Hyper-IgE syndrome with recurrent infections — An autosomal dominant multisystem disorder

Abstract

Hyper IgE syndrome (HIES) is a rare disorder characterized by eczema, recurrent infections of the skin and lungs, typically with Staphylococcus aureus, Candida albicans and certain viruses, and elevated levels of serum IgE. Other clinical manifestations include characteristic facies (prominent forehead, broad nasal bridge and facial asymmetry), chronic eczematous dermatitis, retained primary dentition, recurrent pathological fractures, hyper-extensibility and scoliosis. The respiratory system involvement in HIES has been reported with HRAD. Here we presented a case of HIES with rare associations of Hyperreactive airway disease.