| • | AKA Job syndrome the term “Job syndrome” taken from the biblical story of Job, who is said to have been "smitten with boils from head to toe" and is believed to have suffered from chronic furunculosis |
| • | pathogenesis – diminished neutrophil chemotaxis and impaired regulation of IgE function |
clinical:
| • | triad: atopic-like eczematous dermatitis, recurrent pyogenic infections, high level of serum IgE |
| • | recurrent pyogenic infections – skin and lungs |
| • | pustules and abscesses (30% cold) on scalp, neck, axillae, groin |
| • | face is consistently involved – coarse facies with broad nasal ridge (result of recurrent facial abscesses) |
| • | a defective erythema response may explain the “cold” abscesses |
| • | lungs – recurrent bronchitis, pneumonia, otitis media, sinusitis, pneumatoceles with bacterial/fungal superinfection |
| • | high level of serum IgE (>10,000 IU/ml) and eosinophilia |
| • | ddx: atopic dermatitis, Wiskott-Aldrich syndrome, DiGeorge syndrome |
summary:
Think of hyper-IgE syndrome or “Job syndrome” as a deep pyoderma, with recurrent abscesses and sinopulmonary infections. This is easy to remember because of the biblical story of Job, and it makes sense in that the pathogenesis is a primary defect in neutrophil chemotaxis (this also explains the “cold” abscesses). The coarse facies resulting recurrent facial abscesses.
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