Hyperimmunoglobulin E syndrome

A 24-year old woman had the major features of the hyperimmunoglobulin E syndrome: recurrent staphylococcal skin infections (from the age of 6 months), an extremely elevated serum immunoglobulin E level (25,000 units/ml), and defective neutrophil chemotaxis. This patient also had peripheral blood eosinophilia and cutaneous candidiasis. There was a family history of asthma, but the patient herself did not have a history of asthma or hay fever, and, on examination, had no evidence of atopic dermatitis. The patient has not had any systemic infections. Results of the skin biopsy showed dermal edema and a perivascular infiltrate with eosinophils and an increased number of mast cells. The clinical spectrum of the hyperimmunoglobulin E syndrome may include atopic dermatitis, mucocutaneous candidiasis, systemic infections, and/or the features of Job's syndrome.