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Hyperimmunoglobulinemia IgE Syndrome: Causes, Symptoms, Diagnosis, Treatment
Last reviewed: 23.04.2024
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Syndrome of hyperimmunoglobulinemia IgE combines T- and B-cell insufficiency and is characterized by recurrent staphylococcal abscesses of the skin, lungs, joints, internal organs that begin in early childhood.
Hereditary disease transmitted by autosomal dominant type with incomplete penetrance; genetic defect is unknown. The syndrome of hyperimmunoglobulinemia E is manifested by recurrent staphylococcal abscesses of the skin, lungs, joints, internal organs with pulmonary pneumatology and itching eosinophilic dermatitis. Patients have rough facial features, dysplasia, osteopenia, recurrent fractures. There is eosinophilia in tissues and blood and a very high IgE level [> 1000 IU / ml [> 2400 μg / l]]. Treatment consists in the long-term administration of anti-staphylococcal antibiotics (eg, dicloxacillin, cephalexin).
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