Necrotizing fasciitis in association with hyperimmunoglobulin E syndrome.

A case of necrotizing fasciitis in association with hyperimmunoglobulin E (HIE) syndrome is reported. The patient was a 17-year-old Japanese boy with a clinical history of recurrent skin and pulmonary infections and eczematoid dermatitis, markedly elevated serum levels of IgE, and coarse facies. He had a gangrenous swelling on the lower abdominal wall, and his general condition was poor with high fever. The involved site was accompanied by subcutaneous gas; the culture of the pus of the lesion grew anaerobes without mixed growth of Staphylococcus aureus. Exhaustive debridement of necrotic fascia, which extended much farther than the gangrenous area, and administration of antibiotics had a curative effect on the gangrenous soft tissue infection. To the best of the authors' knowledge, this is the first published case of necrotizing fasciitis in association with HIE syndrome.