Job syndrome vs Chronic granulomatous disease
How to tell Job syndrome and Chronic granulomatous disease apart when a board question makes them look alike.
Job is high IgE/eczema/cold abscesses/retained teeth; CGD is defective oxidative burst with catalase-positive infections.
Side by side
Job syndrome causes high IgE, eczema, retained primary teeth, coarse facies, cold staph abscesses, pneumatocele, and impaired Th17 signaling.
Chronic granulomatous disease causes recurrent catalase-positive infections, granulomas, and abnormal nitroblue tetrazolium or dihydrorhodamine testing from NADPH oxidase failure.
STAT3 signaling failure prevents normal Th17 differentiation, causing hyper-IgE syndrome with eczema, cold staphylococcal abscesses, recurrent pneumonias, retained primary teeth, and coarse facies.
NADPH oxidase defects, classically CYBB/gp91phox, impair the respiratory burst so catalase-positive organisms survive inside phagocytes.
Very high IgE, eosinophilia, eczema, cold abscesses, and STAT3 association support Job syndrome.
Abnormal dihydrorhodamine or nitroblue tetrazolium testing, granulomas, and infections with catalase-positive organisms support CGD.
Watch it explained
Test it nowThe repair move
Before naming the answer, force the stem through the separator: Job is high IgE/eczema/cold abscesses/retained teeth; CGD is defective oxidative burst with catalase-positive infections.
Try a free question
CoreA 10-year-old boy has recurrent 'cold' (non-erythematous, non-tender) Staphylococcus aureus skin abscesses, severe eczema, recurrent pneumonias with pneumatocele formation, retained primary teeth, coarse facial features (prominent forehead, broad nasal bridge), and recurrent fractures. Serum IgE is markedly elevated (12,000 IU/mL). Genetic testing reveals STAT3 mutation. Which condition is most likely?
Show answer and explanation
Answer: A. Job (hyper-IgE) syndrome. Job (hyper-IgE) syndrome is autosomal dominant STAT3 mutation impairing Th17 differentiation. Features: very high IgE, eczema, cold staphylococcal abscesses, recurrent pneumonias with pneumatoceles, retained primary teeth, coarse facies (FATED: Facies, Abscesses, retained Teeth, hyper-E, Dermatitis), bone fragility.
Why the other choices are tempting
- B. CGD has NADPH oxidase deficiency with catalase-positive infections, granulomas, abnormal DHR test, not the very high IgE with cold abscesses pattern.
- C. Wiskott-Aldrich has thrombocytopenia with small platelets, eczema, and immunodeficiency.
- D. Atopic dermatitis can have elevated IgE but lacks cold abscesses, retained teeth, and coarse facies.
- E. Cystic fibrosis has recurrent pulmonary infections (Pseudomonas, Staph) but lacks the very high IgE and cold abscess pattern.
Flashcards
Tap a card to flip it.
What clue supports Job syndrome?
Choose Job syndrome when the stem gives this pattern: Job syndrome causes high IgE, eczema, retained primary teeth, coarse facies, cold staph abscesses, pneumatocele, and impaired Th17 signaling.
What clue supports Chronic granulomatous disease?
Choose Chronic granulomatous disease when the stem gives this contrasting pattern: Chronic granulomatous disease causes recurrent catalase-positive infections, granulomas, and abnormal nitroblue tetrazolium or dihydrorhodamine testing from NADPH oxidase failure.
Quick answers
What is the fastest way to tell Job syndrome from Chronic granulomatous disease?
Job is high IgE/eczema/cold abscesses/retained teeth; CGD is defective oxidative burst with catalase-positive infections.
What is the key clue for Job syndrome?
Job syndrome causes high IgE, eczema, retained primary teeth, coarse facies, cold staph abscesses, pneumatocele, and impaired Th17 signaling.
What is the key clue for Chronic granulomatous disease?
Chronic granulomatous disease causes recurrent catalase-positive infections, granulomas, and abnormal nitroblue tetrazolium or dihydrorhodamine testing from NADPH oxidase failure.