint of hirsutis* and asks a#out therapy. She has a 3irili;ation pattern of hair &rowth. 9n Luestionin&: she relates ha3in& irre&ular *enstrual periods and acne. ,hich of the followin& is the *ost likely dia&nosis. 1. Pheochro*ocyto*a $. Polycystic o3arian disease %. Con&enital adrenal hyperplasia '. 93arian carcino*a (. Eor*al 3ariant ). Show +<plaination B )' 8indsayGs nails =distal nail nor*al: pro<i*al nail white> is characteristic of: 1. Chronic renal failure $. Plu**er"?inson syndro*e %. Fe*ochro*atosis '. +ctoder*al dysplasia (. 8+9PAH0 syndro*e ). Show +<plaination A )( Sellow nail syndro*e has #een associated with use of which of the followin& treat*ents for Hheu*atoid Arthritis. 1. 0"penicilla*ine $. Methotre<ate %. 1n-i<i*a# '. +tanercept (. Adali*u*a# ). Show +<plaination A )) Mutations in c"kit are associated with which of the followin& conditions. 1. ,aarden#ur& syndro*e $. Pie#aldis* %. /u#erous sclerosis '. ?o&t"koyana&i"harada (. Cornelia de 8an&e Syndro*e ). Show +<plaination B )A An 1C year"old &irl who was hospitali;ed last *onth after a serious car accident is noted to ha3e white trans3erse &roo3es on her 6n&ernails and toenails. /he *ost likely dia&nosis is: 1. /wenty"nail dystrophy $. BeauJs lines %. Falf"and"half nails '. MeesJ lines (. /erryJs nails ). Show +<plaination B )C /rian&ular lunulae are seen in what disorder. 1. 0yskeratosis con&entia $. Eeuro6#ro*atosis 11 %. 5orlinJs syndro*e '. Eail"patella syndro*e (. Papillon"8efe3re syndro*e ). Show +<plaination 0 )D ?entral ptery&iu* pictured #elow is characteristically associated with what disorder. 1. 8ichen planus $. Syste*ic sclerosis %. Cicatricial pe*phi&oid '. S7S/+E (. Chronic 5?F0 ). Show +<plaination B A! /rans3erse white #ands on one or two nails is caused #y: 1. /rau*a to the *atri< $. Alopecia areata %. Psoriasis A 1)' '. Syste*ic lupus erythe*atosis (. 8ichen planus ). Show +<plaination A1 ,hat condition *ost likely descri#es a hair defect with ruRed cuticle in '"year old fe*ale with #lond hair. 1. Pili torti $. Pili annulati %. ,ooly hair '. 8oose ana&en hair syndro*e (. Monilethri< ). Show +<plaination 0 A$ Mutations in hair keratins hFB) and hFB1 cause this characteristic 6ndin& pictured #elow J na*e the condition: 1. /richoptilosis $. Fair casts %. /i&er"tail hair '. /richoschisis (. Monilethri< ). Show +<plaination + A% Blue lunulae are characteristic of which disease. 1. Sellow nail syndro*e $. ,ilsonGs disease %. Hu#enstein"/ay#i syndro*e '. Fypertrophic pul*onary osteoarthropathy (. Clu##in& ). Show +<plaination B A' /he *ost co**on cultured a&ent in chronic paronychia associated with a #lack hue of the lateral ed&e of the nail plate is: 1. /richophyton ru#ru* $. &ra*"ne&ati3e #acteria %. Candida spp '. Scytalidiu* di*idiatu* (. Scopulariopsis #re3icaulis ). Show +<plaination C A( /his nail 6ndin& is characteristically associated with what disorder. 1. Con&enital heart failure $. Chronic renal failure %. Ane*ia '. Fyperthyroidis* (. Cirrhosis ). Show +<plaination B A) Papular atrichia is caused #y *utations in which &ene. 1. ,in&less $. Patched %. Fairless '. 2o< forehead (. 0istal"less ho*e#o#o< ). Show +<plaination C AA Sellow nail syndro*e can #e associated with: 1. 0"penicilla*ine use in patients with rheu*atoid arthritis $. ,ilsonJs disease %. Alopecia areata '. 0o<oru#icin (. Minocycline use in acne patients ). Show +<plaination A AC /he hair 6ndin& characteri;ed #y an in3a&ination of the distal hair shaft into the cup for*ed #y the pro<i*al hair shaft is: 1. /richoschisis $. Pili torti %. Pili trian&ulati '. /richorrhe<is nodosa (. /richorrhe<is in3a&inata ). Show +<plaination + AD /hin hair with pre*ature &rayin& is characteristic of which of the followin& syndro*es. 1. ,erner syndro*e $. /urner Syndro*e %. Eoonan Syndro*e '. Cornelia de 8an&e Syndro*e (. ,aarden#ur&Gs syndro*e ). Show +<plaination A C! ,hich of the followin& is the predo*inant der*atoscopic 6ndin& seen in alopecia areata. 1. 0iMuse white knots and a #rush" pattern $. Perifollicular ar#ori;in& 3essels %. Sellow dots '. Heduction of follicular ostia (. S*all o3al nodes C 1)( ). Show +<plaination C1 2rayin& of the cuticles is a si&n of which of the followin& conditions. 1. 0er*ato*yositis $. Syste*ic sclerosis %. 8upus erythe*atosus '. Fypothyroidis* (. 8ead poisonin& ). Show +<plaination A C$ Chronic paronychia is *ost co**only caused #y which of the followin& or&anis*s. 1. Candida al#icans $. Candida tropicalis %. Pseudo*onas aeru&inosa '. Staphylococcus aureus (. /richophyton ru#ru* ). Show +<plaination A C% A wo*an with hypopi&*ented in lines of Blaschko and scarrin& alopecia likely suMers which of the followin& conditions. 1. Chondrodysplasis punctata $. Anhidrotic +ctoder*al 0ysplasia %. 2ocal 0er*al Fypoplasia '. Hoth*und"/ho*son Syndro*e (. Bloch"Sul;#er&er Syndro*e ). Show +<plaination + C' Fow lon& should the last wash"out period #efore perfor*in& *ycolo&ic in3esti&ations in a clinically recurrent onycho*ycosis treated #y ter#ina6ne: itracona;ole or -ucona;ole: 1. 9ne *onth $. /wo *onths %. /hree *onths '. 2our *onths (. Si< *onths ). Show +<plaination C C( Blue lunulae are associated with each of the followin& e<cept: 1. Ar&yria $. Cardiac failure %. Quinacrine '. ,ilsonJs disease (. Phenolphthalein ). Show +<plaination B C) ,hich part of the *atri< &i3es rise to the dorsal nail plate. 