Mayes M D
Division of Rheumatology, Wayne State University, Detroit, MI 48201, USA.
Semin Cutan Med Surg. 1998 Mar;17(1):22-6. doi: 10.1016/s1085-5629(98)80058-8.
Scleroderma is classified as two separate but related entities, a localized form and a systemic form. The classification scheme for morphea presented here is that of Peterson et al, which divides morphea into five categories: plaque, generalized, bullous, linear, and deep. Using this system, these authors estimated the incidence rate of localized scleroderma to be 27 new cases per million population per year. Overall survival was similar to that of the general population. There was a preponderance of female cases (approximately 3:1) for all forms of morphea except for linear scleroderma, which had an even sex distribution. Systemic scleroderma is divided into limited and diffuse disease based on the extent of skin involvement. Recent estimates have placed the incidence rate of systemic sclerosis in the United States at 19 new cases per million adults per year, with an overall prevalence of 240/million adults. The female-to-male ratio is approximately 5:1. The prevalence of scleroderma varies by geographic region and ethnic background and is higher in the United States than in Europe or Japan. Although systemic sclerosis survival has improved over the past two decades, with 5-year survival over 80%, long-term survival is significantly lower than expected, and morbidity is considerable.
硬皮病分为两种独立但相关的类型，即局限性硬皮病和系统性硬皮病。此处介绍的硬斑病分类方案是彼得森等人提出的，该方案将硬斑病分为五类：斑块状、泛发性、大疱性、线状和深部硬斑病。使用该系统，这些作者估计局限性硬皮病的发病率为每年每百万人口中有27例新发病例。总体生存率与普通人群相似。除线状硬皮病性别分布均匀外，所有类型的硬斑病女性病例均占优势（约为3:1）。系统性硬皮病根据皮肤受累程度分为局限性和弥漫性疾病。最近的估计显示，美国系统性硬化症的发病率为每年每百万成年人中有19例新发病例，总体患病率为每百万成年人中有240例。女性与男性的比例约为5:1。硬皮病的患病率因地理区域和种族背景而异，在美国高于欧洲或日本。尽管在过去二十年中系统性硬化症的生存率有所提高，5年生存率超过80%，但长期生存率仍显著低于预期，且发病率较高。
