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Hyper IgE Syndrome in Japan: A Summary of the Clinical and Immunological Features of 40 Patients
Author(s) -
Yanagi Tadamichi,
Tsuji Yoshiro
Publication year - 1985
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1985.tb00680.x
Subject(s) - medicine , immunoglobulin e , staphylococcus aureus , immunology , chemotaxis , antibody , sensitization , disease , pathology , bacteria , biology , genetics , receptor
The authors analyze and discuss the clinical and immunological features of hyper IgE syndrome in 40 patients suffering from the disease in Japan. There was no geographical difference observed and the sexual difference was in a ratio of 2 men to 1 woman. The syndrome appears early in infancy and begins mostly with skin lesions. The age at diagnosis is given in Fig. 1. Staphylococcus aureus is detected in every case as the pathogenic microorganism. Although the majority of the cases are of superficial infections, some cases are severe or of deep infections which become critical. The serum IgE levels are above 1,000 IU/ml in all cases and often allergic diseases are a complication. Neutrophil chemotactic defect is considered a secondary defect because a decreased in vitro chemotaxis may turn to normal skin window tests in vivo. These results indicate that lesions in lymphocytes and polymorphonuclear cells (PMNs) are not a complete defect but an incomplete defect or quantitative decline. While lymphocyte and PMN functions are damaged to various extents, the decrease or defect in the specific protective function against staphylococcus aureus is discussed. With the abnormal IgE production, specific IgE antibodies to staphylococcus aureus are produced as well as IgE antibodies to other allergens with frequent chances of sensitization.