Tuberous Sclerosis: Evaluation Of Myofibroblasts In Cutaneous Angiofibromas - Case Report [esclerose Tuberosa: Avaliação De Miofibroblastos Em Angiofibromas Cutâneos - Relato De Caso]

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. ©2010 by Anais Brasileiros de Dermatologia.8518488Jó-Wiak, S., Schwartz, R.A., Janniger, C.K., Micha-owicz, R., Chmielik, J., Skin lesions in children with tuberous sclerosis complex: Their prevalence, natural course and diagnostic significance (1998) Int J Dermatol, 37, pp. 911-917Louis, D.N., Von Deimling, A., Hereditary tumor syndromes of the nervous system: Overview and rare syndromes (1995) Brain Pathol, 5, pp. 145-151Kandt, R.S., Tuberous sclerosis complex and neurofibromatosis type 1: The two most common neurocutaneous diseases (2002) Neurol Clin, 20, pp. 941-964MacCollin, M., Kwiatkowski, D., Molecular genetic aspects of the phakomatoses: Tuberous sclerosis complex and neurofibromatosis 1 (2001) Current Opinion in Neurology, 14 (2), pp. 163-169. , DOI 10.1097/00019052-200104000-00005Short, M.P., Adams, R.D., Neurocutaneous diseases (1993) Dermatology in General Medicine, pp. 2249-2290. , Fitzpatrick T, Eisen AZ, Wolff K, Freedberg IM, Austen KR, editors. 4th ed. New York: McGraw-HillGabbiani, G., The biology of the myofibroblasts (1992) Kidney International, 41, pp. 530-532Bitu, C.C., Sobral, L.M., Kellermann, M.G., Martelli-Junior, H., Zecchin, K.G., Graner, E., Coletta, R.D., Heterogeneous presence of myofibroblasts in hereditary gingival fibromatosis (2006) Journal of Clinical Periodontology, 33 (6), pp. 393-400. , DOI 10.1111/j.1600-051X.2006.00928.xWillis, B.C., DuBois, R.M., Borok, Z., Ephitelial origin of myofibroblasts during fibrosis in the lung (2006) Proc Am Thorac Soc, 3, pp. 377-382Bozzo, L., Machado, M.A.N., Almeida, O.P., Lopes, M.A., Coletta, R.D., Hereditary gingival fibromatosis: Report of three cases (2000) J Clin Pediatr Dent, 25, pp. 41-46Almeida Jr., H.L., Dermatologia comparative (2005) An Bras Dermatol, 80, pp. 307-308Berbet, A., Fernandes, C.A.A., Esclerose tuberosa (1997) An Bras Dermatol, 72, pp. 73-77Desmouliére, A., Chaponnier, C., Gabbiani, G., Tissue repair, contraction, and myofibroblasts (2005) Wound Repair Regen, 13, pp. 7-12Smith, P.C., Caceres, M., Martinez, J., Induction of the myofibroblastic phenotype in human gingival fibroblasts by transforming growth factor-beta1: Role of RhoA-ROCK and c-Jun N-terminal kinase signaling pathways (2006) Journal of Periodontal Research, 41 (5), pp. 418-425. , DOI 10.1111/j.1600-0765.2006.00886.xGomez, M.R., (1979) Tuberous Sclerosis, , New York: Raven Pres