Hyper-Ige Syndrome

(Hyperimmunoglobulinemia E syndrome (Buckley syndrome)

When Hyper-IgE syndrome is a hereditary combined B- and T-cell defect, which is characterized by the appearance of recurrent Staphylokokkenabszesse skin sinopulmonary infections and severe purulent eosinophilic dermatitis.

(See also Overview of immune deficiency disorders, and approach to the patient with an immunodeficiency disorder.)

When Hyper-IgE syndrome is a hereditary combined B- and T-cell defect, which is characterized by the appearance of recurrent Staphylokokkenabszesse skin sinopulmonary infections and severe purulent eosinophilic dermatitis. (See also Overview of immune deficiency disorders, and approach to the patient with an immunodeficiency disorder.) The hyper-IgE syndrome is a primary immunodeficiency disease, which comprises the combined humoral and cellular immune deficiency disorders. Inheritance can be autosomal dominant: caused by mutations in STAT3 (signal transducer and activator of transcription 3) – gene Autosomal recessive: shining through homozygous null mutations in TYK2- (tyrosine kinase-2) or DOCK8- (Dedicator cytokinesis 8) – gene to be caused. The hyper-IgE syndrome begins in childhood. Symptoms and complaints The hyper-IgE syndrome typically causes recurrent Staphylokokkenabszesse the skin, lungs, joints and viscera, sinopulmonaler infections, pulmonary pneumatoceles and a heavy purulent eosinophilic dermatitis is typical of the hyper-IgE syndrome. Patients have coarse facial features, the dentition is delayed, there are osteopenia and repeated prior fractures. All have a tissue and blood eosinophilia and very high IgE levels (> 2000 I.U./ml). Diagnosis IgE levels in the serum of the suspected diagnosis of hyper-IgE syndrome results from the symptoms and is confirmed by the measurement of Serumbilirubinspiegels. Genetic testing can identify genetic mutations and are mainly performed to confirm the diagnosis or to predict patterns of inheritance. Prophylactic therapy staphylococci Effective antibiotics Sometimes interferon gamma in severe infection, the treatment of a hyper-IgE syndrome consists of prophylactic antibiotics staphylococci Effective (usually trimethoprim / sulfamethoxazole). The dermatitis is treated with the skin moisturizing, emollient creams and antihistamines; when infections are suspected, antibiotics are added to the treatment. Pulmonary complications are treated early and aggressively with antibiotics. Interferon-gamma has been successfully used in life-threatening infections.

Health Life Media Team

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