Friday, July 17, 2009

Question 9

6 year old Caucasian boy was brought to the ER with history of fever since 3 days, there is a rash on scalp since the advent of fever. The mother also complaints that he drinks a lot of water, and passes urine often, sometimes even 20 times a day. The mother is anxious and seems to be concerned about her son's bulging eyes. On examination, the boy looks lethargic, vitals are normal. Exophthalmus is present and Temperature is 99.6 F. X ray of the skull suggests cyctic lesions of the scalp. Urinary Na is low and serum Na is 154. On examination of ears, the physician finds a similar rash in the external canal. What is the most likely diagnosis in this case?

A) Benign Histiocytosis
B) Letterer Siwe disease
C) Hand-Schuller Chrsitian disease
D) Craniopharyngeoma
E) CNS lymphoma
F) Kawasaki disease
G) Meningitis
F) Acute lymphocytic leukemia

For correct answer click comments

1 comments:

Usmle Challenger July 17, 2009 at 3:55 PM  

Answer: C) Hand-Schuller Christian Disease.

Points to note:
Histiocytosis (langerhans) are CD1 Positive and on EM appears as tennis racket (birbeck granules)

Malignant histiocytosis:
1) Letterer Siwe Disease
Children 0 - 2 years
Rapidly fatal
Multiorgan involvement
Rash + Cystic lesions
2) Hand Schuller Christian Disease
Children
Fever + Rash + Classical Triad (expohthalmus+
Diabetes Insipidus + Cystic skull lesions)
Benign Histiocytosis: Eosinophilic granuloma presents with pathological fractures in adoloscents.

About This Blog

  © Blogger templates The Professional Template by Ourblogtemplates.com 2008

Back to TOP