Hyper-IgE syndrome

Abstract

Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency condition. It is characterized by the triad of recurrent skin and lung infections, chronic dermatitis, and elevated serum Ig E level. An 8-year-old girl presented with recurrent papulovesicular lesions on the neck, axillae, cubital, and popliteal fossae since 3 years of age. The lesions healed with scarring. There was a history of recurrent diarrhea. Cutaneous examination revealed flexural dermatitis consisting of crusted lesions on the ears, neck, axillae, groins, and genitalia. The ears were deformed, and there was atrophic scarring on the face and eyelids. Routine blood investigations revealed hypereosinophilia. Serum IgE level was >2000 KIU/L. A diagnosis of HIES was made and treated with a short course of oral antibiotics, topical steroid-antibiotic combination. Children affected with HIES can develop life-threatening pulmonary infections. Hence, early diagnosis and proper management are necessary.