1. 0istal *atri< $. Pro<i*al *atri< %. ?entral *atri< '. 0orsal *atri< (. 8ateral *atri< ). Show +<plaination B CA A patient with sparse hair: a pear"shaped #road nose and cone"shaped epiphyses likely suMers which of the followin& conditions. 1. 2ocal 0er*al Fypoplasia =5olt; syndro*e> $. /richo"rhino"phalan&eal Syndro*e %. Fay",ells Syndro*e '. +ctrodactyly"+ctoder*al dysplasia" Cleftin& =++C> (. /richodentoosseous Syndro*e ). Show +<plaination B CC Atrophy of the isth*us is a characteristic histolo&ic 6ndin& of which of the followin& conditions. 1. 0iscoid lupus erythe*atosus $. 8ichen planopilaris %. Parry"Ho*#er& syndro*e '. 2olliculitis 0ecal3ans (. Acne Keloidalis Euchae ). Show +<plaination B CD A patient with cirrhosis has noticed that the pro<i*al aspect of his nails ha3e #eco*e white. /his characteristic 6ndin& is called: 1. Falf"and"half nails $. MeesG lines %. BeauGs lines '. /erryGs lines (. MuehrckeGs lines ). Show +<plaination 0 D! 2erritin le3els need to #e: at *ini*u*: a#o3e which of the seru* le3els to treat iron" de6ciency related telo&en eRu3iu*. 1. $!n&d8 $. '!n&d8 %. )!n&d8 '. %!n&d8 (. 1!n&d8 ). Show +<plaination B D1 /he *ost co**on cause for super6cial type onycho*ycosis is: 1. /richophyton ru#ru* $. /richophyton *enta&rophytes B 1)) %. +pider*ophyton -occosu* '. 2usariu* o<ysporu* (. Scopulariopsis #re3icalis ). Show +<plaination D$ /he co**on culprit of pro<i*al white su#un&ual onycho*ycosis is : 1. Candida al#icans $. /richophyton ru#ru* %. /richophyton interdi&itale '. Scydalidiu* di*idiatu* (. 2usariu* spp ). Show +<plaination B D% ,hat deter*ines the thickness of the nail plate. 1. Fyponychiu* $. Eail *atri< %. Eail #ed '. Pro<i*al nail fold (. Eail plate ). Show +<plaination B D' ,hich of the followin& syndro*es can present with woolly or curly hair. 1. B4ornstad syndro*e $. Citrulline*ia %. Menkes syndro*e '. Eoonan syndro*e (. Eetherton syndro*e ). Show +<plaination 0 D( 2ollicular atrophoder*a is a feature of which of the followin& conditions. 1. Ba;e< syndro*e $. Menkes kinky hair syndro*e %. Papular atrichia '. /richo"dento"osseous syndro*e (. /richo"rhino"phalan&eal syndro*e ). Show +<plaination A D) A rapid onset of hair &rowth with or without acco*panyin& 3irili;ation can occur in all of the followin& conditions +OC+P/: 1. Adrenal adeno*a $. Adrenal carcino*a %. Arrheno#lasto*a '. Kruken#ur& tu*or of the o3ary (. 93arian cyst ). Show +<plaination + DA A patient with psoriasis has pittin& of the nails. /his 6ndin& is due to in3ol3e*ent of which part of the nail unit. 1. Pro<i*al *atri< $. Eail #ed %. Fyponychiu* '. Pro<i*al nail fold (. 0istal *atri< ). Show +<plaination A DC A '$ year"old wo*an presents with the co*plaint of e<cess hair &rowth on her face. She has nor*al *enses and has recently had her IannualI e<a* and the note relates nor*al si;ed o3aries. ,hat is the *ost lo&ical ne<t step. 1. Check plas*a le3els of androstenedione and testosterone $. Send a $1"hydro<ylase en;y*e de6ciency test %. Biopsy fro* the *ost aMected area '. Hefer her to endocrinolo&y (. 9rder a C/ of the a#do*en ). Show +<plaination A DD /his characteristic nail 6ndin& is seen in what disorder. 1. 8ichen planus $. Pachyonychia con&enita %. 0arierGs disease '. C912 syndro*e (. CoTn"Siris syndro*e ). Show +<plaination C 1! ! /his hair disorder and *utations in A/PAA =MEK> &ene are found in what &enoder*atosis. 1. B4ornstad syndro*e $. MenkeGs kinky hair syndro*e %. CrandallGs syndro*e '. Ba;e<Gs follicular atrophoder*a (. /richothiodystrophy ). Show +<plaination B 1! 1 0istal su#un&ual onycho*ycosis is *ost often caused #y: 1. /richophyton *enta&rophytes $. +pider*ophyton -occosu* %. /richophyton schoenleinii '. /richophyton ru#ru* (. /richophyton *e&ninii 0 1)A ). Show +<plaination 1! $ ,hat is the *ode of inheritance of this disorder of the hair shaft. 1. Autoso*al do*inant $. Autoso*al recessi3e %. O"linked do*inant '. O"linked recessi3e (. Sporadic ). Show +<plaination A 1! % A patient with a history of &astrointestinal polyposis presents with alopecia: &enerali;ed pi&*entation: and nail dystrophy. ,hich of the followin& is the *ost likely dia&nosis. 1. Peut;"7e&her syndro*e $. 5ardner syndro*e %. Cronkhite"Canada Syndro*e '. Cowden syndro*e (. 0yskeratosis con&enita ). Show +<plaination C 1! ' ,hat is the co**on cause of the Jone hand" two"footJ syndro*e. 1. /richophyton ru#ru* $. /richophyton *enta&rophytes = 3ar. 1nterdi&itale> %. Candida al#icans '. Scytalidiu* di*idiatu* (. Scopulariopsis #re3icaulis ). Show +<plaination A 1! ( ,hat nutritional de6ciency can lead to this nail 6ndin&. 1. ?ita*in B1$ $. ?ita*in C %. 1ron '. Ma&nesiu* (. /hia*ine ). Show +<plaination C 1! ) Arsenical poisonin& is associated with what nail 6ndin&. 1. MuehrckeGs lines $. MeeGs lines %. Fapalonychia '. 9nycholysis (. 9il spots ). Show +<plaination B 1! A Anonychia is not a feature of which of the followin& syndro*es. 1. Eail"Patella syndro*e $. CoTn"Siris syndro*e %. 099H syndro*e '. C912 syndro*e (. Fidrotic ectoder*al dysplasia ). Show +<plaination + 1! C /he epitheliu* that lies on the 3olar surface of the di&it is the: 1. Eail #ed $. Eail *atri< %. Eail plate '. Fyponychiu* (. +ponychiu* ). Show +<plaination 0 1! D Best dia&nosis of this type of cicatricial alopecia shown in attached i*a&e is 1. Chronic cutaneous lupus erythe*atosus $. 