Case Report

Details EM, 2.2 year olds boy, history was given by mother. Presenting Complaint

Presented to A&E on Saturday ,9th May 2009 for 3 days history of rash all over his body History of Presenting Complaint

EM had infection on his right ear, brought to the GP by his mother on 29 th April 09 and prescribed with Distaclor for 5 days. On Wednesday (6th May 09)- small itchy rashes started to flare up on his right forearm Right elbow became swollen. He had no fever. On Thursday morning, his mother brought EM to GP, ?Chicken Pox Was referred to Temple Street Hospital (but no bed). Brought in to NCH A&E, FBC and Urine checked, cleared and sent home. By Friday night, rashes have spread all over body-arms, legs, neck, torso, buttock, groin. It was very itchy. He became irritable and has difficulty to sleep that night .He refused taking solids food since but was able to take fluid. His joints swelling become worse, and by that night, elbows, knees and ankles have been affected. They were red and hot to touch. EM cried on standing. There were no swellings on mouth or orbital area. EM body temperature was up and down at that time thus making his parents anxious. On Saturday, he was brought back to the A&E, and admitted to NCH on the same day. Of note- he had been prescribed with Distaclor approximately 6 times before due to recurrent tonsillitis and otitis media. Birth History

Born in Australia Full term, 40 weeks, Assisted Vaginal Delivery (ventouse) No complication during pregnancy- no Gestational Diabetes Mellitus or Hypertension. Birth weight-8 lb 6 oz (3.7 kg) Due to Ventouse usage , EM had chignon on his scalp- put into special care unit that night. Mum went home with EM on the next day.

Nutritional/feeding History

Bottle fed since birth (Aptamil) Started solid (puree foods) at 3 months At 12 month age- he started to drink cow milk on cup He has no food allergy nor on any special diet- he eats everything Development History

Gross Motor Sit? (mum cannot remember).Crawls at 6 months, walks on his own at around 10 months Vision and fine motor Can reach and hold toys properly at 7 months Hearing, speech and language -say dada and mama at 8 months. Now, knows quite a few words eg. Ball, touch, and no. Cannot make proper sentences yet. A little difficulty with pronunciation of words, but at the level that can still be understood (parents not really concern) Social, emotional and behavioural -Now able to feed himself, quite a shy boy but still plays with other kids.

Growth History

Parents has no concern at all regarding growth Growing out of clothes and shoes- every 6 weeks always need new shoes or clothes.

Past Medical and Surgical History

At 12 month age, EM had tonsillitis and Otitis Media the month after. Since then, he had either/both infections in a month. Sleep apnoea due to tonsillitis and adenoiditis. Sleep study conducted in NCH (11th February 09) Scheduled for Tonsillectomy and Adenoidectomy (25th June 09) Due to recurrent OM- scheduled? for grommet insertion on R ear (date not set yet).

Medications Actifed 2.5 mL,tds. Voltarol (diclofenac) suppositories Codeine Syrup Calamine Cream (antihistamine) NKDA until now. ?Cephalosporin Vaccination

Up to date Birth -Hepatitis B 2 months -Hepatitis B, Diphtheria tetanus and acellular pertussis, Hib, inactivated poliomyelitis, Pneumococcal conjugate, Rotavirus 4,6months - 5 in 1( dip, pertussis, tetanus,ipv),Men C. 12-15 months- MMR, Hib ?BCG. Born in Australia, went back to Ireland at 4 month age.

Family History

Parents have no consanguinity. Only child in the family Both maternal and paternal grandmother- allergy to penicilin. History of arthritis on both sides of families. No history of eczema/ asthma in the family. No family history of hearing problem.

Social History

EM lives in Bray with parents. Parents not married. Dad is currently not working. Mum works as part time credit controller. Taken care by dad when mum works. Dad- smoke (outside the house) No pets. Active child.

