Hyper-IgE Syndrome: Report of Three Cases and Review of Literature

Abstract

The hyper-immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder characterized by high serum levels of immunoglobulin E (IgE), recurrent skin and lung infections, chronic dermatitis and a variety of connective tissue and skeletal abnormalities. The bacteria that commonly infect these patients are Staphylococcus aureus and Haemophilus influenzae. These patients share some characteristic facial appearance and many oral manifestations. Therapy should include prolonged antibiotic therapy. We report the clinical picture of three patients that presented recurrent infections and different outcomes, and then we describe and compare with our patients the clinical, laboratory and therapeutic aspects of HIES.




J Med Cases. 2011;2(4):151-155
doi: https://doi.org/10.4021/jmc213w