2008

ORAL PATHOLOGY NOTES

Ankit Gupta
Home
1/1/2008
 HEMATOLOGIC DISORDER

LYMPHOID HYPERPLASIA

Germinal centers – collection of reactive lymphoblasts.

1. B- lymphocytes

Tangible bodies – phagocytized material in macrophages

HEMOPHILIA

Affected males 1 in 8000 to 10,000

Desmopressin – given for patient having factor 8 level more then 5% … it releases bound factor 8

Epsilon amino caproic acid… antifibrinolitic

V.W.F.

Most common inherited blood disorder

Affects 1 in 800 to 1000

SICKLE CELL ANEMIA

Alteration in: 1. Thymine replaced by adenine in DNA

2. Leads to replacement of glutamic acid by valine amino acid in peptide chain at 6th

Position IN BETA GLOBIN CHAIN

Causes ischemia – affects most …. Long bones

Lungs and abdomen

Susceptible to STREPTOCOCCUS PNEUMONIAE - D/T spleen destruction by repeated ischemia

HAIR ON END appearance… less prominent then THALASSEMIA...

HYDROXYUREA – converts adult Hb to fetal Hb… which prevents polymerization of HbS…

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THALASSEMIA

REDUCED SYNTHESIS OF GLOBIN MOLECULE…

Beta thalassemia major – Cooley’s anemia or Mediterranean fever… (HbA2)

Alpha thalassemia - HbH DISEASE – when 3 out of 4 genes are affected

2. HYDROPS FETALIS – when all 4 genes are affected… fatal within hours of birth

Chipmunk facies – d/t maxillary & mandibular enlargement

HAIR ON END APPEARANCE on skull radiograph

APLASTIC ANEMIA

Associated syndromes - Fanconi’s anemia and Dystrophic congenital

< 500 granulocytes per micro liter

<20,000 platelets per micro liter

<10,000 reticulocytes per micro liter

NEUTROPENIA

<1500 PER MICRO LITER

Benign ethnic neutropenia … < 1200 per micro liter, yet people are healthy

… Seen in Africans and Middle East

SEEN IN

GAUCHER’S SYNDROME

CARTILAGE HAIR SYNDROME

SCHWACHMAN DIAMOND SYNDROME

DYSKERATOSIS CONGENITA

Major problem from – Staph aureus and gram –ve organisms

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AGRANULOCYTOSIS

Seen in

KOSTMANN SYNDROME

Punched out ulcers … resemble ANUG

CYCLIC NEUTROPENIA

(Cyclic Hematopoesis)

Cycle of 21 days commonly occurs with LOWEST LEVELS persisting for 3 to 5 days

When neutrophils are at lowest levels MONCYTES AND EOSINOPHILS increase

ERYTHROMELALGIA

Seen in POLYCYTHEMIA VERA

Burning sensation of palm and feet with erythema and warmth

Occlusion of digital capillaries and necrosis is seen

LEUKEMIA

HTLV-1 – virus causing T- CELL LEUKEMI & NON HODGKIN

1. Can be transmitted from infected to uninfected

2. So T-CELL LEUKEMIA caused by HTLV-1 is INFECTIOUS

CHRONIC LYMPHOBLASTIC/LYMPHOCYTIC LEUKEMIA (CLL) – most common

- Affects adults

A.M.L. - AFFECTS BOTH ADULT AND CHILDREN

C.M.L. - AFFECTS MIDDLE AGE PEOPLE (3RD TO 4TH DECADE)

A.L.L. - AFFECTS CHILDREN

CHLOROMA – extramedullary collection of leukemic cells

- Green in color in fresh cut sections.

- Also called GRANULOCYTIC SARCOMA.

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LANGERHNS CELL HISTIOCYTOSIS (HISTIOCYTOSIS-X)

HAND SCHULLER CHRISTIAN DISEASE – bone lesion, exophthalmos and diabetes insipidus

LETTERER SIWE DISEASE – cutaneous, visceral and bone marrow involvement.

