Synonyms
Job's syndrome.
Definition
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.
Therapy
Pharmacological
Prophylactic therapy with a penicillinase-resistant penicillin or cephalosporin, and antimicrobial therapy as indicated during infections.
Prognosis
Prognosis is improved if patients are treated with chronic antibiotic therapy prior to the onset of severe lung infections.
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References
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 713–34
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© 2004 Springer-Verlag
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(2004). Hyper-IgE syndrome. In: Moreland, L.W. (eds) Rheumatology and Immunology Therapy. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-29662-X_1288
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DOI: https://doi.org/10.1007/3-540-29662-X_1288
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-20625-5
Online ISBN: 978-3-540-29662-1
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