Pathogenesis of Scleroderma
Pathogenesis of Scleroderma
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硬皮病的发病机制
DOI：
10.1111/j.1365-4362.1984.tb05678.x
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发表时间：
1984
影响因子：
3.6
通讯作者：
L. Diaz
中科院分区：
文献类型：
--
作者：
Eddie B. Lee;G. Anhalt;J. Voorhees;L. Diaz
术语“硬皮病”传统上已被用于描述涉及皮肤硬化、收紧和弹性降低的一组异质疾病的皮肤变化。“已经确定了两种极端形式-局部和扩散。局部型，特别是硬斑病，其特征是边界清楚，具有象牙色中心和周围紫色晕的斑块。紫色晕圈暗示疾病活动。未观察到内脏受累，因此，预后通常是可预测的。2与之形成鲜明对比的是弥漫型或进行性系统性硬化症（PSS），其与广泛性硬化、雷诺现象和多系统受累相关。预后差，死亡通常继发于肾衰竭或肺纤维化5，在这两个极端之间是中间形式，全身性硬斑病代表局限性硬皮病和肢端硬化症的一种形式，CREST综合征代表PSS的一种形式。1.6制定PSS全谱的定义需要了解病因和发病机制。这反过来又可以阐明进行性系统性硬化症、硬斑病和中间类型之间的相互关系。根据Lever和Schaumberg-Lever，硬斑病与PSS的皮肤病变的组织学区分通常是不可能的;然而，他们确定了两个阶段：（1）早期炎症;和（2）！吃了一顿大餐。在早期炎症阶段，真皮显示出紧密堆积的厚胶原纤维束，
The term "scleroderma" traditionally has been uti l ized to describe the cutaneous changes of a heterogeneous group of disorders involving hardening, tightening, and decreased elasticity of the skin.' Two extreme forms have been identified-localized and diffuse. The localized form, specifically morphea, i s characterized by well-circumscribed, sclerotic plaques with an ivory-colored center and a surrounding violaceous halo. The violaceous halo implies active disease. Visceral involvement is not observed and, therefore, prognosis is generally favorable.2 In sharp contrast is the diffuse type, or progressive systemic sclerosis (PSS), which is correlated with widespread sclerosis, Raynaud's phenomenon, and multisystem invol~ement .~-~ Prognosis is poor, with death often secondary to renal failure or pulmonary fibrosis5 Between these two extremes are intermediate forms, with generalized morphea representing a form of localized scleroderma and acrosclerosis and the CREST syndrome representing a form of PSS.1.6 Formulating a definition of the full spectrum of PSS necessitates an understanding of the etiology and pathogenesis. This, in turn, may clarify the interrelationship between progressive systemic sclerosis, morphea, and the intermediate varieties. According to Lever and Schaumberg-Lever, histologic differentiation of the skin lesions in morphea versus PSS i s usually impossible; however, they identify two stages: (1 1 early inflammatory; and (2 ) !ate sclerotic.' During the early inflammatory stage, the dermis reveals closely packed, thick collagen bundles in conjunction with a
影响因子：
6.5
作者：
FLEISCHMAJER, R;PERLISH, JS;TIMPL, R
通讯作者：
TIMPL, R