ON RDIOGRAPH – “SCOOPED OUT” appearance in mandible alveolar area…

Teeth appear “FLOATING IN AIR”

HISTOLOGY – Histiocytes are seen

HODGKINS LYMPHOMA

B – Lymphocyte origin (Reed Sternberg cells)

Most common site- CERVICAL & SUPRACLAVICULAR lymph nodes > axillary & mediastenal

MALIGNANT CELL IN

1. NODULAR LYMPHOCYTE PREDOMINANT – “POPCORN CELLS”

2. LYMPHOCYTE RICH – SHEETS OF LYMPHOCYTES & “REED STERNBERG CELLS”
3. NODULAR SCLEROSIS –“ LACUNAR CELLS” – R.S. CELLS IN CLEAR SPACE
4. LYMPHOCYTE DEPLETION – BIZARRE GIANT R.S. CELLS

STAGE 1 & 2 – TREATMENT only radiotherapy

STAGE 3 & 4 – Chemotherapy initially then if required Radiotherapy

DRUGS – MOPP (Mechlorethamine, oncovin, prednisone, procarbazine)

ABVD (Adreamycin, bleomycin, vinblastin, DTIC) – safer than MOPP

NON HODGKINS LYMPHOMA

B-CELL derived most common

T-CELL derived less common

HISTIOCYTE derived rare

REAL CLASSIFICATION SYSTEM (Revised European American Lymphoma)

For classifying the N.H. Lymphoma on histologic basis

Most common in BUCCAL VESTIBULE AND POSTERIOR PALATE

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MYCOSIS FUNGOIDES

(CUTANEOUS T-CELL LYMPHOMA)

T-CELL DERIVED (HELPER T-CELLS – CD4+VE)

3 STAGES

1. ECZEMATOUS:- resembles Psoriasis

Shows epidermotropism
2. PLAQUE STAGE:- atypical lymphocytes seen (SEZARY CELLS)
PAUTRIER MICROABSCESS – intraepithelial aggregate of sezary cells
Show cerebriform nucleus (infolding of nuclear membrane)
3. TUMOR STAGE:- visceral involvement may be seen

SEZARY SYNDROME- severe form of lymphoma with spleen liver involvement

And exfoliative erythroderma

BURKITT’S LYMPHOMA

B-cell derived

Endemic form (African form) – caused by EBV

Affects jaws most commonly

Maxilla more affected
Seen in children, more commonly below 3 yrs age

American form

Affects abdominal region more commonly

Seen in adults more commonly

“STARRY SKY APPEARANCE” - due to presence of macrophages amongst tumor cells.

ANGIOCENTRIC T-CELL LYMPHOMA

Affects midline structure of palate and nasal cavity

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MULTIPLE MYELOMA

PLASMA CELL DERIVED

Light chain defective production – Bence jones protein

Monoclonal gammopathy – serum & urine immunelectrophoresis yields m-protein

Punched out lesion on skull radiograph – lesion filled with abnormal plasma cell proliferation

Amyloid deposits in Tongue.

Kidney failure first presenting sign – d/t overload of light chain protein

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 BONE PATHOLOGY

OSTEOGENESIS IMPERFECTA

DEFECT IN COL 1A1 ON chromosome 17

& Col 1A2 on chromosome 7

Wormian bones on skull radiograph – small bones larger then 6 X 4 mm arranged in a mosaic pattern.

Hypoacusis, blue sclera…

Class –III malocclusion common d/t maxillary hypoplasia

TYPE 1 – most common and most mild

Most commonly associated with dentinogenesis imperfect - 1

TYPE 2 – MOST SEVERE in neonates

Most are still born

TYPE 3 – most severe beyond perinatal period

Few survive into adulthood

Most die in childhood

Type 4 – mild to moderate

Bone fragility decreases with age

Blue color of sclera disappear later in life

Woven bone never transforms to LAMELLAR BONE.

OSTEOPETROSIS

(ALBERS SCHONBERG DISEASE)

Defective osteoclasts

Endosteal calsificationn without resorption

Cortical bone thickens

Cancellous bone becomes sclerotic

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INFANTILE FORM – diagnosed at birth, severe disease

(Malignant osteopetrosis)

Autosomal recessive

ADULT OSTEOPETROSIS

Autosomal dominant

Benign osteopetrosis

Bone marrow involvement less severe compared to infantile form

Treatment-

Interferon gamma 1-b

Calcitriol
Limit calcium intake

CLEIDOCRANIAL DYSPLASIA

Defect in CBFA 1 gene… abnormal OSTEOBLASTIC function

Clavicle are malformed, usually the MEDIAL SIDE OF THE BONE IS UNDERDEVELOPED

Normal jaw relation

Numerous supernumerary teeth > 60

Teeth lack secondary cementum (cellular cementum)

