title: Clinical and oral findings in an Afro-Brazilian family with Gorlin-Goltz syndrome: case series and literature review.
abstract: Gorlin-Goltz syndrome (GGS) seems to be unusual in black persons. The authors present an Afro-Brazilian family case report of GGS. The main complaint of the index case was a painless swelling of the left mandible, which was diagnosed as an odontogenic keratocyst. Further classical features of the Syndrome were present in this patient. Other two family members were diagnosed as cases of GGS and one of them presented 11 clinical findings characteristic of the syndrome. From the three cases reported, two of them presented five major diagnostic criteria for the GGS, and the diagnosis was only made because of an oral complaint. This case series emphasizes the importance of carefully examining the patient and close relatives for signs of GGS, even if they belong to an ethnic group in which this diagnosis is unusual.
authors.affiliation: Department of Pediatric Dentistry, School of Dentistry, Universidade Salgado de Oliveira (UNIVERSO), Rio de Janeiro, Brazil.
authors.forename: Viviane Santos
authors.initials: VS
authors.lastname: da Silva Pierro
authors.forename: Marcello Roter
authors.initials: MR
authors.lastname: Marins
authors.forename: Renata Cabral
authors.initials: RC
authors.lastname: Borges de Oliveira
authors.forename: Wladimir
authors.initials: W
authors.lastname: Cortezzi
authors.forename: Maria Elisa
authors.initials: ME
authors.lastname: Janini
authors.forename: Lucianne Cople
authors.initials: LC
authors.lastname: Maia
mesh_terms: D000293:Adolescent; D000328:Adult; D000349:Africa / Q000208:ethnology; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis* / Q000208:ethnology* / Q000235:genetics; D001938:Brazil; D002648:Child; D003952:Diagnostic Imaging; D005260:Female; D006801:Humans; D008297:Male
keywords: African continental ancestry group; basal cell nevus syndrome; diagnosis; neoplasms; odontogenic cysts