局限性硬皮病，也被称作局部硬皮病，包括一组特发的皮肤硬化疾病，疾病谱包括从相对较轻的外表型—通常除引起局部不适外，还可引起少量其他不适并可看到局部皮肤病变;到包括严重并发症的亚型，例如关节挛缩和双侧肢体长度不等;嗜酸性筋膜炎(EF，舒尔曼综合征)通常被认为属于局限性硬皮病谱系中的不良转归，局限性硬皮病和嗜酸性筋膜炎疾病的明确发病机理尚待进一步研究阐明。而广泛的细胞外基质的信息及自身免疫功能紊乱被认为是发病过程中的关键。同样的，这类致病过程也被认为是系统性硬化症发病过程中的基本条件;此外局限性硬皮病和系统性硬化症的临床表现存在相似之处，很多证据表明2者之间存在关联。局限性硬皮病区别于系统性硬化症主要在于局限性硬皮病没有指端硬化、雷诺现象及甲襞毛细血管改变;局限性硬皮病的诊断通常基于特征性临床表现。皮肤活检的组织病理学评价和实验室检查在局限性硬皮病中不是主要的,然而，包括肌肉和筋膜组织的全层皮肤活检，在嗜酸性筋膜炎的诊断以及评估疾病活动度和破坏程度上是必要的，特别要包含皮下组织，这也是局限性硬皮病治疗的主要挑战之一。因此，数据的协调性对最优标准化治疗和研究结果的可比较性很重要。近期，局限性硬皮病皮肤受侵犯的评估工具(LoSCAT)被开发出来，LoSCAT是当前被报道结果最多的局限性硬皮病的测量工具;医护人员在开始治疗前应将疾病亚型、病情活动程度、累及范围及生活质量损害纳入评估。对于大部分局限于表皮的亚型，局部治疗有效。对于更大范围的疾病，起始治疗应为长波紫外线1(UVA1)的光线疗法或应用甲氨蝶呤的系统治疗，伴或不伴全身激素治疗;令人失望的是，目前仅有少数甲氨蝶呤替代方案被报道，在目前不利条件下应进行更多研究来优化治疗方案。在这篇回顾性文献中，我们将呈现最先进的流程图指导医护人员治疗局限性硬皮病和嗜酸性筋膜炎。

END

附原文：

Abstract：

Morphea, also known as localized cleroderma,encompasses agroup of idiopathic sclerotic skin diseases. The spectrum ranges fromrelatively mild phenotypes, which generally cause few problems besides localdiscomfort and visible disfigurement, to subtypes with severe complicationssuch as joint contractures and limb length discrepancies. Eosinophilic fasciitis(EF, Shulman syndrome) is often regarded as belonging to the severe end of themorphea spectrum. The exact driving

mechanisms behind morphea and EF pathogenesisremain to be elucidated. However, extensive extracellular matrix formation andautoimmune dysfunction are thought to be key pathogenic processes. Likewise,these processes are considered essential in systemic sclerosis (SSc)pathogenesis. In addition, similarities in clinical presentation between morpheaand SSc have led to many theories about their relatedness. Importantly, morpheamay be differentiated from SSc based on absence of sclerodactyly, Raynaud’sphenomenon, and nailfold capillary changes. The diagnosis of morphea is oftenbased on characteristic clinical findings. Histopathological evaluation of skinbiopsies and laboratory tests are not necessary in the majority of morphea cases.However, full-thickness skin biopsies, containing fascia and muscle tissue, arerequired for the diagnosis of EF Monitoring of disease activity and damage,especially of subcutaneous involvement, is one of the most challenging aspects ofmorphea care. Therefore, data harmonization is crucial for optimizing standardcare and for comparability of study results. Recently, the localizedscleroderma cutaneous assessment tool (LoSCAT) has been developed and validatedfor morphea. The LoSCAT is currently the most widely reported outcome measure formorphea. Care providers should take disease subtype, degree of activity, depthof involvement, and quality-of-life impairments into account when initiatingtreatment. In most patients with circumscribed superficial subtypes, treatmentwith topical therapies suffices. In more widespread disease, UVA1 phototherapyor systemic treatment with methotrexate (MTX), with or without a systemiccorticosteroid combination, should be initiated. Disappointingly, fewalternatives for MTX have been described and additional research is stillneeded to optimize treatment for these debilitating conditions. In thisreview,we present a state-of-the-art flow chart that guides care providers inthe treatment of morphea and EF.

引自：

Mertens J S, Seyger MM, Thurlings R M, et al. Morphea and Eosinophilic Fasciitis: An Update[J].American Journal of Clinical Dermatology, 2017:1-22.