Affiliations: Department of Pediatrics, Advanced Pediatric Centre,
Postgraduate Institute of Medical Education and Research, Chandigarh, India
Note: [] Correspondence: Sanjay Verma, Department of Pediatrics, Advanced
Pediatric Centre, Postgraduate Institute of Medical Education and Research,
Chandigarh, 160012, India. E-mail: sanjay06verma@ yahoo.com
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.