8ichen planopilaris %. Central centrifu&al cicatricial alopecia '. Pseudopelade of BrocL (. Alopecia *ucinosa ). Show +<plaination C 11 ! 2un&al 6n&er onycholysis usually results fro*: 1. /richophyton ru#ru* $. /richophyton interdi&itale %. Candida al#icans '. Scopulariopsis #re3icaulis (. 2usariu* spp ). Show +<plaination C 11 1 Cutaneous si&ns of cortisol e<cess include all of the followin& +OC+P/: 1. Plethora $. Atrophicfra&ile skin %. Striae distensae '. 0orsocer3icalSupracla3icular fat pads (. Peripheral o#esity ). Show +<plaination + 1)C 11 $ Steatocysto*a *ultiple< and natal teeth are associated with which of the followin& conditions. 1. 7adassohn"8ewandowsky pachyonychia con&enita =/ype 1> $. 7ackson"Sertole pachyonychia con&enita =/ype $> %. Eail"patella syndro*e '. CoTn"Siris syndro*e (. Hu#enstein"/ay#i syndro*e ). Show +<plaination B 11 % Pili Annulati is associated with which of the followin& syndro*es. 1. Eetherton $. P1B10S %. Citrulline*ia '. Fidrotic ectoder*al dysplasia (. Alopecia areata ). Show +<plaination + 11 ' /he co*#ination of ankylo#lepharon: ectoder*al dysplasia: and cleft palate with wiry sparse hair is characteristic of which of the followin& syndro*es. 1. 2ocal 0er*al Fypoplasia =5olt; syndro*e> $. /richo"rhino"phalan&eal Syndro*e %. Fay",ells Syndro*e '. +ctrodactyly"+ctoder*al dysplasia" Cleftin& =++C> (. /richodentoosseous Syndro*e ). Show +<plaination C 11 ( A defect in which part of the nail cause a true leukonychia. 1. Cuticle $. Fyponychiu* %. 8unula '. Eail #ed (. Eail plate ). Show +<plaination + 11 ) ,hich of the followin& conditions with hair a#nor*alities is caused #y *utations in a 0EA helicases. 1. Anhidrotic +ctoder*al 0ysplasia $. Fidrotic +ctoder*al 0ysplasia %. K10 Syndro*e '. Hoth*und"/ho*son Syndro*e (. 1ncontinentia Pi&*enti ). Show +<plaination 0 11 A Sellow nail syndro*e is associated with which of the followin&. 1. Multiple *yelo*a $. Panhypopituitaris* %. 0ia#etes *ellitus '. 0er*atophyte infection (. 8y*phede*a and #ronchiectasis ). Show +<plaination + 11 C Patho&no*onic nails chan&es in F990 syndro*e or Fereditary osteoonychodysplasia are: 1. Hed and white lon&itudinal #andin& $. Ptery&iu* in3ersu* %. Sellow nails '. Pincer nails (. /rian&ular lunulae ). Show +<plaination + 11 D ,hich of the followin& are characteristics of /richorhinophalan&eal syndro*e. 1. Kinky hair: frontal #ossin&: s*all widely spaced teeth with poor ena*el $. Pal*oplantar keratoder*a: trichorrhe<is nodosa: sinus infections %. Shortened phalan&es: sparse hair: #ul#ous nose '. Brittle hair: short stature: cere#ellar ata<ia (. Sparse 6ne hair: Short e<tre*ities: 1**unode6ciency ). Show +<plaination C 1$ ! ,hich of the followin& is caused #y *utations in &ap 4unction proteins. 1. Hoth*und"/ho*pson syndro*e $. Fidrotic ectoder*al dysplasia %. Anhidrotic ectoder*al dysplasia '. Eetherton syndro*e (. Ea<os syndro*e ). Show +<plaination B 1$ A patient presents with $! nails with a#sent 1. Bpper e<tre*ity ede*a C 1)D 1 cuticles and lunulae: slow &rowth: dystrophic shape and a yellow hue. ,hich of the followin& 6ndin&s isare associated. $. Cirrhosis %. Pleural eMusions '. Chest pain (. Pul*onary 6#rosis ). Show +<plaination 1$ $ Splinter he*orrha&e of the nail can #e seen with which of the followin& parasitic infections. 1. Sca#ies $. /richinosis %. Spar&anosis '. 0racunculiasis (. 5nathosto*iasis ). Show +<plaination B 1$ % A patient with yellow nail syndro*e should #e e3aluated for syste*ic disease in3ol3in& which or&an syste*. 1. Fepatic $. Henal %. Pul*onary '. Cardio3ascular (. Fe*atolo&ic ). Show +<plaination C 1$. Bullous and ?esicular 0er*atoses # Question Answers Corre ct Answ er 1 Eickel can #e detected in 4ewelry #y applyin&: 1. 0i*ethyl&lyo<i*e $. Methylchloroisothia;olinone %. 0owicil '. Quinone (. /hiocyanate ). Show +<plaination A $ ,hich pair is not associated with aller&ic cross" reacti3ity. 1. /hi*erosal : piro<ica* $. +thylenedia*ine : hydro<y;ine %. PABA : procaine '. Balsa* of Peru : cinna*on (. /hiura* : sulfa dru&s ). Show +<plaination + % ,hat is the *ost co**on site of in3ol3e*ent of this autoi**une #listerin& disease. 1. 9ral *ucosa $. +yes %. Skin '. 5enitalia (. Easopharyn&eal *ucosa ). Show +<plaination A ' A child has a defect in type ?11 colla&en and presents with acral #listers which heal with *ilia and scarrin&. ,hat is the dia&nosis. 1. Al#opapuloidPasini 3ariant of dystrophic epider*olysis #ullosa $. BartGs syndro*e %. Cockayne"/ouraine disease '. +pider*olysis #ullosa acLuisita (. ,e#er"Cockayne ). Show +<plaination C ( Patients with Senear"Bsher syndro*e are *ost likely to present with: 1. +rythe*atous papules and plaLues around the u*#ilicus $. /ransient 3esicles on the oral *ucosa + 1A! %. Se3erely pruritic &rouped 3esicles sy**etrically distri#uted pri*arily on e<tensor surfaces '. +rythe*a *ultifor*e"like oral ulcerations which are se3ere (. +rythe*atous crusts and hyperkeratotic lesions on the nose: ears: cheeks: scalp: and chest ). Show +<plaination ) ,hich is incorrect. 1. /ulipalin : alstroe*eria $. Brushiol : anacardia %. SesLuiterpene lactones : artichokes '. Bsnic acid : lichen (. 