Treatment of Hailey-Hailey disease with biologics and small-molecule inhibitors: a systematic review.

IF 3.7 4区医学 Q1 DERMATOLOGY

Clinical and Experimental Dermatology Pub Date : 2024-12-23 DOI:10.1093/ced/llae298

Wei Liu, Xiao Xue, Shanshan Li

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Abstract

Hailey-Hailey disease (HHD) is a rare genetic dermatosis characterized by recurrent flaccid vesicles and blisters on erythematous skin in friction areas. The disease follows a chronic relapsing course and has a significant psychological and social impact. Currently, there is no standardized therapeutic regimen for HHD, posing a challenge for dermatologists in managing the condition. We performed this systematic review to investigate the therapeutic role of biologics and small-molecule inhibitors in the treatment of HHD. A systematic search was conducted of the PubMed, Embase, Web of Science, Scopus and Cochrane databases from inception to 1 January 2024. In total, 31 patients with HHD from 18 articles were included in the analysis. Biologics and small-molecule inhibitors were evaluated, including dupilumab, apremilast, upadacitinib, abrocitinib, adalimumab and etanercept. Most reported cases demonstrated clinical improvement after treatment initiation, with few major adverse events. However, some patients experienced recurrences. In conclusion, biologics and small-molecule inhibitors may offer a treatment alternative for patients with refractory HHD, but further confirmation is necessary through large-scale randomized controlled clinical trials.

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用生物制剂和小分子抑制剂治疗海利-海利病：系统综述。

海利-海利病（Hailey-Hailey disease，HHD）是一种罕见的遗传性皮肤病，其特征是在摩擦部位的红斑皮肤上反复出现松弛性水泡和水疱。该病呈慢性复发性，对患者的心理和社会影响很大。目前，HHD 还没有标准化的治疗方案，这给皮肤科医生的治疗带来了挑战。我们进行了这项系统性综述，以研究生物制剂和小分子抑制剂在治疗 HHD 中的治疗作用。我们在 PubMed、Embase、Web of Science、Scopus 和 Cochrane 数据库中进行了系统检索，检索时间从开始到 2024 年 1 月 1 日。共有18篇文章中的31名HHD患者被纳入分析。对生物制剂和小分子抑制剂进行了评估，包括杜必鲁单抗、阿普瑞米拉斯特、达帕替尼、阿昔替尼、阿达木单抗和依那西普。大多数报告病例显示，开始治疗后临床症状有所改善，很少出现重大不良反应。不过，也有一些患者病情复发。总之，生物制剂和小分子抑制剂可能为难治性HHD患者提供了一种治疗选择，但还需要通过大规模随机对照临床试验来进一步证实。

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

Clinical and Experimental Dermatology 医学-皮肤病学

CiteScore

3.20

自引率

2.40%

发文量

389

审稿时长

3-8 weeks

期刊介绍： Clinical and Experimental Dermatology (CED) is a unique provider of relevant and educational material for practising clinicians and dermatological researchers. We support continuing professional development (CPD) of dermatology specialists to advance the understanding, management and treatment of skin disease in order to improve patient outcomes.