WORMIN BONES

Over retention of deciduous teeth and delayed eruption of permanent

Less growth of maxilla leading to class III malocclusion later in life

FOCAL OSTEOPOROTIC MARROW DEFECT

MARROW EXPANSION

Jaw not expands

Women more affected

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IDIOPATHIC OSTEOSCLEROSIS

COMMONLY AFFECTS MANDIBULAR 1ST MOLAR

MNDIBLAR 2ND PREMOLAR AND 2ND MOLAR ARE LESS COMMONLY AFFECTED

DENSE LAMELLAR BONE WITH SCANT INFLAMMATORY CELLS

MASSIVE OSTEOLYSIS

(GORHAM – STOUT SYNDROME)

(PHANTOM BONE DISEASE)

(VANISHING BONE DISEASE)

MEDULLARY BONE RESORPTION

COLLAGEN SEEN IN LESION SITE AT LATER STAGES

PAGET’S DISEASE (OSTEITIS DEFORMANS)

PRAMYXOVIRUS detected in osteoclasts

Initial stage of OSTEOLYSIS

Later stage of BONE FORMATION (OSTEOBLASTIC)

BONE ENLARGES

HYPERCEMENTOSIS SEEN

“Cotton wool” appearance on x-ray in later osteoblastic stage

MONOSTOTIC OR POLYOSTOTIC

LEONTIASIS OSSEA – lion like face d/t mid face enlargement in MAXILLARY involvement

“Lincon’s beard” on BONE SCAN when MANDIBLE involved

HISTOLOGICALLY –“JIGSAW puzzle” or “mosaic” arrangement of bone

SERUM ALKALINE PHOSPHATSE level is HIGH

Serum CALCIUM & PHOSPHORUS are normal

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GIANT CELL GRANULOMA

Histologically similar to BROWN TUMOR OF hyperparathyroidism

Unifocal involvement

CHERUBISM (FAMILIAL FIBROUS DYSPLASIA)

Giant cell seen

Affects posterior mandible

Involvement of infraorbital plate and rim causes “eyes turned towards heaven” appearance

Self-limiting condition

SIMPLE BONE CYST

Affects commonly mandibular premolar and molar area…

Pseudo cyst

ANERYSMAL BONE CYST

“Blood soaked in sponge” appearance

Bone expansion unlike traumatic bone cyst

FIBROUS DYSPLASIA

GNAS 1 gene abnormality

Monostotic form and polyostotic form

Monostotic form commonly affects jaws

Maxilla more commonly affected (craniofacial fibrous dysplasia – as adjacent bones are also affected)

“Ground glass” appearance on x-ray

Bone expansion is slow

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POLYOSTOTITC FIBROUS DYSPLASIA

JAFFE-LICHTENSTIEN SYNDROME – FIBROUS DYSPLASIA + CAFÉ – AU – LAIT SPOTS

McCUNE-ALBRIGHT SYNDROME – ENDOCRINE DEFECT + JAFFE-LICHTENSTIEN SYNDROME

CAFÉ AU LAIT SPOTS – irregular margins

(Smooth margins in neurofibromatosis)

“Chinese letter pattern” of bone arrangement – HISTOLOGICALLY

GARDNER SYNDROME

Chromosome no 5 affected

BONE LESION INVOLVING angle of mandible

Skin lesion- epidermoid cysts

Intestinal polyps

Thyroid carcinoma – female at higher risk

Supernumerary teeth

OSTEOBLASTOMA

>2 cm in size

PAIN not relieved by aspirin

Radiolucency with flecks of radiopacity

OSTEOID OSTEOMA

<2cm in size

Tumor cells produce PROSTAGLANDINS - pain

SO pain is relieved by ASPIRIN (PROSTAGLANDIN INHIBITOR)

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CEMENTOBLASTOMA (TRUE CEMENTOMA)

