Department of Dermatology, Venerology and Allergology, Ruhr-University Bochum, Bochum, Germany.
Dermatol Ther. 2012 Mar-Apr;25(2):135-47. doi: 10.1111/j.1529-8019.2012.01479.x.
Localized scleroderma (also called morphea) is a term encompassing a spectrum of sclerotic autoimmune diseases that primarily affect the skin, but also might involve underlying structures such as the fat, fascia, muscle, and bones. Its exact pathogenesis is still unknown, but several trigger factors in genetically predisposed individuals might initially lead to an immunologically triggered release of pro-inflammatory cytokines, resulting in a profound dysregulation of the connective tissue metabolism and ultimately to induction of fibrosis. To date, there are no specific serological markers available for localized scleroderma. Within the last years, several validated clinical scores have been introduced as potential outcome measures for the disease. Given the rarity of localized scleroderma, only few evidence-based therapeutical treatment options exist. So far, the most robust data is available for ultraviolet A1 phototherapy in disease that is restricted to the skin, and methotrexate alone or in combination with systemic corticosteroids in more severe disease that additionally affects extracutaneous structures. This practical review summarizes relevant information on the epidemiology, pathogenesis, clinical subtypes and classifications, differential diagnoses, clinical scores and outcome measures, and current treatment strategies of localized scleroderma.
局限性硬皮病（也称为硬斑病）是一个涵盖了一系列主要影响皮肤的硬化性自身免疫性疾病的术语，但也可能涉及潜在的结构，如脂肪、筋膜、肌肉和骨骼。其确切的发病机制尚不清楚，但在遗传易感个体中，一些触发因素可能最初导致免疫触发释放促炎细胞因子，导致结缔组织代谢严重失调，最终诱导纤维化。迄今为止，局限性硬皮病尚无特异性的血清学标志物。在过去的几年中，已经引入了几种经过验证的临床评分作为疾病的潜在预后指标。鉴于局限性硬皮病的罕见性，只有少数基于证据的治疗选择存在。到目前为止，在仅累及皮肤的疾病中，紫外线 A1 光疗的证据最为充分，而在更严重的疾病中，单独使用甲氨蝶呤或联合全身皮质类固醇治疗，同时累及皮肤外结构。本实用综述总结了局限性硬皮病的流行病学、发病机制、临床亚型和分类、鉴别诊断、临床评分和预后指标以及当前治疗策略的相关信息。