Systemic Review Bowel-no bowel work from Thursday to Sunday nothing in between. Bladder- water work was fine. Feeding- food refused since Friday. Appetite improved after admission on Saturday. Behaviour- irritable, cried on walking until admission. Skin rashes all over body ( refer skin examination for details) Examination

General inspection

Colour-Pink in the room air Hydration- No dry skin.Well hydrated and well nourished. Alertness and spontaneous movement- playing and able to walk on his own. Dysmorphic features- absent External support- Canulla on right hand. Distress- no obvious distress. Vital Signs T= 36. 7C BP = 119/53 Pulse=136 beats/min (normal volume and character) Respiratoty Rate=24/min O Saturation=97% Hand, Face, Neck There is no clubbing, no anemia, no peripheral cyanosis. No conjunctival pallor. No lymphadenopathy found in my examination. * Clinical notes-small lymph nodes palpable on right axilla. Skin Examination Confluent erythematous maculopapular rash and wheals on the trunk area (front and back), cheek, neck, arms and legs. Genital area not checked. No angioedema present. Scratching marks seen all over body especially on cheek and arms. On 2nd visit, Wednesday (13th May 09), the area of erythematous maculopapular rash is much reduced, especially on the torso . Rash still present on neck, arms and legs. On the right cheek, there were obvious scratching marks . Musculoskeletal Examination Look Swollen ankles and elbows. Feel- ankles and elbows tender to touch, feel hot. Movement and function- All active movement present (EM able to run, walk and play with toys) Ears and Throat Tried twice (1st and 2nd visit)-Not consented to do this examination. *Clinical notes Tonsil enlarged,dull right tympanic membrane. *From mum- tonsil red and large all the time. Developmental Examination

Done during 2nd visit (Wednesday) in the play room Gross Motor Walks, runs and jumps. Vision and fine motor Able to scribble and build toys model Hearing, speech and language Follows instruction given by mum or myself- eg give me the ball Said a few words (easy to understand) and short sentences (only mum understand) Social, emotional and behavioural Good social interaction. Play with me and other kids in the play room. Growth Examination Current Weight 16.7kg (above 97th centile) Current Height- 94 cm (97th centile) Head Circumference not taken. Respiratory system Sign of respiratory distress-absent. Equal bilateral air entry, vesicular breathing, no added sounds. Cardiovascular system HS1 & 2 present. No murmur or any added sounds. Abdomen Not distended, no scar, or obvious movement/peristalsis. Abdomen was soft,non tender, no organomegaly Liver edge palpable ,was soft ,fairly sharp and approx. 1 cm from costal margin. Kidney and spleen non palpable. Bowel sound present and there is no renal bruits.

Summary

This is EM, 2.2 year old boy who presented to NCH A&E on Saturday, 9th May 2009 with 3 days history of rash all over his body that spread to the arms, legs,neck, torso, buttock, groin and was very Itchy. He also had swollen joints, became irritable, cried on walking, unable to sleep at night, refusing solids food and taking only liquid. His temperature was ups and down. On examination, there is confluent erythematous maculopapular rash and wheals on the trunk area (front and back), cheek, neck, arms, legs with notable scratch marks. Ankles and elbows were swollen and hot to touch Since admission, EM conditioned improved greatly and was waiting to be discharged from hospital.

Differential Diagnosis Urticaria rash due to cephalosporin allergy. Urticarial vasculitis Contact dermatitis Henoch-Schnlein Purpura. Juvenile idiopathic arthritis Septic Arthritis Investigation FBC lymphocytes 7.19x 10/L (), Hb 11.3 MSU-normal Throat swab for Culture and Sensitivity. Blood allergy test Management Stop the intake of any drug that may cause the rash episode. Antihistamine for relieve from itchiness. Adequate analgesics for pain on the joints. Referral to dermatologist for further evaluation and management. Outcome EM was seen by dermatology team on Tuesday. Diagnosis was not confirmed even on my 2nd visit on Wednesday, 13th May 2009. Working Diagnosis urticaria rash due to cephalosporin allergy. ?Urticarial vasculitis. Waiting for Allergy blood test to come back Skin biopsy? Will only be done if rash is not settling down.

