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

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

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 α-SMA was done to determine the presence of myofibroblasts, and the reaction was negative. Since α-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.

Angiofibroma; Genetics; Tuberous sclerosis

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