Syndrome in question: Gorlin-Goltz syndrome.

Autor:	Ribeiro PL; Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil., Souza JB Filho; Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil., Abreu KD; Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil., Brezinscki MS; Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil., Pignaton CC; Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil.
Jazyk:	angličtina
Zdroj:	Anais brasileiros de dermatologia [An Bras Dermatol] 2016 Jul-Aug; Vol. 91 (4), pp. 541-3.
DOI:	10.1590/abd1806-4841.20164428
Abstrakt:	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. Competing Interests: None.
Databáze:	MEDLINE
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