Wojas-Pelc Anna, Lipko-Godlewska Sylwia
Katedra i Klinika Dermatologii, Collegium Medicum, Uniwersytetu Jagiellońskiego w Krakowie.
Przegl Lek. 2005;62(5):310-3.
The pathogenesis of skin scleroderma (LS) is still unknown. Disturbances of vessels system, connective tissue metabolism and humoral and cellular immunological response is observed. Antinuclear antibodies are detected in 30-80% of patients with different types of skin scleroderma. They are present more often in patients with disseminated lesions and linear type of LS compared to morphoea au plaque. In our own analysis 28.5% of patients had also antibodies directed against Borrelia burgdorferi. It is believed that the injury of endothelial cells and proliferation in medial part of small vessels - which both lead to chronic ischemia - are the earliest disturbances observed in histopathological examination of the skin taken from systemic as well as from skin scleroderma patients. During last few years, there were some interesting reports concerning functional changes of endothelial cells which led to disturbances in tension of vessels smooth muscles. Free radicals - in genetically predispose people--can also provoke scleroderma lesions through their injury action on endothelial cells and stimulation of fibroblasts. In morphoea, the process of fibrosis begins around vessels. Deposition of connective tissue matrix is observed, especially collagen type I and III. This stimulation of fibroblasts as well as accumulation of connective tissue matrix are secondary to some stimulatory factors. These are: PDF, bFGF, TGFbeta and some cytokines. In morphoea patients serum levels of IL-1, IL-2, IL-4, IL-6 and IL-8 were elevated. In literature, levels and production of collagenases were decreased, although more authors say that tissue inhibitors of metalloproteinases are the main factor in fibrosis. The analysis of data tends to suspicion that enormous fibrosis observed in different types of scleroderma can be the result of increased production of collagen and other components of connective tissue as well as their incomplete degradation. Presented clinical and laboratory data show how many different factors influence etiopathogenesis of morphoea.
局限性硬皮病（LS）的发病机制仍不清楚。观察到血管系统、结缔组织代谢以及体液和细胞免疫反应存在紊乱。在30%至80%的不同类型皮肤硬皮病患者中可检测到抗核抗体。与斑块状硬斑病相比，它们更常见于有播散性损害和线状LS的患者中。在我们自己的分析中，28.5%的患者还具有抗伯氏疏螺旋体抗体。据信，内皮细胞损伤和小血管中层增殖——这两者都会导致慢性缺血——是在系统性硬皮病以及局限性硬皮病患者皮肤的组织病理学检查中最早观察到的紊乱。在过去几年中，有一些关于内皮细胞功能变化的有趣报告，这些变化导致血管平滑肌张力紊乱。自由基——在有遗传易感性的人群中——也可通过其对内皮细胞的损伤作用和成纤维细胞的刺激引发硬皮病损害。在硬斑病中，纤维化过程始于血管周围。观察到结缔组织基质的沉积，尤其是I型和III型胶原。成纤维细胞的这种刺激以及结缔组织基质的积累是某些刺激因子的继发结果。这些因子包括：血小板衍生生长因子（PDF）、碱性成纤维细胞生长因子（bFGF）、转化生长因子β（TGFβ）和一些细胞因子。在硬斑病患者中，白细胞介素-1（IL-1）、白细胞介素-2（IL-2）、白细胞介素-4（IL-4）、白细胞介素-6（IL-6）和白细胞介素-8（IL-8）的血清水平升高。在文献中，胶原酶的水平和产生量降低，尽管更多作者认为金属蛋白酶组织抑制剂是纤维化的主要因素。数据分析倾向于怀疑在不同类型硬皮病中观察到的大量纤维化可能是胶原和结缔组织其他成分产生增加以及它们不完全降解的结果。所呈现的临床和实验室数据显示了有多少不同因素影响硬斑病的病因发病机制。
