Hyperimmunoglobulinemia E Syndrome with Recurrent Infections

Hyperimmunoglobulinemia E syndrome (HIES) is a rare primary immunodeficiency disorder characterized by markedly elevated serum immunoglobulin E (IgE) levels (＞2000 IU/ml), recurrent pyogenic infections of the skin and the respiratory tract, chronic eczematous dermatitis, skeletal abnormalities, and peripheral eosinophilia. We describe a 29-year-old female with HIES, who suffered from chronic generalized eczema and recurrent pyogenic cutaneous infection since early childhood. Laboratory analysis revealed a persistent elevation of total serum IgE level (＞8000 IU/ml). Escherichia coli and Enterobacter aerogenes were isolated from a cold abscess on the buttocks. Flow cytometry showed impairment of neutrophil phagocytotic uptake capacity of gram-negative Klebsiella pneumoniae. The cutaneous abscess responded to incision, drainage, and prolonged oral antibiotic therapy. Improvement of the skin condition was achieved after treatment with antihistamines, topical steroids, and prophylactic antibiotics.