Hyper-IgE syndrome

Reference work entry
DOI: https://doi.org/10.1007/3-540-29662-X_1288

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.

References

  1. 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

Copyright information

© Springer-Verlag 2004