A rare primary immune deficiency syndrome of unknown etiology characterized by recurring staphylococcal infections of the skin, lungs and solid organs; and elevated serum levels of IgE and IgD. Patients exhibit marked blood and sputum eosinophilia. The memory immune response to recall antigens is diminished.
Prophylactic therapy with a penicillinase-resistant penicillin or cephalosporin, and antimicrobial therapy as indicated during infections.
Prognosis is improved if patients are treated with chronic antibiotic therapy prior to the onset of severe lung infections.
- Buckley RH (1998) Primary immunodeficiency diseases. In: Middleton E, Reed CE, Ellis EF, Adkinson NF, Yunginger JW, Busse WW (eds) Allergy: Principles and Practice. Mosby, Philadelphia, pp 713–34Google Scholar