Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSPNICO, Marcello Menta SimonsenHAMMERSCHMIDT, MarianaLOURENCO, Silvia Vanessa2014-01-282014-01-282013EUROPEAN JOURNAL OF DERMATOLOGY, v.23, n.5, p.581-591, 20131167-1122https://observatorio.fm.usp.br/handle/OPI/4031The clinical picture of several genetic skin diseases may include the presence of oral mucosal lesions. These manifestations, however, have not been granted much attention in most dermatological publications. In this article, we fully review the oral mucosal lesions of tuberous sclerosis, dyskeratosis congenita, lipoidoproteinosis, Cowden disease, Darier's disease and pachyonychya congenita and compare these with their respective cutaneous lesions. Some dental aspects are discussed as well. This unifying approach may allow a better understanding of these oral lesions, avoiding obscure nomenclature and classification.engrestrictedAccessoral lesionstuberous sclerosislipoidoproteinosisCowden diseaseDarier's diseasepachyonychya congenitamultiple hamartoma syndromeof-the-literaturetuberous sclerosis complexlipoid proteinosispachyonychia-congenitadyskeratosis-congenitacowdens-syndromedariers diseasefeaturesinvolvementarticleCopyright JOHN LIBBEY EUROTEXT LTD10.1684/ejd.2013.2073Dermatology