{"title":"Treatment of Dystrophic Epidermolysis Bullosa Pruriginosa: A Systematic Review of Clinical Outcomes after Initiation of Dupilumab Therapy","authors":"Christopher J. Issa, Aubrey C. Hong, Peter A. Lio","doi":"10.1155/2023/3863357","DOIUrl":null,"url":null,"abstract":"Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr), a highly pruritic subtype of dystrophic epidermolysis bullosa (DEB), can substantially impact patients’ quality of life due to symptom severity. This review features 10 patients diagnosed with DEB-Pr and a history of insufficient symptom relief following anti-inflammatory treatment. However, after initiation of dupilumab therapy, these patients exhibited marked clinical improvements in pruritic and cutaneous symptoms. Interestingly, one study showed an increase in type VII collagen following dupilumab therapy. These findings highlight the influence of T helper 2 (Th2)-mediated immunity in the pathogenesis of itch in DEB-Pr and dupilumab’s potential in the treatment of refractory pruritus.","PeriodicalId":11045,"journal":{"name":"Dermatologic Therapy","volume":"37 1","pages":"0"},"PeriodicalIF":3.7000,"publicationDate":"2023-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Dermatologic Therapy","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1155/2023/3863357","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"DERMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr), a highly pruritic subtype of dystrophic epidermolysis bullosa (DEB), can substantially impact patients’ quality of life due to symptom severity. This review features 10 patients diagnosed with DEB-Pr and a history of insufficient symptom relief following anti-inflammatory treatment. However, after initiation of dupilumab therapy, these patients exhibited marked clinical improvements in pruritic and cutaneous symptoms. Interestingly, one study showed an increase in type VII collagen following dupilumab therapy. These findings highlight the influence of T helper 2 (Th2)-mediated immunity in the pathogenesis of itch in DEB-Pr and dupilumab’s potential in the treatment of refractory pruritus.
期刊介绍：
Dermatologic Therapy has been created to fill an important void in the dermatologic literature: the lack of a readily available source of up-to-date information on the treatment of specific cutaneous diseases and the practical application of specific treatment modalities. Each issue of the journal consists of a series of scholarly review articles written by leaders in dermatology in which they describe, in very specific terms, how they treat particular cutaneous diseases and how they use specific therapeutic agents. The information contained in each issue is so practical and detailed that the reader should be able to directly apply various treatment approaches to daily clinical situations. Because of the specific and practical nature of this publication, Dermatologic Therapy not only serves as a readily available resource for the day-to-day treatment of patients, but also as an evolving therapeutic textbook for the treatment of dermatologic diseases.
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