Page 1 of 1: pediatrics - clinical problem

neuron03 · Credits: 3504 My Scrapbook

i must say at the outset that this is a real clinical case and not an objective type question.

a 14 yr old male came with complaint of dyspnea on exertion since last 1 yr which has increased during last 2-3 mo. o/e his wt is 53 kg (~ 50th centile for age), ht 130 cm (<5th centile), pulse 80/min, bp 110/ 84. he has seborrheic dermatitis on scalp, skin is dry coarse n seborrheic, constant sweating on forehead, congenital myopia of 2.5d which has incr to 4d during last 3 yrs, lungs and cvs is normal. cbc, bld sugar, electrolytes, and T4 TSH are normal.

any suggestions?

neuron03 · Credits: 3504 My Scrapbook

i m really surprised, that 25 ppl read this post but none offered a realistic n intelligent suggestion. the case proved to be one of type 1 histiocytosis x or langerhan's cell histiocytosis. i did an 2d echo which was normal and pulm fn testing suggested interstitial lung disease. cxr showed a ?small lytic lesion, which came out nicely on skull xr. i've sent the biopsy for electron microscopy.

parin · Credits: 14640 My Scrapbook My Reading List 1 Books

Absolutely neuron03!

Histiocotosis X includes- Eosinophilic granuloma, Hand-Schuller0Christioan disease & Letterer-Sewe disease, together being also known as Langerhans call histiocytosis..

It can present as..

1. commonest- skeletal lesions with calvaarial defect in skull, compression of spinal cord, lytic lesions in bones..

2. Seborrheic dermatitis of scalp, may involve palms, soles & back..

3. Chronically discharging infected ears..

4. pneumothorax & pulmonary infiltrates

5. hypothalamic dysfunction & pituitary involvement leading to DI & GR

6. Hepatosplenomegaly & lymphadenopathy

7. systemic manifestations in severe disease- fever, malaise, irritability, wt loss, failure to thrive, etc...

EM finding of Birbeck's granules being diagnostic of the disease..

jayanta · Credits: 36294 My Scrapbook My Reading List 45 Books

OOPS.

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