0iallyl disul6de : 6& ). Show +<plaination + A Anti#odies a&ainst type ?11 colla&en are seen in: 1. +pider*olysis #ullosa si*ple< $. Pe*phi&us erythe*atosus %. Cicatricial pe*phi&oid '. +pider*olysis #ullosa acLuisita (. Bullous pe*phi&oid ). Show +<plaination 0 C Atopic der*atitis is associated with all e<cept: 1. 8chthyosis hystri< $. Central facial pallor %. Pityriasis al#a '. Eipple ec;e*a (. Fyperlinear pal*s ). Show +<plaination A D Perioral e<u#erant &ranulation tissue is so*eti*es seen in: 1. Cicatricial pe*phi&oid $. Ferlit; %. 0uhrin&Gs '. ,e#er"Cockayne (. Ste3ens"7ohnson ). Show +<plaination B 1! Co**on causes of dru&"induced linear 1&A: 1. ?anco*ycin $. Penicillin %. Cephalosporins '. Captopril (. All of these answers are correct ). Show +<plaination + 11 ,hich of the followin& concernin& 0e&osG 0isease is true. 1. After under&oin& *ultiple sta&es: it resol3es without scarrin& $. 1t aMects wo*en *ore than *en %. 5atrointestinal in3ol3e*ent is a poor pro&nosis '. 5lucocorticoids are standard of treat*ent (. 8a# results indicate a low plas*a 6#rino&en le3el and decreased platelet a&&re&ation ). Show +<plaination C 1$ /he ocular for* of cicatricial pe*phi&oid is *ost likely to #e associated with anti#odies to: 1. Beta"'"inte&rin $. 8a*inin ( %. BPA&1 '. BPA&$"EC1)A (. Plectin ). Show +<plaination A 1% /his is found in #oth des*oso*es and adherens 4unctions: 1. Alpha"catenin $. 0es*o&lein % %. 0es*o&lein 1 '. Plako&lo#in (. 8a*in ). Show +<plaination 0 1' Anti#odies to which anti&en are *ost likely 1. Bullous pe*phi&oid anti&en 11 A 1A1 responsi#le for this disease. $. 0es*o&lein 1 %. 0es*ocollin '. Keratin ( (. Plako&lo#in ). Show +<plaination 1( An elderly &entle*an with a history of hypertension and a thy*o*a: presents to the +*er&ency Hoo* with oral and con4uncti3al erosions and he*orrha&ic #ullae on his hands and feet. /hese 6nds are seen in: 1. Cicatricial pe*phi&oid $. SweetJs syndro*e %. /o<ic epider*al necrolysis '. Paraneoplastic pe*phi&us (. +rythe*a *ultifor*e ). Show +<plaination 0 1) ,hich of the followin& is true of herpes &estationis. 1. 0e*onstrates anti#odies to the C" ter*inal do*ain of BPA&$ $. Fas hi&her freLuency in fe*ales with F8A"0Q$ %. 1s associated with 5ra3eGs disease '. Bsually occurs in the 6rst tri*ester of pre&nancy (. Shows su#epider*al #ullae *ostly with neutrophils ). Show +<plaination C 1A Characteristic eosinophilic a#scesses are seen in: 1. Bullous dru& $. Pe*phi&us 3e&etans %. Ferpes &estationis '. 1ncontinentia pi&*enti (. Paraneoplastic pe*phi&us ). Show +<plaination B 1C At what le3el is the #lister separation plane in linear 1&A der*atosis. 1. 5ranular layer $. Supra#asal layer %. Basal layer '. 8a*ina lucida (. 8a*ina densa ). Show +<plaination 0 1D A )! year old &entle*an presents with a recurrent pustular eruption. Biopsy de*onstrates su#corneal pustules with a#undant neutrophils and a seru* protein electrophoresis shows a *onoclonal 1&A &a**opathy. /he autoanti&en responsi#le for this condition: 1. 0es*o&lein 1 $. 0es*ocollin 1 %. 0es*oplakin '. Plako&lo#in (. Beta ' inte&rin ). Show +<plaination B $! Anti#odies in chronic disease of childhood disease: *ap to the: 1. Basal layer $. 8a*ina lucida %. 8a*ina densa '. Su#la*ina densa (. 8a*ina lucida and su#la*ina densa ). Show +<plaination + $1 A %( year"old dentist presents with tin&lin& in his 6n&ertips. ,hat aller&en is *ost likely to cause this der*atitis. 1. Methyl *ethacrylate $. Paraphenylenedia*ine %. Colophony '. /hiura* *i< (. +thylenedia*ine dihydrochloride ). Show +<plaination A $$ ,hich of the followin& proteins is a 1)!k0a cadherin. 1. 0es*o&lein % $. 0es*o&lein 1 %. 0es*oplakin 1 '. +n3oplakin (. Plectin ). Show +<plaination B $% ,hich of the followin& i**unosuppressi3e a&ents has #een *ost eMecti3e in cicatricial pe*phi&oid. 1. Cyclophospha*ide $. A;athioprine %. Mycophenolate *ofetil '. Cyclosporine (. Methotre<ate ). Show +<plaination A $' Ferpes &estationis is e<acer#ated #y: 1. 9ral contracepti3es + 1A$ $. Menstruation %. /hird tri*ester '. Postpartu* state (. All of these answers are correct ). Show +<plaination $( 0er*atitis herpetifor*is is associated with: 1. F8A"Cw) $. F8A"B$A %. F8A"0H' '. F8A"0Q1 (. F8A"0H% ). Show +<plaination + $) ,hat is the anti&en found in the 3ariant of cicatrical pe*phi&oid that is associated with internal *ali&nancies. 1. BPA&1 $. BPA&$ %. /ype ?11 colla&en '. +pili&rin (. Keratins ( @ 1' ). Show +<plaination 0 $A /he rate li*itin& step in the porphyria pathway is *ediated #y the en;y*e: 1. 2errochetalase $. A8A dehydratase %. A8A synthase '. Broporphyrino&en decar#o<ylase (. Porpho#ilino&en dea*inase ). Show +<plaination C $C Co**on dru&s causin& S7S/+E include all e<cept: 1. Sulfona*ides $. Car#a*a;epi*e %. Allopurinol '. 9<ica* (. Colchicine ). Show +<plaination + $D ,hich association is incorrect. 1. +pider*olysis #ullosa acLuisita : in-a**atory #owel disease $. 0er*atitis herpetifor*is : s*all #owel ly*pho*a %. Paraneoplastic pe*phi&us : Castle*an[s '. Ferpes &estationis : *enopause (. Porphyria cutanea tarda : he*ochro*atosis ). Show +<plaination 0 %! ,hich of the followin& is true of a phototo<ic reaction. 1. HeLuires prior sensiti;ation of photoaller&en in suscepti#le indi3iduals $. Photoaller&en *ust #ind to carrier protein %. 