Commonly affects mandibular 1st molar

Root outline not distinguishable on x-ray

Radiolucent line surrounding tumor

Giant cells seen

CHONDROMAS

COMPOSED OF MATURE HYALINE CARTILAGE

Condyle or anterior maxilla

Radiolucencies with central radiopcity

OLLIER DISEASE – Multiple widespread unilateral involvements

MAFFUCCCI SYNDROME – skeletal chondromatosis with soft tissue angiomas

SYNOVIAL CHONDROMATOSIS (CHONDROMETAPLASIA)

LOOSE BODIES – seen in joints

Affects joints

OSTEOSARCOMA

INTRAMADULLRY
JUXTACORTICAL (PERIPHERAL)

Affects mandibular posterior and horizontal ramus most commonly (on face)

Maxillary lesion in inferior portion

Symmetric widening of PERIODONTAL LIGAMANT SPACE around 1 or more teeth

“SUN BURST” Appearance on x-ray

“SPIKING” – Root resorption

PAIN NOT RELIEVED BY MEDICATION

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POSTIRRADIATION BONE SARCOMA

ABOVE 7000cGy (rad)

No risk below 1000cGy

CHONDROSARCOMA

MOST commonly involves HUMERUS, FEMUR, and ILEUM

“Sunburst” appearance may be seen

Jaw lesions are of grade 1 and 2

Grade 3 lesions are uncommon in jaw

EWING’S SARCOMA

MOST COMMON BONE MALIGNANCY IN “CHILDREN”

LONG BONES, PELVIS & RIBS – affected most frequently

PAIN

“ONION SKIN” periosteal appearance

Positive reaction to CD99

METASTATIC TUMOR OF JAW

Most common from BREAST >2/3RD

PROSTATE > ½
LUNG & KIDNEY = 1/3RD

COMMON TO MANDIBLE

Numb – chin syndrome – seen d/t involvement of I.A.N.

Most tumors cause radiolucency – “MOTH EATEN APPEARANCE”

BREAST & PROSTATE metastasis produce new bone “RADIOPAQUE”

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 NERVE DISEASE

TRIGEMINAL NEURALGIA

(TIC DOULOUREUX, FOTHERGILL’S DISEASE)

(TRIFACIAL NEURALGIA)

RIGHT side of face more (1.7 x) affected then LEFT

TRIGEMINAL NEURITIS

(TRIGEMINAL NEUROPATHY)

DIFFERS FROM TRIGEMINL NEURALGIA

LONGER duration of pain (hours – week)

Ache

HORTON’S SYNDROME (SPHENOPALATINE NEURALGIA)

(HISTAMINE HEADACHE, HISTAMINE CEPHALGIA)

(MIGRANOUS NEURALGIA, VIDIAN NERVE NEURALGIA)

(CLUSTER HEADACHE, SLUDER’S HEADACHE)

(LOWER HALF HEADACHE)

D/T vasodilatation of INTERNAL MAILLARY ARTERY

SEVERE PAIN, persistent

IN eye, maxilla, ear and mastoid,

Base of nose& beneath zygoma
“ALARM CLOCK” headache – d/t pain at same time each day in some patients

No trigger zones.

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TROTTER’S SYNDROME

Caused d/t NASOPHARYNGEAL TUMOR…

Pain similar to trigeminal neuralgia

d/t involvement of MANDIBULAR nerve at foramen ovale

tumor enters skull via foramen ovale
causes trismus by involvement of internal pteregoid(medial)

Manifested in LOWER JAW, TONGUE, SIDE OF HEAD

DEAFNESS MAY OCCUR.

PARATRIGEMINAL SYNDROME

(RAEDER’S SYNDROME)

Signs similar to HORNOR’S syndrome BUT NO ANHYDROSIS

Pain in region of TRIGEMINAL NERVE

BELL’S PALSY

More common in female

Involved in MELKERSON ROSENTHAL SYNDROME

GLOSSOPHARYNGEAL NEURALGIA

TRIGGER ZONES posterior oropharynx & tonsillar fossa.

