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[结节性硬化症:皮肤血管纤维瘤中肌成纤维细胞的评估——病例报告]

[Tuberous sclerosis: evaluation of myofibroblasts in cutaneous angiofibromas - case report].

作者信息

Perez Erick Gomes, Paranaíba Lívia Ribeiro, Bonan Paulo Rogério, Orsi Júnior Julian Miranda, Oliveira Adriano Macedo de, Martelli Júnior Hercílio

机构信息

Universidade de Alfenas, Brasil.

出版信息

An Bras Dermatol. 2010 Jan-Feb;85(1):84-8. doi: 10.1590/s0365-05962010000100013.

Abstract

Tuberous sclerosis is a rare autosomal dominant disorder. Myofibroblasts are cells with a hybrid phenotype between fibroblasts and smooth muscle cells. The objective of this study is to describe clinical and histopathological characteristics of tuberous sclerosis and to conduct an immunohistochemical evaluation of myofibroblasts in cutaneous angiofibromas present in this condition. Lesion sections removed were stained with hematoxylin-eosin and Masson's trichrome. Immunohistochemistry against alpha-SMA was done to determine the presence of myofibroblasts, and the reaction was negative. Since alpha-SMA is a specific marker for myofibroblasts, this result suggests that myofibroblasts are not involved in cutaneous angiofibromas present in the tuberous sclerosis case reported.

摘要

结节性硬化症是一种罕见的常染色体显性疾病。肌成纤维细胞是具有成纤维细胞和平滑肌细胞混合表型的细胞。本研究的目的是描述结节性硬化症的临床和组织病理学特征,并对该疾病中出现的皮肤血管纤维瘤中的肌成纤维细胞进行免疫组织化学评估。切除的病变切片用苏木精-伊红和马松三色染色法染色。进行抗α-平滑肌肌动蛋白的免疫组织化学检测以确定肌成纤维细胞的存在,结果为阴性。由于α-平滑肌肌动蛋白是肌成纤维细胞的特异性标志物,该结果表明在报道的结节性硬化症病例中,肌成纤维细胞不参与皮肤血管纤维瘤的形成。

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