0e3elops after repeated e<posures '. Produces an ec;e*atous reaction (. Hesults in direct tissue in4ury ). Show +<plaination + %1 ,hich of the followin& is the *ost co**on photoaller&en. 1. octyl di*ethyl PABA $. 0ihydro<yacetone %. Ben;ophenone '. 9<y#en;one (. &lyceryl thio&lycolate ). Show +<plaination 0 %$ Bullous dia#eticoru* typically presents on the: 1. 2ace $. Chest %. Ar*s '. 8e&s (. 5roin ). Show +<plaination 0 %% +pider*olysis #ullosa si*ple< is caused #y *utations in: 1. Keratins 1@1! $. Keratins 1@D %. Keratins (@1' '. Keratins )a @1) C 1A% (. Keratins K$e@1! ). Show +<plaination %' 2or*aldehyde releasers include all e<cept: 1. Methylchoroisothia;olinone $. 5er*all %. Quaterniu* J 1( '. 0owicil (. 0M0M hydantoin ). Show +<plaination A %( 2i#er&lass der*atitis can #e pre3ented #y: 1. ,ater $. Acetic acid (N %. Sodiu* chloride '. Alkali (. /alcu* powder ). Show +<plaination + %) ,hich type of porphyria disease is *ost likely to #e associated with cholelithiasis. 1. Acute inter*ittent porphyria $. Con&enital erythropoeitic porphyria %. Fereditary coproporphyria '. +rythropoetic protoporphyria (. ?arie&ate porphyria ). Show +<plaination 0 %A /he la*ina densa is pri*arily co*posed of. 1. Conne<ins $. Plectin %. Colla&en O?11 '. Colla&en ?11 (. Colla&en 1? ). Show +<plaination + %C +tyhlenedia*ine dihydrochloride is known to cross"react with all the followin& su#stances e<cept. 1. Eeo*ycin $. A*inophylline %. Pro*etha;ine '. Mecli;ine (. Fydro<y;ine ). Show +<plaination A %D /he A#soe"Fansen si&n as applies to #lister for*ation refers to 1. 8ateral slippin& of the epider*is when perilesional is ru##ed $. 8ateral dissection of the #lister when pressure is applied directly to a #lister %. Blister for*ation followin& strokin& of the lesion '. Blister induction with incised trau*a to skin (. Blister for*ation after cryosur&ery ). Show +<plaination B '! /he C"ter*inal do*ain of BPA&$ is tar&eted in: 1. Bullous pe*phi&oid $. Cicatricial pe*phi&oid %. 1&A pe*phi&us '. 0uhrin&Js disease (. Pe*phi&oid &estationis ). Show +<plaination B '1 /he *ost co**on cause of contact der*atitis in the Bnited States is: 1. Fair dyes $. Eickel %. Eail lacLuers '. /o<icodendrons (. Hu##er ). Show +<plaination 0 '$ A patient who works in a photo&raphy de3elopin& la# co*plains of ec;e*atous der*atitis on his hands. ,hich one of the followin& che*icals is he *ost likely aller&ic to. 1. Mercapto#en;othia;ole $. 8anolin %. +po<y resin '. Parapheylenedia*ine (. Colophony ). Show +<plaination 0 '% Bullous lupus erythe*atosus is *ost co**only associated with anti#odies to: 1. /ype O11 colla&en $. /ype ?11 colla&en B 1A' %. Plectin '. 0es*o&lein 1 (. 8a*inin ( ). Show +<plaination '' Pinus palustris is the source of: 1. Brea $. 0"li*onene %. Quinones '. 8actones (. A#ietic acid ). Show +<plaination + '( ,hich of the followin& for*s of epider*olysis #ullosa is autoso*al recessi3e. 1. ,e#er"Cockayne $. 0owlin&"Meara %. 7unctional +B with pyloric atresia '. +B si*ple< with *uscular dystrophy (. Fyperplastic cockayne"touraine ). Show +<plaination C ') /his *edicine causes de&ranulation of *ast cells 1. ESA10s $. ASA %. opiates '. poly*i<in B (. All of these answers are correct ). Show +<plaination + 'A 8ate< aller&y can cross"react with all e<cept: 1. A3ocado $. Banana %. Kiwi '. Chestnut (. Artichoke ). Show +<plaination + 'C 8einerGs disease =erythroder*a desLua*ati3u*> is associated with: 1. C("D de6ciency $. Eu*erous infections %. 0iarrhea '. Eu*erous infections and diarrhea (. All of these answers are correct ). Show +<plaination 0 'D /he croton plant is irritatin& secondary to: 1. Calciu* o<alate $. /hiocyanate %. Protoane*onin '. Phor#ol esters (. Capsaicin ). Show +<plaination 0 (! Ferpes &estationis is *ost co**only associated with: 1. 5ra3eGs $. Fashi*otoGs %. 0ia#etes '. 8upus (. Hheu*atoid arthritis ). Show +<plaination A (1 Clu*ped tono6la*ents are seen on electron *icroscopy in: 1. 0owlin&"Meara $. ,e#er"Cockayne %. 5untherJs '. Ea<os disease (. ?ohwinkelJs syndro*e ). Show +<plaination A ($ ,hat is the *ost co**on cause of this entity. 1. 0ru&s $. 1nfections %. Mali&nancies '. 1diopathic (. B? e<posure ). Show +<plaination A (% 5ap 4unctions consist of: 1. 8a*inin $. Plectin %. Bncein '. 2ila&rin (. Conne<ins ). Show +<plaination + 1A( (' /his is deri3ed fro* Myro<ylon pereirae: 1. /hi*erosol $. Aniline dyes %. Balsa* of Peru '. Colophony (. /hiura* ). Show +<plaination C (( ,hich of the followin& a&ent=s> has #een *ost eMecti3e in treatin& se3ere ocular cicatricial pe*phi&oid. 1. Cyclophospha*ide $. Mycophenolate *ofetil %. Cyclophospha*ide P corticosteroids '. Cyclosporin (. A;athioprine ). Show +<plaination C () Chloracne *ay #e secondary to e<posure to: 1. Chloroacetophenone $. Fydrochloric acid %. Fydrocar#ons '. 0io<in (. Methylchloroisothia;olinone ). Show +<plaination 0 (A Ferpes &estationis is *ost co**only associated with: 1. 8y*pho*a $. Multiparity %. 5ra3eGs disease '. 1n-a**atory #owel disease (. 8upus ). Show +<plaination C (C ,hich for* of epider*olysis #ullosa presents with &enerali;ed #ullae: a#sent nails: dysplastic teeth =due to ena*el defects>: nonhealin& &ranulation tissue periorally: and is often fatal #y a&e %"'. 