Pain in ear, pharynx, oropharynx, posterior portion of tongue

JAW WINKING SYNDROME

(MARCUS GUNN PHENOMENON)

(PTERYGOID-LEVATOR SYNKINESIS)

Unilateral ptosis with elevation of ptotic eyelid on movement of mandible

LEFT UPPER EYELID MORE CMMONLY INVOLVED THEN RIGHT

MARIN AMAT SYNDROME – REVERSE MARCUS GUNN SYNDROME

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MULTIPLE SCLEROSIS

(DISSEMINATED SCLEROSIS)

Demyelinating disease - affecting white matter…

CHARCOATS TRIAD IS diagnostic

intention tremors
nystagmus
dysarthria (scanning speech)

MENIERE’S DISEASE

DEAFNESS – inner ear (conductive)

TINNITUS

VERTIGO

TEMPORAL ARTERITIS

(GIANT CELL ARTERITIS)

Granulomatous inflammation of arteries

CAUSALGI

SEVERE PAIN due to trauma or sectioning of PERIPHERAL SENSORY NERVE

HORNER’S SYNDROME

(SYMPATHETIC OPHTHALMOPLEGIA)

PTOSIS
ANHYDROSIS
VSODILATION
MIOSIS

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 DISEASE OF MUSCLES

DUCHENNE MUSCULAR DYSTROPHY

(SEVERE GENERALIZED MUSCULAR DYSTROPHY)

MALE AFFECTED

DYSTROPHIN DOES NOT FORM IN MUSCLE

USUALLY BEGINS BEFORE 6YRS OF AGE

DYSTROPHY OF LANDOUZY & DEJERINE

(FACIOSCAPULOHUMERAL DYSTROPHY)

Proximal myopathy

“Tapir-lips” – protruded lips due to muscle weakness, unable to smile or whistle

Eyes open while sleep

DYSTROPHIC MYOTONIA

DISTAL MYOPATHY

Autosomal dominant

“SWAN NECK”

Ptosis
Massater atrophy causing narrow lower face
Sternocledomastoid atrophy
All these give a appearance of “swan neck”

CONGENITAL MYOTONIA

THOMSEN’S DISEASE

“Herculean appearance” – muscle hypertrophy

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 SKIN DISEASE

ECTODERMAL DYSPLASIA

(CHRIST SIEMEN’S TOURAINE SYNDROME)

CHONDROECTODERMAL DYSPLASIA

(ELLIS-VAN CREVELD SYNDROME)

Nail, teeth and sweat glands involved as in ectodermal dysplasia

Chondrodysplasia

Polydactyly

Congenital heart disease

CANNON’S DISEASE

(WHITE SPONGE NEVUS)

(FAMILIAL WHITE FOLDED DYSPLASIA)

Autosomal dominant

FEATURES

Hyperperakeratosis
Acanthosis
Cytoplasmic clearing of spinous cells

WITKOP-VON SALLMANN SYNDROME

(HEREDITARY BENIGN INTRAEPITHELIAL DYSKERATOSIS)

SIMILAR TO WHITE SPONGE NEVUS

Ocular findings – plaque develops on bulbar congenctiva – may lead to blindness

Autosomal dominant

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DYSKERATOSIS CONGENITA

(COLE ENGMAN SYNDROME)

X-LINKED RECESSIVE

Nail changes

Thrombocytopenia – 1st hematological change

Aplastic anemia later in life

Leukoplakia – progresses rapidly then normal person

Hyperorthokeratosis

XERODERMA PIGMENTOSUM

AUTOSOMAL RECESSIVE

Not a pemalignant condition

But efficiency to repair UV damaged DNA is impaired

SCC & BCC develop in sun damaged skin

Malignant melanoma also is seen

INCONTINENTIA PIGMENTI

(BLOCH SULZBERGER SYNDROME)

X-LINKED DOMINANT

FEMALE MORE AFFECTED

INITIAL LESION – VESICULAR

OLIGODONTIA

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DARIER’S DISEASE

(KERATOSIS/DYSKERATOSIS FOLLICULARIS)

White surfaced papules – may give cobblestone appearance to mucosa

Palate most often affected

Intraepithelial acanthosis

“TEST TUBE” shaped rete ridges

Dyskeratosis cells

Corps ronds – round bodies

Grains – resemble cereal grains

PEUTS JEGHRS SYNDROME

INTESTINAL POLYPS – affect jejunum and ileum

Not premalignant

Blue brown macules on oral mucosa – mainly vermilion border

LKB 1 gene defect

HEREDITARY HEMORRHAGIC TELENGIECTSIA (HHT)

(OSLER-WEBER-RENDU SYNDROME)

