School of Medicine, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX, 77030, USA.
Department of Dermatology, Baylor College of Medicine, Houston, TX, USA.
Am J Clin Dermatol. 2022 Sep;23(5):673-688. doi: 10.1007/s40257-022-00700-4. Epub 2022 May 23.
Genodermatoses are genetically inherited dermatologic conditions. The management of cutaneous findings in genodermatoses is challenging, and first-line therapies, such as steroids and/or retinoids, are often inadequate. In recent years, research on the molecular basis of genodermatoses has led to the use of biologic therapies for intractable disease. Here, we review the evidence regarding the use of available biologic therapies for the management of dermatologic findings in genodermatoses. Biologic therapies appear to be promising therapeutic options for several recalcitrant genodermatoses, especially those with underlying immune dysregulation. However, not all genodermatoses are amenable to biologic therapies, and some have been shown to paradoxically worsen under treatment. Biologic therapies offer a novel avenue to target refractory genodermatoses. However, evidence supporting the use of biologic therapies in the management of genodermatoses is mostly limited to case reports and case series. Further studies are warranted to determine the safety and efficacy of biologic therapies for the management of cutaneous findings in genodermatoses.
遗传性皮肤病是一种遗传性皮肤疾病。遗传性皮肤病的皮肤表现的治疗具有挑战性，一线治疗方法，如类固醇和/或维甲酸，往往效果不佳。近年来，对遗传性皮肤病的分子基础的研究导致了使用生物疗法治疗难治性疾病。在这里，我们回顾了关于使用现有生物疗法治疗遗传性皮肤病皮肤表现的证据。生物疗法似乎是几种难治性遗传性皮肤病的有前途的治疗选择，特别是那些潜在免疫失调的疾病。然而，并非所有遗传性皮肤病都适合生物疗法，并且一些疾病在治疗下显示出反常恶化。生物疗法为治疗难治性遗传性皮肤病提供了一种新的途径。然而，支持生物疗法治疗遗传性皮肤病的证据主要限于病例报告和病例系列。需要进一步的研究来确定生物疗法治疗遗传性皮肤病皮肤表现的安全性和疗效。
