Syndrome in question: Gorlin-Goltz syndrome
An. bras. dermatol
;
91(4): 541-543, July-Aug. 2016. graf
Article
Dans Anglais
| 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.
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Tumeurs cutanées
/
Naevomatose basocellulaire
Limites du sujet:
Adulte
/
Humains
/
Mâle
langue:
Anglais
Texte intégral:
An. bras. dermatol
Thème du journal:
Dermatologie
Année:
2016
Type:
Article
Pays d'affiliation:
Brésil
Institution/Pays d'affiliation:
Hospital Santa Casa de Misericórdia de Vitória/BR
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