Autosomal dominant

HHT 1

More severe
Pulmonary involvement
Early onset

HHT 2

Late onset
Mild
No pulmonary involvement

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Epistaxis is common

Common site – lips

EHLERS DANLOS SYNDROME

CLASSICAL TYPE – collagen -1 affected

Sometimes collagen – 5 is affected

VASCULAR TYPE – collagen – 3 is affected (ECHYMOTIC TYPE)

Blood vessel rupture with minor trauma

Decreased life expectancy

TYPE 7 – dental findings are hallmark

GORLIN SIGN – patient able to touch tip of nose with tongue

PAPYRACEOUS SCARRING – healing skin – resembles crumpled cigarette paper

TUBEROUS SCLEROSIS

(BUNREVILLE-PRINGLE SYNDROME)

(EPILOIA)

Tuberous growth – most common in NASOLABIAL FOLD area

“SHAGREEN PATCHES” – resemblance of skin to shark skin derived cloth – shagreen

Seen on trunk and waist

ASHLEAF SPOTS – seen best in UV light

COWDEN SYNDROME

(MULTIPLE HAMARTOMA SYNDROME)

TRICHILEMMOMAS – hair follicle hamartomas

high arched palate
periodontitis and dental caries
hemngioma, xenthoma and lipoma

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EPIDERMOLYSIS BULLOSA

SIMPLEX TYPE – mild

Defective keratin 5 & 14

No oral findings
Intraepithelial clefting on light microscopic view

JUNCTIONAL TYPE

MOST severe – death while delivery

Defective laminin-5 & collagen-17
Clefting in lamina Lucida of basement membrane

DYSTROPHIC TYPE

Defective collagen-7
Autosomal dominant and recessive
AUTOSOMAL DOMINANT- not life threatening
Autosomal recessive – life threatening
Oral findings are common
CLEFTING below LAMINA DENSA of basement membrane.

Mittin like deformity of extrimities- d/t repeated trauma and scarring

Microstomia

PEMPHIGUS VULGARIS

AUTOIMMUNE DISEASE

Autoantibodies directed against DESMOGLEIN -1 AND 3 of desmosomes in epithelium

Intraepithelial clefting occurs just above basal cell layer

Vesicles and bullae are initial lesions

+ve NIKOLSKY SIGN

TZANK cells are diagnostic – rounded acantholysed spinous cells

Ocular involvement without scarring

Immunofluorescence within epithelium (intraepithelial)

Often associated with other autoimmune diseases – particularly MYASTHENIA GRAVIS

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PEMPHIGUS VEGETANS

Neumann type and Hallopeau type

Neumann type more common

Initial lesion is pustule and not bullae

PARANEOPLASTIC PEMPHIGUS

SIMILAR TO PEMPHIGUS

DIFFERENCES ARE

Ocular involvement results in SCARRING

Intraepithelial and subepithelial clefting may be observed
Antibodies against DESMOPLAKIN-1 & 2

CICATRICIAL PEMPHIGOID

(BENIGN MUCOS MEMBRANE PEMPHIGOID)

Any oral mucosa may be involved

Subepithelial clefting
Antibody against basement membrane component
Antiepilegri antibody – most serious cases
“IgG autoantibodies found on the ROOF OF BULLA”
OCULR INVOLVEMENT LEADS TO CICATRIX FORMATION
DIRECT IMMUNOFLOUROSENCE – linear deposition of antibodies

EPIDERMOLYSIS BULLOSA AQUISITA

Similar to cicatricial pemphigoid

Linear deposition of antibodies on direct immunofluorescence
Autoantibody IgG found on the FLOOR OF THE BULLA

BULLOUS PEMPHIGOID

SUBEPITHELIAL CLEFTING

Antibodies against – bullous pemphigod antigen (BP 180 & 230)

BP180 present in upper layer of LAMINA LUCIDA

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ERYTHEMA MULTIFORME

Autoimmune disease

“Bulls eye” lesion with central region of necrosis is seen on skin

Oral lesion – vesiculo-bullous

More severe forms are

Erythema multiform major (STEVEN JHONSON SYNDROME)

