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嗜酸性筋膜炎。受累皮肤来源的成纤维细胞中胶原蛋白生成增加及I型前胶原信使核糖核酸水平升高。

Eosinophilic fasciitis. Increased collagen production and type I procollagen messenger RNA levels in fibroblasts cultured from involved skin.

作者信息

Kähäri V M, Heino J, Niskanen L, Fräki J, Uitto J

机构信息

Department of Dermatology, Jefferson Medical College, Philadelphia, Pa.

出版信息

Arch Dermatol. 1990 May;126(5):613-7.

PMID:2334180
Abstract

Two patients with eosinophilic fasciitis were studied to elucidate the activation of collagen production in this disorder. Histologic examination of biopsy specimens from the involved area of skin revealed the presence of inflammatory cell infiltrates and various degrees of collagen accumulation in the dermis, subcutis, fascia, and underlying muscle. Fibroblast cultures initiated from the involved skin exhibited 2.0- to 3.7-fold increased collagen production when compared with control fibroblast cultures established from the uninvolved skin of the same patients. Eosinophilic fasciitis fibroblasts also displayed 2.4- to 6.2-fold higher steady-state levels of type I procollagen messenger RNA than did the control cells, indicating pretranslational activation of type I procollagen gene expression. In addition, cellular fibronectin messenger RNA steady-state levels were elevated 1.9- to 3.3-fold in eosinophilic fasciitis fibroblasts. These results suggest that fibroblasts in the involved skin of patients with eosinophilic fasciitis exhibit an activated phenotype, similar to that of scleroderma fibroblasts, leading to accumulation of collagen in the skin and the underlying structures.

摘要

对两名嗜酸性筋膜炎患者进行了研究,以阐明该疾病中胶原蛋白生成的激活情况。对受累皮肤区域活检标本的组织学检查显示,真皮、皮下组织、筋膜及深层肌肉中有炎性细胞浸润以及不同程度的胶原蛋白积聚。与从同一患者未受累皮肤建立的对照成纤维细胞培养物相比,从受累皮肤起始的成纤维细胞培养物产生的胶原蛋白增加了2.0至3.7倍。嗜酸性筋膜炎成纤维细胞中I型前胶原信使核糖核酸的稳态水平也比对照细胞高2.4至6.2倍,表明I型前胶原基因表达存在翻译前激活。此外,嗜酸性筋膜炎成纤维细胞中细胞纤连蛋白信使核糖核酸的稳态水平升高了1.9至3.3倍。这些结果表明,嗜酸性筋膜炎患者受累皮肤中的成纤维细胞表现出一种激活的表型,类似于硬皮病成纤维细胞,导致皮肤及深层结构中胶原蛋白的积聚。

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