Improvement in Atopic Dermatitis and Recurrent Infection With Dupilumab in Children With Distinct Genetic Types of Hyper-IgE Syndrome: A Case Series and Literature Review.

Autor: Dick JK; Division of Pediatric Dermatology, Department of Dermatology, University of Minnesota, Minneapolis, Minnesota, USA., Boull C; Division of Pediatric Dermatology, Department of Dermatology, University of Minnesota, Minneapolis, Minnesota, USA., Pozos TC; Department of Clinical Immunology, Children's Minnesota, Minneapolis, Minnesota, USA., Maguiness SM; Division of Pediatric Dermatology, Department of Dermatology, University of Minnesota, Minneapolis, Minnesota, USA.
Jazyk: angličtina
Zdroj: Pediatric dermatology [Pediatr Dermatol] 2024 Oct 18. Date of Electronic Publication: 2024 Oct 18.
DOI: 10.1111/pde.15780
Abstrakt: Hyperimmunoglobulin E syndrome (HIES) is a group of rare genetic disorders characterized by severe atopic dermatitis and recurrent skin and pulmonary infections. The efficacy of dupilumab in pediatric patients with HIES-associated severe atopic dermatitis is relatively understudied. Here, we present a series of three children with HIES, two with AD-HIES caused by STAT3 mutations, and one with AR-HIES caused by biallelic mutations in ZNF341. In all cases, dupilumab treatment led to sustained clearance of severe atopic dermatitis over multiple years, as well as improvements in systemic symptoms of HIES.
(© 2024 The Author(s). Pediatric Dermatology published by Wiley Periodicals LLC.)
Databáze: MEDLINE
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