Treatment of severe atopic dermatitis and eosinophilic esophagitis with dupilumab in a 14-year-old boy with autosomal dominant hyper-IgE syndrome

Abstract

Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Job syndrome and Buckley syndrome, is characterized by elevated IgE levels, eosinophilia, recurrent cutaneous and internal organ abscesses, pneumonia leading to pneumatoceles, mucocutaneous candidiasis, characteristic facies, delayed shedding of primary teeth, bone fractures due to minor trauma, joint hypermobility, and scoliosis.1 In addition, allergic disorders such as atopic dermatitis (AD), food allergy, and eosinophilic esophagitis (EoE)2 may be associated with AD-HIES.