Treatment efficacy of dupilumab in a hyper-immunoglobulin E syndrome patient with severe atopic dermatitis
Chia‐Jui Su, Han‐Chi Tseng
Abstract
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.1,2 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.
Topics & Concepts
Atopic dermatitisDupilumabMedicinePrimary immunodeficiencyImmunoglobulin EImmunologyDermatologyIncidence (geometry)AntibodyImmunodeficiencyAllergyImmune systemPhysicsOpticsImmunodeficiency and Autoimmune DisordersImmune Cell Function and InteractionT-cell and B-cell Immunology