Hyper-IgE syndrome: Varied infectious presentations in four cases and review of literature

Abstract

The hyper-IgE syndrome (HIES) is a relatively rare primary immunodeficiency syndrome characterized by recurrent severe staphylococcal abscesses of skin, lungs, and other viscera as well as sinusitis, mastoiditis, eczema, markedly elevated levels of serum IgE and distinctive musculoskeletal features. High index of suspicion is required in children who present with recurrent pyogenic infections (especially staphylococcal) to diagnose this rare condition. We report four cases of HIES with different infectious presentations in whom diagnosis was established clinically and supported by elevated IgE levels and hyper-IgE score. The management includes use of intravenous antibiotics and early surgical intervention (in whom required) which resulted in resolution of symptoms in all patients. These patients were discharged on antibiotic prophylaxis and are well on follow-up.

Keywords

Job's syndrome - recurrent infections - Staphylococcus - eosinophilia - IgE - Scoliosis