Francès Camille, Ayoub Nakhle, Barete Stéphane
Service de médecine interne Groupe hospitalier La Pitié-La Salpêtrière 75651 Paris.
Rev Prat. 2002 Nov 1;52(17):1884-90.
Systemic sclerosis is a multisystem disorder characterized by the presence of connective tissue sclerosis, vascular abnormalities concerning small-sized arteries and the microcirculation, and autoimmune changes. This disease is usually preceded by Raynaud's phenomenon. Cutaneous changes are useful for diagnosis. Sclerosis is initially proeminent on hands with frequently pitted scars. Telangiectases and calcium deposits are mainly observed in a good prognosis subset of the disease (CREST) characterized by the association of limited skin involvement and anti-centromere antibodies with possible late onset of pulmonary hypertension. The bad prognosis subset is associated with diffuse cutaneous sclerosis, pulmonary fibrosis, gastrointestinal disease, myocardial involvement and anti-topo-isomerase I antibodies. Histological skin changes are similar to those observed in morphea. In morphea, there is only localized sclerosis of the skin with highly variable aesthetic disability. The condition may be subdivided clinically into the following types: circumscribed plaques, linear morphea and frontoparietal lesions. The last two forms may be associated with atrophy of the underlying anatomic structures. Many other diseases may have a scleroderma-like appearance. In these diseases, nail folds abnormalities, visceral involvement and immunological changes are usually different from those observed in systemic scleroderma.
系统性硬化症是一种多系统疾病，其特征为存在结缔组织硬化、小动脉和微循环的血管异常以及自身免疫改变。这种疾病通常先出现雷诺现象。皮肤改变有助于诊断。硬化最初在手部较为突出，常有凹陷性瘢痕。毛细血管扩张和钙质沉积主要见于疾病预后较好的一个亚型（CREST），其特征为皮肤受累局限、抗着丝点抗体阳性，可能后期出现肺动脉高压。预后较差的亚型与弥漫性皮肤硬化、肺纤维化、胃肠道疾病、心肌受累及抗拓扑异构酶I抗体相关。组织学上皮肤改变与硬斑病所见相似。在硬斑病中，仅皮肤有局限性硬化，美观残疾程度差异很大。临床上该病可分为以下类型：局限性斑块型、线状硬斑病和额顶部损害。后两种类型可能伴有深部解剖结构萎缩。许多其他疾病可能有硬皮病样表现。在这些疾病中，甲襞异常、内脏受累及免疫改变通常与系统性硬化症不同。
