Hyper-IgE syndrome: A rare cause of recurrent infections in children

Abstract

Hyperimmunoglobulinemia E or hyper-IgE syndrome (HIES) is a complex primary immunodeficiency disorder characterized by recurrent skin and pulmonary infections, eczema, eosinophilia, and high serum levels of IgE. The exact prevalence of HIES remains unknown, although this condition is considered to affect less than 1 per million people worldwide. We present a case-report of a 9 year-old girl, symptomatic since early infancy, with distinctive facial features (broad nasal bridge, antimongoloid palpebral fissures), eczema, persistence of primary teeth and recurrent bacterial infections (skin and pulmonary abscesses) due to Staphyloccocus aureus. The diagnosis of HIES was established according to National Institutes of Health scoring system based on both clinical and laboratory findings. Despite antibacterial prophylaxis with trimethoprim-sulfamethoxazole, the patient developed a staphylococcal abscess of right temporal region one year after the last admission in the hospital.

Keywords

• hyperimmunoglobulinemia e
• recurrent infections
• children