[Gorlin-Goltz syndrome with odontogenic keratosis. Report on a patient followed for 10 years].
A patient with Gorlin-Goltz syndrome (nevoid basal cell carcinoma syndrome) is described. This disease has an autosomal dominant inheritance pattern with complete penetrance and extremely variable expressivity. The case report seems to represent a new mutation. Gorlin-Goltz syndrome is characterized by a lot of symptoms primarily involving the skin, central nervous system, and skeletal system. In 90% of the patients, nevoid basal cell carcinoma syndrome is associated with recurring odontogenic keratocysts. Also our patient showed recurrent jaw and maxillary cysts, for this reasons he has been followed for 10 years to the Oral Pathology Service of Galliera Hospital.
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Servizio di Odontostomatologia, Ospedali Galliera di Genova, Università degli Studi di Genova.
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D001478:Basal Cell Nevus Syndrome / Q000628:therapy; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D007972:Leukoplakia, Oral / Q000628:therapy; D008297:Male; D009062:Mouth Neoplasms / Q000628:therapy; D009378:Neoplasms, Multiple Primary / Q000628:therapy; D012878:Skin Neoplasms / Q000628:therapy
Sindrome di Gorlin-Goltz con cheratocisti odontogeniche. Presentazione di un paziente seguito per 10 anni.