Relieving job: Dupilumab in autosomal dominant STAT3 hyper-IgE syndrome

Abstract

Autosomal dominant hyper-IgE syndrome (AD-HIES) was initially described as Job syndrome”1 and renamed because of high IgE levels.2 Heterozygous mutations in STAT3 were initially identified as the genetic cause of AD-HIES.3 Patients exhibit (1) pulmonary infections, mainly owing to Staphylococcus aureus and Streptococcus pneumoniae; (2) bone brittleness and retention of decidual and delayed dentition of permanent teeth; and (3) dermatitis and skin infections with S aureus and Candida spp.4 Although prophylaxis and treatment of infections have advanced, options for HIES-related dermatitis are limited and consist of emollients, topical steroids, and calcineurin inhibitors.