Scharffetter K, Kind P, Wollny-Protzel D, Schuppe H C, Lakomek H J, Goerz G
Hautklinik, Universität Düsseldorf.
Z Hautkr. 1990 Mar;65(3):237-42, 245-6.
Sclerodermas may occur in two basic forms: localized sleroderma (LSc) and systemic scleroderma (SSc). Pseudoscleroderma as well as overlap syndromes have to be differentiated from these two variants. From the clinical point of view, localized scleroderma can be subdivided into type I = plaque-like LSc (= morphea), type II = linear LSc, and type III = deep LSc. According to the degree of the cutaneous involvement, systemic scleroderma can likewise be classified into type I = sclerodactylia, type II = acrosclerosis, and type III = scleroderma with primary involvement of the trunk (diffuse scleroderma). In LSc, we never find systemic involvement; SSc, in contrast, is almost always associated with Raynaud's phenomenon, changes of the esophagus, as well as an increased titer of antinuclear antibodies (Hep-2 cell test). Only 23% of our patients with LSc showed elevated ANA titers. We present and discuss data of 56 patients with LSc and 52 patients with SSc. Evidence in the literature as well as our own findings suggest that the pathogenesis of LSc is different from that of SSc. The influence of various mediators and cytokines on the collagen metabolism might be regarded as a theoretical approach in order to develop new therapeutic regimens. This is even more important since there is still no efficient mode of treatment for neither localized nor systemic scleroderma.
局限性硬皮病（LSc）和系统性硬皮病（SSc）。必须将假性硬皮病以及重叠综合征与这两种类型区分开来。从临床角度来看，局限性硬皮病可细分为I型 = 斑块状LSc（= 硬斑病），II型 = 线状LSc，以及III型 = 深部LSc。根据皮肤受累程度，系统性硬皮病同样可分为I型 = 硬指，II型 = 肢端硬化，以及III型 = 以躯干为主受累的硬皮病（弥漫性硬皮病）。在局限性硬皮病中，我们从未发现有系统性受累情况；相比之下，系统性硬皮病几乎总是与雷诺现象、食管改变以及抗核抗体滴度升高（Hep-2细胞检测）相关。我们的局限性硬皮病患者中只有23%显示抗核抗体滴度升高。我们展示并讨论了56例局限性硬皮病患者和52例系统性硬皮病患者的数据。文献证据以及我们自己的研究结果表明，局限性硬皮病的发病机制与系统性硬皮病不同。各种介质和细胞因子对胶原蛋白代谢的影响可能被视为开发新治疗方案的一种理论方法。鉴于局限性和系统性硬皮病目前仍然都没有有效的治疗方式，这一点就显得尤为重要。