1. ,e#er"cockayne $. Ferlit; %. Eon"Ferlit; '. Fyperplastic cockayne"touraine (. Al#apapuloid Pasini 3ariant ). Show +<plaination B (D ,hich of the followin& is not a for*aldehyde" releasin& preser3ati3e. 1. Bronopol $. Methylchloroisothia;inolone %. Quaterniu*"1( '. 1*ida;olidinyl urea (. 0M0M hydantoin ). Show +<plaination B )! /he epider*olysis #ullosa acLuisita anti&en is: 1. A trans*e*#rane protein $. An intracellular protein %. A des*oso*al protein '. 8ocated in the la*ina lucida (. An anchorin& 6#ril ). Show +<plaination + )1 ,hich type of porphyria *anifests with peripheral neuropathy: colicky a#do*inal pain: #ut E9 cutaneous sy*pto*s. 1. Acute 1nter*ittent Porphyria $. 5untherGs disease %. ?arie&ate porphyria '. Fereditary Copropophyria (. +rythropoietic protopophyria ). Show +<plaination A )$ 0irect i**uno-uorescent studies in chronic #ullous disease of childhood is *ost likely to show: 1. 1&a depostion in the super6cial #lood 3essels $. 8inear 1&5 at the #ase*ent *e*#rane %. 8inear 1&A at the #ase*ent *e*#rane '. 5ranular 1&5 (. 8inear C% at the #ase*ent *e*#rane ). Show +<plaination C )% Circulatin& autoanti#odies to type O?11 colla&en are *ost characteristic of which disease. 1. +pider*olysis #ullosa accLuisita $. Ferpes &estationis %. Pe*phi&us 3ul&aris '. Pe*phi&us foliacious (. Paraneoplastic pe*phi&us ). Show +<plaination B 1A) )' A wo*an in her $nd tri*ester of pre&nancy presents to clinic with urticarial plaLues and papules around her u*#ilicus: chest and e<tre*ities. /ense 3esicles are present within a few of the erythe*atous plaLues. /his wo*anXaYs condition is *ost co**only associated with: 1. 8y*pho*a $. Multiparity %. 5ra3eX Ys 0isease '. 1n-a**atory Bowel 0isease (. 8upus ). Show +<plaination C )( /he picture shown is considered to #e dia&nostic for what type of epider*olysis #ullosa 1. 7unctional epider*olysis #ullosa" Eon Ferlit; type $. 7unctional epider*olysis #ullosa" Ferlit; type %. 0owlin&"Meara for* of +pider*olysis Bullous si*ple< '. 0o*inant dystrophic epider*olysis #ullosa (. Hecessi3e dystrophic epider*olysis #ullosa ). Show +<plaination + )) Co**on cause=s> of dru&"induced pe*phi&us foliaceus: 1. Captopril $. Penicilla*ine %. Captopril and penicilla*ine '. Methotre<ate (. 0ilantin ). Show +<plaination C )A ,hich autoanti#odies would *ost likely #e found in an indi3idual with eye 6ndin&s without any cutaneous in3ol3e*ent. 1. Alpha ) #eta ' inte&rin $. Bullous pe*phi&oid anti&en 1 %. Bullous pe*phi&oid anti&en $ '. 0es*ocollin (. 8a*inin ( ). Show +<plaination A )C ,hich pair is incorrect. 1. 0es*o&lein 1 : 1)! k0a $. 0es*oplakin 1 : $(! k0a %. /ype ?11 colla&en : $D! k0a '. /ype O?11 colla&en: 1C! k0a (. +n3oplakin : 1D! k0a ). Show +<plaination + )D Patients with epider*olysis #ullosa acLuisita ha3e: 1. A defect in plectin $. A defect in colla&en O?11 %. Anti#odies to colla&en O?11 '. Anti#odies to colla&en ?11 (. A defect in la*inin ( ). Show +<plaination 0 A! A one"day old infant presents with papules and pustules on the face: trunk: and pro<i*al e<tre*ities. Pal*s and soles are spared. /he patient is otherwise doin& well. A #iopsy shows nu*erous eosinophils. /he dia&nosis is: 1. Acropustulosis of infancy $. Ferpes infection %. +rythe*a to<icu* neanotoru* '. Sca#ies (. /ransient neonatal pustular *elanosis ). Show +<plaination C A1 ,hich pair is incorrect. 1. ,rinkle"resistance : for*aldehyde $. Chewin& &u* : colophony %. Para#ens : artichokes '. 5lyceryl thio&lycolate : acid per*anent wa3e (. Per*ethrin : chrysanthe*u* ). Show +<plaination C A$ Pe*phi&us erythe*atosus: 1. 1s also called Fallopeau syndro*e $. May #e an a#orti3e for* of su#corneal pustulosis %. 1s often in a *alarse#orrheic distri#ution '. 0oes not ha3e linear 1&5 and C% at the #ase*ent *e*#rane ;one (. All of these answers are correct ). Show +<plaination C 1AA A% /he 3ector of fo&o sel3a&e* *ay #e: 1. /riato*a $. Si*uliu* %. Ci*e< '. 9rnithodorus (. Mus ). Show +<plaination B A' /he *ost co**on *ali&nancy associated with paraneoplastic pe*phi&us is: 1. Eon"Fod&kinGs ly*pho*a $. Chronic ly*phocytic leuke*ia %. Multiple *yelo*a '. Acute *yelocytic leuke*ia (. Fod&kinGs ly*pho*a ). Show +<plaination A A( /he defect in 4unctional epider*olysis #ullosa occurs at the: 1. 5ranular layer $. Spinous layer %. 8a*ina lucida '. 8a*ina densa (. Su#la*ina densa ). Show +<plaination C A) A $!"year"old fe*ale presents with an aller&ic contact der*atitis to a perfu*e containin& 8ily of the 3alley. ,hat is the causati3e aller&en. 1. Cinna*ic acid $. ?anillin %. Fydro<ycitronellal '. Atranorin (. +3ernic acid ). Show +<plaination C AA ,hich neoplas* is the *ost co**on cause of paraneoplastic pe*phi&us. 1. /hy*o*a $. C88 %. Castle*anGs disease '. Hetroperitoneal sarco*a (. Eon"Fod&kinGs ly*pho*a ). Show +<plaination + AC ,hich of the followin& aller&ens is a non" for*adelhyde releasin& preser3ati3e with a low sensiti;ation potential. 1. Methylisothia;olone $. Para#en %. Bronopol '. Quterniu*"1( (. 0M0M hydantoin ). Show +<plaination B AD Patients with this disorder *ay de3elop e<a&&erated reactions to insect #ites: 1. Chronic ly*phocytic leuke*ia $. Bullous pe*phi&oid %. 8upus erythe*atosus '. Atopic der*atitis (. 1ncontinentia pi&*enti ). Show +<plaination A C! ,hat is the anti&en associated with der*atitis herpetifor*is. 1. /issue trans&luta*inase $. +pile&rin %. 0es*ocollin '. 