TOXIC EPIDERML NECROLYSIS

BOTH THESE SEVERE FORMS ARE INITIATED BY DRUGS

REITER’S SYNDROME

Non gonococcal Urethritis

Conjunctivitis
Arthritis

Male > female

Urethritis appears first

Arthritis appears last

“BALANITIS CIRCINATA” – lesion noted on glans penis

Well circumscribed erythematous erosion with scalloped whitish linear boundary

HISTOPATHOLOGICALLY SIMILAR TO “PSORIASIS”

LICHEN PLANUS

“SAW TOOTH” rete ridges

Band like lymphocyte infiltrate immediately subjacent to epithelium (T-lymphocyte predominantly)

“HYDROPIC DEGENERATION” of basal cell layer of epithelium

CIVATTE BODIES (CYTOID, HYALINE, COLLOID)

DEGENERATING KERATINOCYTES
FOUND AT JUNCTION OF EPITHELIUM AND CONNECTIVE TISSUE

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GRAFT VERSUS HOST DISESE (GVHD)

Grafted cells recognize the foreign environment and attack the host system

PSORIASIS

ACTIVATED T-CELLS CAUSE THE PROBLEM

ORAL lesions are rare

Erythema migrans (geographic tongue) has similar lesion

Erythematous plaque with silver scales

“AUSPITZ SIGN” on removal of these plaques “pin-point” bleeding spots are seen

“MUNROE’S ABSCESS” visible histologically

UV light worsens the lesion

LUPUS ERYTHEMATOSUS

SLE – butterfly rash seen on malar area and nose

DLE – part of chronic cutaneous LE

Hyperkeratosis with keratin plugged in opening of HAIR FOLLICLE (FOLLICULAR PLUGGING)

LIBMAN-SACKS ENDOCRDITIS – warty vegetations affecting heart valves in SLE

LUPUS CHELITIS – involvement of lips in SLE

DEGENERTION OF BASAL CELL LAYER

PAS-positive material deposited in basement membrane zone

Subepithelial edema and lymphocyte infiltration

POSITIVE LUPUS BAND TEST – Deposit of IgG or IgM at basement membrane in shaggy granular form

ANAs – anti nuclear antibodies are found

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SYSTEMIC SCLEROSIS (HIDE-BOUND DISEASE)

Deposition of dense collagen in all the organs and tissues of body

Acro-osteolysis – resorption of terminal phalanges

“Mouse facies” – due to loss of alae of nose (pinched appearance of nose)

RAUNAUDES PHENOMENON – often the first presenting sign

Diffuse widening of periodontal ligament space involving all teeth

Tooth resorption is seen

LOCALIZED SCLERODERMA

Less severe then systemic form

Coup de saber (strike of sword) – affects only localized region of skin (scarring)

ANTICENTROMERE ANTIBODIES or ANTI SCL70 (TOPOISOMERASE 1) is diagnostic

CREST SYNDROME

(Acrosclerosis)

Calcinosis cutis – subcutaneous nodules, mobile 0.5 to 2.0 cm in size

Raunaudes phenomenon
Esophageal dysfunction
Sclerodactyly – clawed fingers
Telengeactesia – similar to HHT

ACANTHOSIS NIGRICANS

Increased melanin deposit – specially in FLEXURAL AREAS

MALIGNANT FORM – associated with ADENOCARCINOMA of intestine

Benign form usually is not associated with any tumor

Lesion itself is not malignant

Associated with CROUZON SYNDROME:-

Fontanelle remain open

Cloverleaf skull

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OROFACIAL CLEFTS
CL+CP = 45% females <males (1:2)

CP = 30% male < female

CL = 25% female < male (1:1.5) unilateral CL more common on LEFT side

Cleft lip
Due to non fusion of median nasal process and maxillary process

Lateral facial cleft

D/T non fusion of maxillary ad mandibular processes

Extends from commisure to external ear

May be associated with mandibulofacial dysostosis

Oblique facial cleft

Extends from upper lip to eye

Median cleft of upper lip

D/T non fusion of median nasal processes

Associated with – ellis van crevald syndrome

Orfacial digital syndrome

COMMISURAL LIP PITS

More common in males

Autosoml dominant

1-4mm tract

Sometimes secrete saliva from minor salivary glands

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PARAMEDIAN LIP PITS (CONGENITALFISTULAS)
On lower lip

Blind tracts up-to 1.5cm depth

Associated with syndromes – VAN DER WOUDE

POPLETIAL PTEREGIUM

DOUBLE LIP
Occurs due to persistent cleft between pars villosa and pars glabarosa

