背景:脂类坏死病(NL)是一种罕见的,特发性,和难以治疗的胶原变性疾病,其治疗方案研究甚少。由于其反复出现的性质,溃疡的风险,高发病率,有必要了解现有的治疗方式,以更好地为临床护理提供信息。
目的:这篇综述旨在描述文献中报道的治疗NL的治疗方式。
方法:通过搜索2016年1月至2022年5月在PubMed和Scopus上发表的出版物,对治疗方法进行了文献检索。鉴于高质量证据有限,纳入病例报告和系列.仅包括提供有关尝试治疗和结果的信息的出版物。
结果:共确定了60篇新文章(54例病例报告,两个案例系列,和四项回顾性队列研究)。这些研究累计报道了274名患者,并涵盖了包括光疗在内的治疗方法。外用皮质类固醇,局部钙调磷酸酶抑制剂,生物制剂,免疫抑制剂,JAK抑制剂,联合疗法,和其他几个人。光动力疗法的证据最多(80例患者中有72例得到改善),基于UVA的光疗(33个中的12个),外用皮质类固醇(46个中的21个),压缩疗法(20个中的15个),和局部钙调磷酸酶抑制剂(17个中的11个)。还描述了几种较新的治疗方法,包括ustekinumab和JAK抑制剂。
结论:本系统综述对最近发表的NL治疗方法进行了全面总结。由于现有数据主要来自病例报告和系列,没有评估统计结论。需要更多的具有标准化终点的随机对照试验来比较治疗效果。
BACKGROUND: Necrobiosis lipoidica (NL) is a rare, idiopathic, and recalcitrant disease of collagen degeneration for which treatment options have been poorly studied. Due to its recurring nature, risk for ulceration, and high morbidity, there is a need to understand existing treatment modalities to better inform clinical care.
OBJECTIVE: This review aims to describe the therapeutic modalities reported in the literature for the treatment of NL.
METHODS: A literature search of treatments was performed by searching for publications between January 2016 and May 2022 on PubMed and Scopus. Given the limited high-quality evidence, case reports and series were included. Only publications presenting information on both attempted treatments and outcomes were included.
RESULTS: A total of 60 novel articles were identified (54 case reports, two case series, and four retrospective cohort studies). These studies cumulatively reported on 274 patients and covered treatments including phototherapy, topical corticosteroids, topical calcineurin inhibitors, biologics, immunosuppressants, JAK inhibitors, combination therapies, and several others. The greatest amount of evidence was found for photodynamic therapy (improvement in 72 of 80 patients), UVA-based phototherapy (12 of 33), topical corticosteroids (21 of 46), compression therapy (15 of 20), and topical calcineurin inhibitors (11 of 17). Several newer treatments were also described, including ustekinumab and JAK inhibitors.
CONCLUSIONS: This systematic review provides a comprehensive summary of recently published treatments for NL. As the existing data comes predominantly from case reports and series, statistical conclusions are not assessed. A greater number of randomized controlled trials with standardized endpoints are necessary to compare treatment efficacy.