Syndrome in question: Gorlin-Goltz syndrome



Título del documento: Syndrome in question: Gorlin-Goltz syndrome
Revista: Anais brasileiros de dermatologia
Base de datos: PERIÓDICA
Número de sistema: 000400650
ISSN: 0365-0596
Autores: 1
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Instituciones: 1Hospital Santa Casa de Misericordia de Vitoria, Vitoria, Espirito Santo. Brasil
Año:
Periodo: Jul-Ago
Volumen: 91
Número: 4
Paginación: 541-543
País: Brasil
Idioma: Inglés
Tipo de documento: Artículo
Enfoque: Caso clínico, descriptivo
Resumen en inglés 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
Disciplinas: Medicina
Palabras clave: 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
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