title: Oral mucosal manifestations in some genodermatoses: correlation with cutaneous lesions.
abstract: The 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.
authors.affiliation: Department of Dermatology, University of São Paulo- Brazil, Rua Itapeva 500, CEP-01332-000 São Paulo-Brazil.
authors.forename: Marcello Menta Simonsen
authors.initials: MM
authors.lastname: Nico
authors.forename: Mariana
authors.initials: M
authors.lastname: Hammerschmidt
authors.forename: Silvia Vanessa
authors.initials: SV
authors.lastname: Lourenço
mesh_terms: D007644:Darier Disease / Q000235:genetics / Q000473:pathology; D019871:Dyskeratosis Congenita / Q000235:genetics / Q000473:pathology; D006223:Hamartoma Syndrome, Multiple / Q000235:genetics / Q000473:pathology; D006801:Humans; D008065:Lipoid Proteinosis of Urbach and Wiethe / Q000235:genetics / Q000473:pathology; D009059:Mouth Diseases / Q000473:pathology*; D009061:Mouth Mucosa / Q000473:pathology*; D053549:Pachyonychia Congenita / Q000235:genetics / Q000473:pathology; D012873:Skin Diseases, Genetic / Q000235:genetics / Q000473:pathology*; D014076:Tooth Diseases / Q000473:pathology; D014402:Tuberous Sclerosis / Q000235:genetics / Q000473:pathology*
keywords: Cowden disease; Darier's disease; dyskeratosis congenita; lipoidoproteinosis; oral lesions; pachyonychya congenita; tuberous sclerosis