Upper lip more often involved

Significant when lips are tensed as in smiling

FORDYCE GRANULES
More common in adults then children

Sebaceous cells are polygonal with centrally placed nucleus and foamy cytoplasm

MACROGLOSSIA
Diffuse generalized smooth – beckwith-weidman syndrome & hypothyroidism

Pebbly tongue – lymphangioma

Fissured, papillary surface – down syndrome

Nodular – amyloidosis, MEN-2B, neurofibromatosis

LINGUAL THYROID
Normally thyroid descends to its pos by 7th week intrauterine

More common n females

Diagnosis – thyroid scan using iodine isotope or technetium 99m

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FISSURED TONGUE
Micro abscess in upper epithelial layer

Hyperplasia of rete ridges

Loss of keratin hair on filliform papillae

Often associated with geographic tongue

Component of MELKERSON ROSENTHLL SYNDROME

STAFNE DEFECT
Defect seen below mandibular canal

PALATINE CYST OF NEWBORN

Also called Epstein pearl or Bhon’s nodule

Fissural cysts developing along the mid palatal raphae

1-3mm in size

May also arise from palatal minor salivary gland epithelium

NASOLABIAL CYST (NASOLACRIMAL/KALSTEADT)

Found on line of fusion of median and lateral nasal process with maxillary process

From remnants of nasolacrimal duct cells

No radiographic changes

Female : male = 3 : 1

Goblet cells and cilia are seen

GLOBULOMAXILLARY CYST
Seen between lateral incisor and cuspid in maxilla

Inverted pear shaped radiolucency

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Often monolocular

NASOPALATINE DUCT CYST

In midline near nasopalatine canal

Most common intraoral developmental cyst

Heart shaped radiolucency

Intermittent pain and drainage

EPIDERMOID CYST
Also called infundibular cyst

Arise from follicular infundibulum

Young adults – face more affected

Older adults – back more affected

Associated with gardner syndrome

GIANT CELLS may be seen in the wall of cyst

DERMOID CYST
Epithelial lined cyst

Teratoma

Most common in mid-line of floor of mouth

Painless, doughy or rubbery mass

Skin appendages like hair follicle, sebaceous glands may be seen

BRANCHIAL CLEFT CYST (CERVICAL LYMPHOEPITHELIAL CYST)

Most common on left side of neck

Occurs on upper part of neck along the anterior border of sternocledo-mastoid

Painless, fluctuant & 1-10 cm in diameter

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PROGRESSIVE HEMIFACIAL ATROPHY
(PARRY ROMBERG SYNDROME)

Affects females more then males

Coup de saber lesion between normal and abnormal regions

Facial palsy, trigeminal palsy and contra-lateral jacksonian epilepsy may be seen

Nose and chin deviate to the affected side

CROUZON SYNDROME (CRANIOFACIAL DYSOSTOSIS)

Defect in FGFR2 gene

Premature closure of cranial sutures resulting in

Brachycephaly (short head)

Scaphocephaly (boat shaped)

Trigonocephaly (triangular)

Severely affected patients – clover-leaf skull (KLEEBLATT SCHADEL deformity)

Radiograph – digital marking ‘beaten-metal’ pattern

Underdeveloped maxilla

APERT SYDROME (ACROCEPHALOSYNDACTYLY)

Defect in FGFR2 gene

Autosomal dominant

Premature closure of sutures

Acrobrachycephaly – tower like skull

Clover leaf skull may be seen

Ocular proptosis – due to small orbits, as in crouzon syndrome

Syndactyly – 2nd 3rd and 4th digits of hand and feet are fused

Synonychia

Hypoplastic maxilla

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MANDIBULOFACIAL DYSSTOSIS (TREACHER COLLINS SYNDROME;
FRANCESCHETTI-ZWAHLEN-KLEIN SYNDROME)
Defect in structure derived from 1st and 2nd arches

Hypoplastic zygoma – narrow face with depressed cheeks

Coloboma – notch occurs at the outer portion of lower eyelid

Eyelashes are often absent medial to coloboma

Coronid process and condyle are hypoplastic

Underdeveloped mandible

Sideburns – extend in a ‘tongue’ like projection

Parotid glands – hypoplastic or totally absent

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