S�ndrome de Brooke-Spiegler: presentaci�n de caso cl�nico

espanolEl sindrome de Brooke-Spiegler (SBS) es una genodermatosis poco frecuente, herencia autosomica dominante, con penetrancia y expresividad variables, que predispone a la produccion neoplasias anexiales. En clinica se caracteriza por aparicion simultanea progresiva cilindromas, tricoepiteliomas y, en ocasiones, espiradenomas. El SBS produce mutaciones el gen CYLD1 localizado cromosoma 16 forma parte del cutaneo CYLD, junto cilindromatosis familiar tricoepitelioma multiple familiar. este articulo presenta caso mujer antecedente carcinoma tiroideo anacusia, afectada esta rara genodermatosis. EnglishBrooke-Spiegler’s syndrome is rare genodermatosis, of autosomal dominant inheritance. It presents variable penetrance and expressiveness which predisposes the production adnexal neoplasms. Clinically it characterized by simultaneous progressive appearance cylindromas, trichoepitheliomas and, occasionally, spiradenomas. produced mutations in gene located on chromosome part CYLD skin with familial cylindromatosis trichoepithelioma. Herein, we report case patient history thyroid anacusis, affected this