Discharge Plan
1. Avoid triggers (if its already identified)- in this case avoids cephalosporin and penicillin

(Usually, there is cross reaction with penicillin in those allergic to cephalosporin) 2. Parents should learn resuscitation technique. 3. Review child in OPD in 6 weeks time. * Epipen(adrenaline) should be ready anytime,if anaphylaxis shock is one of the presenting symptoms (but not in this case)

URTICARIA

Definition
A vascular reaction of the skin characterized by erythema and wheal formation due to localized increase of vascular permeability. It usually appears to be raised, well circumscribed areas of erythema and oedema of varying

sizes involving the dermis and epidermis and are very pruritic. Urticaria also known as hives.

Incidence Lifetime incidence is higher than 20%. Pathophysiology Urticaria results from the release of histamine, bradykinin, leukotriene C4, prostaglandin D2, and other vasoactive substances from mast cells and basophils in the dermis. Histamine released into the dermis result the intense pruritus of urticaria.In fact, histamine effects plays pivitol role in clinical and histologic findings of urticaria. Histamine is the ligand for 2 membrane-bound receptors, the H1 and H2 receptors that are present on many cell types. The activation of the H1 histamine receptors on endothelial and smooth muscle cells leads to increased capillary permeability whilst H2 histamine receptors activation leads to arteriolar and venule vasodilation. Classification of Urticaria There are 3 mains types ie: Spontaneous Urticaria (80%), Physical Urticaria (about 10%) and special type of urticaria (less than 10%). Urticaria Group Spontaneous Causes Acute
Infections - GI infections, genitourinary infections, respiratory infections, fungal infections , HIV, parasitic infections Foods (particularly shellfish, fish, eggs, cheese, chocolate, nuts, berries, tomatoes) Environmental factors (eg, pollens, chemicals, plants, danders, dust, mold) Drugs (beta lactamase antibiotics) Emotional stress Pregnancy (ie, pruritic urticarial papules and plaques of pregnancy [PUPPP]) Idiopathic in many cases

Subtypes of urticaria Acute Chronic

Definition Spontaneous wheals less than 6 weeks Spontaneous wheals more than 6 weeks

Chronic
All causes of acute urticaria Autoimmune disorders (SLE, rheumatoid arthritis, polymyositis, thyroid autoimmunity, and other connective tissue diseases) Chronic medical illness, such as hyperthyroidism, amyloidosis, polycythemia vera, malignant neoplasms, and lymphoma Familial cold auto inflammatory syndrome Idiopathic in 80%-90

Diagnosis Acute
1. Careful history to identify potential triggers (ask for atopic disease, known allergies, drug

intake, signs of infection. 2. Physical examination (BP,pulse,lung auscultation) 3. If no causes identified from history, no further investigation needed due to self limiting nature with this type of urticaria. Chronic
Infection -, culture and sensitivity, serology, etc. Autoreactivity aotulogous serum skin test, cellular activation test (eg. Serum induced histamine release, thyroid auto abs, antinuclear antibodies Non allegic Hypersensitivity- consider triggering by aspirin/Nsaids, in rare casesdo allergen low diet test.

Through History, skin test, FCC, U&E, and general parameters for inflammation

Managemant
In patient care Stops using substances that give rise to the urticaria Symptomatic treatment for itchiness-antihistamine Steroids (glucocorticoids ) to stabalise mass cell membranes and inhibit further histamine release In progressive cases, ie: anaphylactic shock, adrenaline must be administered Referral to dermatologist Acute Chronic

Initially, review in outpatients clinics. Patients and family education-avoids triggers Epipen (adrenaline), if anaphylaxis shock presented with urticaria

Goal is to maximise quality of life Patients and family education-avoids triggers Epipen (adrenaline), if anaphylaxis shock presented with urticaria Long term histamine for symptomatic urticaria Oral steroids - only after maximal dose of antihistamine Immunosuppressive agents eg cyclosporine in autoimmune urticaria Frequent review in outpatients clinics.