Clearance of atypical cutaneous manifestations of hyper‐IgE syndrome with dupilumab

Hyper‐IgE syndromes (HIES) are a heterogeneous group of rare primary immunodeficiency diseases classically characterized by the triad of atopic dermatitis, and recurrent cutaneous and pulmonary infections. Autosomal dominant, loss‐of‐function STAT3 pathogenic variants are the most common genetic cause, which lead to deficiency of Th17 lymphocytes, impaired interferon gamma production, and IL‐10 signal transduction, and an unbalanced IL‐4 state. Dupilumab, a monoclonal antibody to the IL‐4a receptor, inhibits both IL‐4 and IL‐13, and has been shown to improve atopic dermatitis and other manifestations of HIES including asthma and allergic bronchopulmonary aspergillosis. We present a pediatric patient with HIES who presented predominantly with eosinophilic folliculitis, recurrent cutaneous infections, and other non‐eczematous findings and achieved sustained clearance with dupilumab.