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