0es*o&lein % (. Periplakin ). Show +<plaination A C1 ,hat percenta&e of der*atitis herpetifor*is patients are asy*pto*atic #ut ha3e 6ndin&s consistent with celiac sprue on &astrointestinal #iopsy. 1. (N $. 1(N %. '!N '. A!N (. D(N ). Show +<plaination + C$ ,hich of the followin& is 8+AS/ 81K+8S to cross react with /o<icodendron plant der*atitis. 1. 7apanese lacLuer tree $. Cashew nut %. Man&o rind '. 5in&ko tree (. Kiwi ). Show +<plaination + C% /he di*ethyl&lyo<i*e test is used to detect the presence of: 1. 5old $. Sil3er %. Mercury '. Eickel (. 8ate< 0 1AC ). Show +<plaination C' ,hich of the followin& proteins is typically E9/ found to #e anti&enic in paraneoplastic pe*phi&us. 1. BPA&1 $. BPA&$ %. 0es*oplakin '. +n3oplakin (. Periplakin ). Show +<plaination B C( ,hich pair is incorrect. 1. 0er*atitis herpetifor*is : &ranular 1&A $. 8ichen planus : linear C% %. Bullous pe*phi&oid : linear C% '. Syste*ic lupus erythe*atosus : linear 1&5 (. 1&A pe*phi&us: intercellular 1&A ). Show +<plaination B C) Paraneoplastic pe*phi&us: 1. 1s characteri;ed #y a patho&no*onic $(! k0a anti&en $. 1s *ost often seen in association with lun& cancer %. 0oes not re*it e3en if the cancer is e<cised co*pletely '. May #e caused #y a #eni&n neoplas* (. All of these answers are correct ). Show +<plaination 0 CA Patients with late< aller&y are 8+AS/ 81K+8S to de3elop a reaction to: 1. Cashew $. A3ocado %. Chestnut '. Banana (. Kiwi ). Show +<plaination A CC A patient de3elops erythe*a and 3esiculation on his dorsal foot that spares the toewe#s and soles after #uyin& a new pair of shoes. ,hat is the *ost likely aller&en. 1. /hiura* *i< $. Car#a*ates %. 8ate< '. Mercapto#en;othia;ole (. 2or*aldehyd&e ). Show +<plaination 0 CD 1rritation of the hand produced #y capsaicin can #e relie3ed #y application of 1. ,ater $. Acetic acid (N %. Sodiu* chloride '. Alkali (. /alcu* powder ). Show +<plaination B D! ,hich of the followin& su#stances is known to cause a delayed positi3e patch test reaction. 1. 5old $. Eickel %. Bacitracin '. 2ra&rance *i< (. Hosin ). Show +<plaination A D1 ,hich of the followin& can #e responsi#le for contact der*atitis to K"S 7elly. 1. 8anolin alcohol $. Budesonide %. Alpha tocopherol '. Propylene &lycol (. /riclosan ). Show +<plaination 0 D$ /he anti&en in linear 1&A is: 1. BPA&1 $. Plectin %. DAk0a part of BPA&$ '. 8a*inin ( (. Alpha"): #eta inte&rin ). Show +<plaination C D% ,hich pair is incorrect. 1. +yedrops : thi*erosal $. +ar drops : neo*ycin %. /heophylline : ethylenedia*ine '. Anta#use : thiura* + 1AD (. Co#ala*in : #alsa* ). Show +<plaination D' /he pri*ary autoanti&en in pe*phi&oid &estationis is 1. 0es*oplakin $. BPA51 %. BPA5$ '. Plako&lo#in (. Anchorin& 6#rils ). Show +<plaination C D( ,hich of the followin& is known as Iprickly heatI. 1. Miliaria crystallina $. Miliaria ru#ra %. Miliaria profunda '. 5ro3erGs disease (. 2o<"2ordyce disease ). Show +<plaination B D) Patients with 0uhrin&Gs disease are *ost likely to ha3e: 1. Mutations in plectin $. Mutations in la*inin ( %. Mutations in trans&luta*inase 1 '. Anti#odies to trans&luta*inase % (. Anti#odies to BPA&$ ). Show +<plaination 0 DA 1n staphylococcal scalded skin syndro*e: the e<foliati3e to<in clea3es: 1. 0es*o&lein 1 $. 0es*o&lein % %. 0es*ocollin 1 '. 0es*ocollin % (. BpA&$ ). Show +<plaination A DC ,hich type of epider*olysis #ullosa has the &reatest risk of de3elopin& sLua*ous cell carcino*as. 1. ,e#er"Cockayne $. 0owlin&"Meara %. Ferlit; '. +B Si*ple< with *uscular dystrophy (. Fallopeau"Sie*ens type of recessi3e dystrophic +B ). Show +<plaination + DD 2ecal isocoproporphyrin is seen in: 1. Porphyria cutanea tarda $. Farderoporphyria %. ?arie&ate porphyria '. Acute inter*ittent porphyria (. Coproporphyria ). Show +<plaination A 1! ! 1n Brunstin&"Perry pe*phi&oid: the recrurrent crops of #listers are *ost likely to appear on: 1. 5enitals $. Pal*s and soles %. B*#ilicus '. Fead and neck (. Buttocks ). Show +<plaination 0 1! 1 Bullous pe*phi&oid anti&en 1 =BPA&1> is a *e*#er of this fa*ily: 1. Cadherin $. 1nte&rin %. 1nter*ediate 6la*ent '. Plakin (. Selectin ). Show +<plaination 0 1! $ Bullous lesions are seen in: 1. Con&enital syphilis $. Pri*ary syphilis %. Secondary syphilis '. /ertiary syphilis (. All of these answers are correct ). Show +<plaination A 1! % /ransient #ullous disorder of childhood has a defect in: 1. Alpha)"inte&rin $. Plectin %. BPA&$ '. +pili&rin (. Colla&en ?11 ). Show +<plaination + 1! /he *ost i*portant &roup of disease linked with 1. 8y*phoproliferati3e disorders A 1C! ' the patho&enesis of paraneoplastic pe*phi&us in adults is $. Colerectal adenocarcino*a %. Breast Cancer '. Sarco*as (. Melano*a ). Show +<plaination 1! ( Sou are consulted on this patient who has 4ust under&one an orthopedic spinal procedure. ,hat is true of this patient. 1. She should a3oid #ananas $. She is aller&ic to #etadine %. She is *ost likely ha3in& an aller&ic response to an anti#iotic '. She has *iliaria crystallina (. /his is not related to her procedure ). Show +<plaination A 1! ) ,hat is the en;y*e defect in 5untherGs disease. 1. Porpho#ilino&en dea*inase $. Broporphyrino&en synthetase 111 %. Broporphyrino&en decar#o<ylase '. 2errochetalase (. Protoporphyrino&en o<idase ). Show +<plaination B 1! A Cicatricial pe*phi&oid can #e induced #y: 1. A*ino&lycosides $. Ben;ene %. Clonidine '. ?anco*ycin (. Cipro-o<acin ). Show +<plaination C 1! C /he #est 6rst line treat*ent for der*atitis herpetifor*is is: 1. Methotre<ate $. 