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IgE hyperimmunoglobulinemia syndrome: causes, symptoms, diagnosis, treatment
Medical expert of the article
Last reviewed: 07.07.2025
IgE hyperimmunoglobulinemia syndrome combines T- and B-cell deficiency and is characterized by recurrent staphylococcal abscesses of the skin, lungs, joints, and internal organs, beginning in early childhood.
An inherited disorder transmitted in an autosomal dominant manner with incomplete penetrance; the genetic defect is unknown. Hyperimmunoglobulinemia E syndrome presents with recurrent staphylococcal abscesses of the skin, lungs, joints, and internal organs with pulmonary pneumatoceles and pruritic eosinophilic dermatitis. Patients have coarse facies, impaired tooth eruption, osteopenia, and recurrent fractures. There is tissue and blood eosinophilia and very high IgE levels [> 1000 IU/mL [> 2400 μg/L)]. Treatment consists of long-term administration of antistaphylococcal antibiotics (eg, dicloxacillin, cephalexin).
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