Treatment efficacy of dupilumab in a hyper-immunoglobulin E syndrome patient with severe atopic dermatitis

Abstract

EASI: eczema area and severity index HIES: hyper-immunoglobulin E syndrome IgE: immunoglobulin E IU: international units INTRODUCTION Hyper-immunoglobulin E syndrome (HIES) is a rare immunodeficiency characterized by a high serum IgE level, atopic dermatitis, and recurrent skin and lung infections. The exact prevalence of HIES is unknown, but the overall incidence rate of primary immunodeficiency was reported to be 4.6 per 100,000 person-years. Genetic variants in STAT3, DOCK8, and PGM3 have been associated with increased HIES risk. Traditionally, the main treatments include long-term prophylactic antibiotics, topical anti-inflammatory agents, and supportive care. Recently, biologics have emerged as an additional option for treating HIES with treatmentrefractory atopic dermatitis. Dupilumab, a human monoclonal antibody that blocks the shared receptor subunit of IL-4 and IL-13, mitigates the signs and symptoms of atopic dermatitis. Previously, 2 case reports have demonstrated the substantial improvements of atopic dermatitis following the injection of dupilumab. Here, we report a rare HIES case whose atopic dermatitis was successfully controlled with a lower dose of dupilumab.