0apsone %. Colchicine '. Prednisone (. 1?15 ). Show +<plaination B 1! D I9rientalI ti&er #al* cross"reacts with: 1. Balsa* of Peru $. Slan&"Slan& oil %. Eeo*ycin '. 8anolin (. Hosin ). Show +<plaination A 11 ! ,hich steriod screenin& a&ent should #e used when an aller&ic contact der*aititis is suspected to hydrocortisone. 1. 5lutaral $. /i<ocortol"$1"pi3alate %. Budesonide '. Fydrocortisone"1A"#utyrate (. Ben;alkoniu* chloride ). Show +<plaination B 11 1 5allstones are associated with: 1. +rythropoietic protoporphyria $. Fepatoerythropoietic porphyria %. ?arie&ate porphyria '. Coproporphyria (. Farderoporphyria ). Show +<plaination A 11 $ Mutations in #eta"catenin are *ost co**only associated with: 1. Ea<os disease $. Bullous pe*phi&oid %. Pilo*atrico*as '. +ctoder*al dysplasia with skin fra&ility (. 9cular cicatricial pe*phi&oid ). Show +<plaination C 11 % ,hich of the followin& is the *ost co**on cause of contact der*atitis due to a for*aldehyde releasin& preser3ati3e. 1. Para#en *i< $. /hi*erosal %. Quaterniu* 1( '. 1*ida;olidinyl urea (. Paraphenylenedia*ine ). Show +<plaination C 11 ' Clea3a&e of this adhesion *olecule is seen in Staphylococcal Scalded Skin Syndro*e: 1. 0es*oplakin 1 $. 0es*oplakin $ %. 0es*o&lein 1 C 1C1 '. 0es*o&lein % (. 0es*oplakin 1 and des*oplakin $ ). Show +<plaination 11 ( /his is associated with deafness: 1. Claudin $. Cadherin %. Conne<in '. 0es*in (. 0es*oplakin ). Show +<plaination C 11 ) +ach of the followin& is true a#out anti"p$!! pe*phi&oid e<cept: 1. Hesponsi3e to dapsone $. Su#epider*al #ullae %. $!!"kd anti&en '. features of linear 1&A disease (. *ucosal in3ol3e*ent ). Show +<plaination + 11 A ,hich of the followin& che*icals is the *ost co**on cause of shoe contact der*atitis. 1. /hiura* *i< $. $"Mercapto#en;othia;ole %. Car#a *i< '. Mi<ed dialkyl thioureas (. 2or*aldehyde ). Show +<plaination B 11 C Papillary der*al deposits of 1&A and a papillary der*al in6ltrate of neutrophils is dia&nostic of: 1. SweetGs syndro*e $. 8eukocytoclastic 3asculitis %. 0er*atitis herpetifor*is '. 8inear 1&A der*atosis (. Bullous pe*phi&oid ). Show +<plaination C 11 D Anti#odies in so*e for*s of Ste3ens"7ohnson #ind to: 1. 0es*oplakin 1 $. 0es*o&lein 1 %. 0es*o&lein % '. Plako&lo#in (. 0es*oplakin 1 and des*o&lein % ). Show +<plaination A 1$ ! /his disease is inherited in an O"linked recessi3e *anner: 1. Chronic &ranulo*atous disorder $. 7o#Js syndro*e %. 0owlin&"Meara '. Mon&o*eryJs syndro*e (. /reacher"Collins ). Show +<plaination A 1$ 1 A (D"year old fe*ale has erythe*a and 3esiculation in an area of application of tincture of #en;oin. ,hich aller&en is she also *ost likely aller&ic to. 1. A**oniu* persulfate $. Black ru##er *i< %. Balsa* of Peru '. +u&enol (. +po<y resin ). Show +<plaination C 1$ $ ,hich of the associated anti&ens for this condition has #een associated with colon cancer. 1. Anti"epili&rin $. DA k0 linear 1&A der*atosis anti&en %. 0es*oplakin '. Bullous pe*phi&oid anti&en $ (. Beta"' inte&rin ). Show +<plaination A 1$ % ,hich of the followin& adhesion *olecules are i*portant for *aintainin& adhesion #etween keratinocytes. 1. Selectins $. 1nte&rins %. Cadherins '. 5lycans (. 1**uno&lo#ulin superfa*ily ). Show +<plaination C 1$ ' /h$"related cytokines include: 1. 181! $. 12E&a**a %. 12Ealpha '. 181! and 12E&a**a (. All of these answers are correct ). Show +<plaination A 1C$ 1$ ( Aller&ic contact der*atitis is characteri;ed histolo&ically #y: 1. Psoriasifor* der*atitis $. 8ichenoid in6ltrate %. Spon&iosis '. Parakeratosis (. 5ranulo*a ). Show +<plaination C 1$ ) 9cular cicatricial pe*phi&oid has anti#odies a&ainst: 1. 5a**a"catenin $. Peripherin %. Beta'"inte&rin '. Kalinin (. ?inculin ). Show +<plaination C 1$ A 1n penicilla*ine"induced pe*phi&us: the split is *ost often: 1. Su#corneal $. 1ntraspinous %. Supra#asal '. 1ntraepider*al and su#epider*al (. Su#epider*al ). Show +<plaination A 1$ C +pider*olysis #ullosa si*ple< with *uscular dystrophy is associated with a *utation in: 1. 8a*inin ( $. Plectin %. colla&en ?11 '. uncein (. alpha ) #eta inte&rin ). Show +<plaination B 1$ D ,hich of the followin& contains the sa*e aller&en as poison su*ac. 1. Man&o fruit $. Bra;ilian Pepper /ree %. Balsa* of Peru '. Ha&weed (. Artichoke ). Show +<plaination B 1% ! 0irect i**uno-uorescent studies in a patient with #ullous pe*phi&oid is *ost likely to show: 1. 8inear 1&A at the #ase*ent *e*#rane $. 8inear C% at the #ase*e*ent *e*#rane %. 5ranular 1&A in der*al papillae '. 1ntercellular 1&5' throu&hout the epider*is (. C% in the der*al papillae ). Show +<plaination B 1% 1 ,hat is the *ost co**on aller&en causin& aller&ic contact der*atitis. 1. Eickel $. Bacitracin %. 2or*aldehyde '. Quaterniu*"1( (. Eeo*ycin ). Show +<plaination A 1% $ A child de3elops an aller&ic reaction at the site of an in-uen;a 3accine. /o which of the followin& su#stances *ay she #e aller&ic to. 1. 8anolin $. /hi*erosol %. +thylenedia*ine dichloride '. /riclosan (. 5luteraldehyde ). Show +<plaination B 1% % /he a&ent *ost likely to cause pseudoporphyria is: 1. ESA10Gs $. Penicillin %. Beta"#lockers '. ?anco*ycin (. Ace"inhi#itors ). Show +<plaination A 1C%