Syndrome in question: Gorlin-Goltz syndrome
An. bras. dermatol
;
91(4): 541-543, July-Aug. 2016. graf
Article
in English
| LILACS
| ID: lil-792451
ABSTRACT
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Skin Neoplasms
/
Basal Cell Nevus Syndrome
Limits:
Adult
/
Humans
/
Male
Language:
English
Journal:
An. bras. dermatol
Journal subject:
Dermatology
Year:
2016
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Hospital Santa Casa de Misericórdia de Vitória/BR
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