It is characterized by recurrent "cold" staphylococcal infections (due to impaired recruitment of neutrophils),[2] unusual eczema-like skin rashes, severe lung infections that result in pneumatoceles (balloon-like lesions that may be filled with air or pus or scar tissue) and very high (> 2000 IU/mL or 4800 mcg/L)[3] concentrations of the serum antibody IgE.

[5] Abnormal neutrophil chemotaxis due to decreased production of interferon gamma by T lymphocytes is thought to cause the disease.

[20] Most patients with hyper IgE syndrome are treated with long-term antibiotic therapy to prevent staphylococcal infections.

[21] HIES was first described by Davis et al. in 1966 in two girls with red hair, chronic dermatitis, and recurrent staphylococcal abscesses and pneumonias.

In 1972, Buckley et al. described two boys with similar symptoms as well as coarse facies, eosinophilia, and elevated serum IgE levels.