Restrepo José Félix, Guzmán Renato, Rodríguez Gerzaín, Iglesias Antonio
Internal Medicine and Rheumatology Department, Universidad Nacional de Colombia, Bogotá, D.C., Colombia.
Biomedica. 2003 Dec;23(4):408-15.
Linear scleroderma (LS) is a localized form of scleroderma characterized by mononuclear cell infiltration and fibroblast proliferation. In the later stages of the disease, excessive collagen is deposited with concomitant skin and appendage atrophy. These symptoms suggest a breakdown of fibroblast cell function, and consequently, growth factors have been thought to play a role in the pathogenesis of LS. The present study examined the expression of TGF-beta and PDGF in skin biopsies obtained from patients with LS and from normal subjects. Samples were prepared for immunohistochemistry. To identify TGF-beta, two polyclonal antibodies were used: TGF-beta1 (RaB4) and TGF-beta2 (CL-B1/29) and, to identify PDGF, two monoclonal antibodies were used: PDGF-AA (3E-205) and PDGF-BB (1F-133). Staining for TGF-beta1 and TGF-beta2 was observed around blood vessels (endothelial cells), and sweat glands in both LS and normal skin. Staining for PDGF-AA and PDGF-BB was intense in endothelial cells and sweat glands in LS and normal skin. Mononuclear cell infiltrates and abnormal collagen bundles did not stain for TGF-beta or PDGF. The strength and extent of staining was evaluated in tissues using a scale from zero (no staining) to four (strong staining). The amount of TGF-beta1, TGF-beta2, PDGF-AA and PDGF-BB was found similar in LS and normal skin. These results do not support the hypothesis that the excessive fibroblast cell activity and abnormal collagen deposition observed in LS are associated with downregulation of TGF-beta or PDGF.
线性硬皮病(LS)是硬皮病的一种局限性形式,其特征为单核细胞浸润和成纤维细胞增殖。在疾病后期,会伴有皮肤和附属器萎缩,同时有过量胶原蛋白沉积。这些症状表明成纤维细胞功能出现故障,因此,生长因子被认为在LS的发病机制中发挥作用。本研究检测了从LS患者和正常受试者获取的皮肤活检样本中转化生长因子-β(TGF-β)和血小板衍生生长因子(PDGF)的表达。样本被制备用于免疫组织化学。为鉴定TGF-β,使用了两种多克隆抗体:TGF-β1(RaB4)和TGF-β2(CL-B1/29);为鉴定PDGF,使用了两种单克隆抗体:PDGF-AA(3E-205)和PDGF-BB(1F-133)。在LS皮肤和正常皮肤的血管(内皮细胞)及汗腺周围均观察到TGF-β1和TGF-β2染色。在LS皮肤和正常皮肤的内皮细胞及汗腺中,PDGF-AA和PDGF-BB染色强烈。单核细胞浸润和异常胶原束未显示TGF-β或PDGF染色。使用从0(无染色)到4(强染色)的评分标准对组织中的染色强度和范围进行评估。发现LS皮肤和正常皮肤中TGF-β1、TGF-β2、PDGF-AA和PDGF-BB的量相似。这些结果不支持以下假设:在LS中观察到的成纤维细胞过度活动和异常胶原沉积与TGF-β或PDGF的下调有关。