| Journal: | Anais brasileiros de dermatologia |
| Database: | PERIÓDICA |
| System number: | 000400650 |
| ISSN: | 0365-0596 |
| Authors: | Ribeiro, Pauline Lyrio1 Souza-Filho, Joao Basilio de1 Abreu, Karina Demoner de1 Brezinscki, Marisa Simon1 Pignaton, Christine Chambo1 |
| Institutions: | 1Hospital Santa Casa de Misericordia de Vitoria, Vitoria, Espirito Santo. Brasil |
| Year: | 2016 |
| Season: | Jul-Ago |
| Volumen: | 91 |
| Number: | 4 |
| Pages: | 541-543 |
| Country: | Brasil |
| Language: | Inglés |
| Document type: | Artículo |
| Approach: | Caso clínico, descriptivo |
| English abstract | The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an uncommon disorder caused by a mutation in Patched, tumor suppressor gene. It is mainly characterized by numerous early onset basal cell carcinomas, odontogenic cysts of jaw and skeletal abnormalities. Due to the wide clinical spectrum, treatment and management of its modalities are not standardized and should be individualized and monitored by a multidisciplinary team. We report a typical case in a 30-year-old man with multiple basal cell carcinomas, keratotic pits of palmar creases and bifid ribs, with a history of several corrective surgeries for keratocystic odontogenic tumors, among other lesions characteristic of the syndrome |
| Disciplines: | Medicina |
| Keyword: | Dermatología, Diagnóstico, Síndrome de Gorlin-Goltz, Carcinoma basocelular, Síndrome de nevo basocelular, Quiste odontogénico |
| Keyword: | Medicine, Dermatology, Diagnosis, Gorlin-Goltz syndrome, Basal cell carcinoma, Basal cell nevus syndrome, Odontogenic cyst |
| Full text: | Texto completo (Ver HTML) |
