Treatment of Dystrophic Epidermolysis Bullosa Pruriginosa: A Systematic Review of Clinical Outcomes after Initiation of Dupilumab Therapy

IF 3.7 4区医学 Q1 DERMATOLOGY

Dermatologic Therapy Pub Date : 2023-09-29 DOI:10.1155/2023/3863357

Christopher J. Issa, Aubrey C. Hong, Peter A. Lio

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.

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治疗大疱性priginosa营养不良表皮松解症:开始Dupilumab治疗后临床结果的系统回顾

大疱性营养不良表皮松解症(debp - pr)是一种高度瘙痒的大疱性营养不良表皮松解症(DEB)亚型，由于症状严重，可严重影响患者的生活质量。本研究回顾了10例诊断为DEB-Pr的患者，他们在抗炎治疗后症状缓解不足。然而，在开始dupilumab治疗后，这些患者在瘙痒和皮肤症状方面表现出显着的临床改善。有趣的是，一项研究显示，dupilumab治疗后，VII型胶原蛋白增加。这些发现强调了T辅助2 (Th2)介导的免疫在DEB-Pr中瘙痒发病机制中的影响，以及dupilumab治疗难治性瘙痒的潜力。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Dermatologic Therapy 医学-皮肤病学

CiteScore

7.00

自引率

8.30%

发文量

711

审稿时长

3 months

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.