[Necrotising fasciitis in hyper-IgE syndrome]

Hyper-IgE syndrome is a rare primary immunodeficiency disease characterized by recurrent staphylococcal skin abscesses, chronic eczematoid dermatitis, pneumonia, pneumatoceles, and extreme elevation of serum IgE. The most common pathogens are S. aureus, and C. albicans. Abnormalities of dentition, bone manifestations, and connective tissue disorders are also common features of the disease. The authors report here a 19-year-old female with hyper-IgE syndrome who developed necrotising fasciitis and toxic shock syndrome. Methicillin resistant S. aureus and S. pyogenes cultured from the skin lesions. Association of hyper-IgE syndrome with necrotising fasciitis is a rarity in the medical literature. In addition to the case report, the authors describe here the major immunologic and clinical manifestations of hyper-IgE syndrome.