Müller-Felber W, Toepfer M, Müller T, Müller-Höcker J, Fischer P, Lochmüller H, Pongratz D
Friedrich-Baur-Institut, University of Munich, Ziemssenstr. 1a, D-80336 Munich, Germany.
Eur J Med Res. 1998 Jun 17;3(6):281-7.
The expression of tenascin, NCAM, vimentin and HSP-70 was examined in 15 patients with dermatomyositis and 12 patients with polymyositis. All biopsies with dermatomyositis showed a confluent network of tenascin expression in the extracellular space. This network surrounded numerous fibers (39 up to 320). Tenascin expression preceded other histological and immunohistochemical signs of dermatomyositis such as the expression of N-CAM and vimentin and the appearance of perifascicular atrophy and inflammatory response. Tenascin expression was found in 3 biopsies where microtubular inclusions in the arteriolar walls were absent. In contrast, tenascin expression was confined to the surroundings of necrotic fibers also expressing vimentin or N-CAM in polymyositis. In general, tenascin was found in the vicinity of inflammatory cells, only. These findings suggest that tenascin is induced by ischemic stress in dermatomyositis. Widespread tenascin expression in a fascicular distribution is a specific marker, if an inflammatory myopathy has been proven by standard techniques. In some cases, tenascin staining might confirm the clinical diagnosis of dermatomyositis, where other histological findings are inconclusive.
在15例皮肌炎患者和12例多发性肌炎患者中检测了腱生蛋白、神经细胞黏附分子(NCAM)、波形蛋白和热休克蛋白-70(HSP-70)的表达。所有皮肌炎活检标本均显示细胞外间隙中有融合的腱生蛋白表达网络。该网络围绕着大量纤维(39根至320根)。腱生蛋白的表达先于皮肌炎的其他组织学和免疫组化特征,如N-CAM和波形蛋白的表达以及束周萎缩和炎症反应的出现。在3例小动脉壁无微管包涵体的活检标本中发现了腱生蛋白表达。相比之下,在多发性肌炎中,腱生蛋白表达局限于也表达波形蛋白或N-CAM的坏死纤维周围。一般来说,仅在炎症细胞附近发现腱生蛋白。这些发现表明,腱生蛋白在皮肌炎中是由缺血应激诱导产生的。如果通过标准技术已证实为炎性肌病,那么呈束状分布的广泛腱生蛋白表达是一个特异性标志物。在某些情况下,当其他组织学发现不明确时,腱生蛋白染色可能有助于皮肌炎的临床诊断。