Systematic review on antipruritic therapies for patients with Epidermolysis bullosa.

Tobias Welponer, Christian Puttinger, Eva W H Korte, Sjoukje van der Werf, Christine Prodinger, Marieke C Bolling, Martin Laimer
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Abstract

Introduction: Itch is one of the most burdensome symptoms in epidermolysis bullosa (EB), indicating a hitherto unmet therapeutic need. This review leverages existing data on efficacy of itch treatment in EB to support sound decision making.

Methods: A systematic literature search was performed on 29 March 2022. Studies written later than 1991 and reporting outcomes in patients with EB treated for itch were considered.

Results: Of the 3,099 articles screened, 21 studies met eligibility criteria, comprising 353 patients (65.9%) diagnosed for recessive dystrophic EB. Only two studies (9.5%) evaluated itch as primary endpoint, of which solely one revealed a significant relief of self-reported itch upon topical skin care. In those studies assessing itch as secondary endpoint (19/21, 90.5%), only 36.8% studies (n = 7/19) revealed a statistically significant itch reduction of up to 42%. Methodological limitations (heterogeneity of outcomes, inconsistent data assessment) in addition to limited superiority over control were implicated to account for low treatment efficacy observed in most studies.

Conclusion: Current data quality impairs comparative efficacy analyses of itch treatments in EB. Large scale randomized clinical trials and more personalized approaches applying validated measurement instruments for core outcomes are needed to substantiate evidence-based treatment approaches for EB-associated itch.

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关于大疱性表皮松解症患者止痒疗法的系统综述。
简介:瘙痒是大疱性表皮松解症(EB)最令人头疼的症状之一,表明治疗需求迄今尚未得到满足。本综述利用现有的EB瘙痒治疗疗效数据,为合理决策提供支持:方法:2022 年 3 月 29 日进行了系统性文献检索。方法:于 2022 年 3 月 29 日进行了系统性文献检索,考虑了 1991 年以后撰写的、报告 EB 患者瘙痒治疗结果的研究:在筛选出的 3099 篇文章中,有 21 项研究符合资格标准,包括 353 名(65.9%)被诊断为隐性营养不良性 EB 的患者。只有两项研究(9.5%)将瘙痒作为主要终点进行了评估,其中只有一项研究显示局部皮肤护理能显著缓解患者自述的瘙痒症状。在那些将瘙痒作为次要终点进行评估的研究中(19/21,90.5%),只有 36.8% 的研究(n = 7/19)显示瘙痒在统计学上有显著缓解,缓解率高达 42%。大多数研究中观察到的疗效较低的原因是方法上的局限性(结果的异质性、数据评估的不一致性):结论:目前的数据质量影响了EB瘙痒治疗的疗效比较分析。需要进行大规模的随机临床试验,并采用更个性化的方法,使用经过验证的测量工具来测量核心结果,以证实以证据为基础的 EB 相关瘙痒治疗方法。
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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