The Hyper-IgE Syndrome

Author: Rebecca H Buckley

Source: Clinical Reviews in Allergy and Immunology, Volume 20, Number 1, February 2001 , pp. 139-154(16)

Publisher: Humana Press

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Abstract:

The hyper-IgE syndrome is a primary immunodeficiency disorder first described in 1972 (1) characterized by recurrent staphylococcal abscesses and markedly elevated serum IgE concentrations. Those affected have lifelong histories of severe recurrent staphylococcal abscesses involving the skin, lungs, joints, and other sites. In addition, there is a unique tendency of these patients to form persistent pneumatoceles following staphylococcal pneumonias. Although there usually is a history of a pruritic dermatitis, it is not typical atopic dermatitis, and respiratory allergic symptoms are usually absent.

Keywords: Hyper-IgE Syndrome, Chronic Granulomatous Disease

Document Type: Miscellaneous

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