Try it
Dermatology · Infections: hyper-IgE syndrome
Sample itemA child has recurrent cold staphylococcal skin abscesses, eczema, pneumatoceles, retained primary teeth and very high serum IgE. What is the likely diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Hyper-IgE (Job) syndrome
Hyper-IgE syndrome, usually due to STAT3 mutation, presents with eczema, cold abscesses, pneumonias with pneumatoceles, retained teeth and fractures. Serum IgE is markedly raised. Chronic granulomatous disease gives catalase-positive infections with abnormal nitroblue tetrazolium reduction.
Option by option
A. Atopic dermatitis alone
Atopic dermatitis alone does not explain recurrent deep abscesses and pneumatoceles.
B. Hyper-IgE (Job) syndrome (correct)
Correct. Cold abscesses, eczema, pneumatoceles and raised IgE indicate Job syndrome.
C. Wiskott-Aldrich syndrome
Wiskott-Aldrich has thrombocytopenia with small platelets.
D. Chronic granulomatous disease
CGD shows infections with catalase-positive organisms and granulomas, without retained teeth.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
More Dermatology questions
- Blistering disordersA patient has flaccid blisters that rupture easily and a positive Nikolsky sign. Which c…
- LeprosyIn which type of leprosy is the lepromin test strongly positive?
- Pigmentary disordersA man has well-demarcated chalk-white macules on his hands and around his mouth. A skin…
- Drug reactionsWhich of the following drugs is a well-recognised trigger of Stevens–Johnson syndrome an…