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Hyperimmunoglobulin‐E syndrome with recurrent infection: A review of current opinion and treatment
Author(s) -
ErlewynLajeunesse Michel D. S.
Publication year - 2000
Publication title -
pediatric allergy and immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.269
H-Index - 89
eISSN - 1399-3038
pISSN - 0905-6157
DOI - 10.1034/j.1399-3038.2000.00091.x
Subject(s) - medicine , etiology , immunoglobulin e , primary immunodeficiency , atopy , immunology , atopic dermatitis , immunodeficiency , dermatology , respiratory tract infections , pediatrics , immunopathology , antibody , respiratory system , immune system
Hyperimmunoglobulin E (hyper‐IgE) syndrome with recurrent infection is a rare idiopathic primary immunodeficiency. It consists of a severe dermatitis with recurrent abscess formation, respiratory tract infections and very high titres of serum immunoglobulin E (IgE). Hyper‐IgE syndrome is also associated with skeletal abnormalities. Variability of presentation makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiencies. The aim of this article is to review the literature in order to consider the clinical findings, etiology and treatment of this syndrome.