Gorlin-Goltz syndrome: A rare case report

Abstract

Gorlin-Goltz syndrome (GGS) is a rare genetic disease that is transmitted as an autosomal-dominant trait showing high level of penetrance and varying expressivity affecting multiple systems of the body. Characteristic clinical manifestations include the presence of multiple basal cell carcinomas, odontogenic keratocysts of the jaws, palmar/plantar pits and calcification of falx cerebri. Early diagnosis of GGS is of great importance due to susceptibility of affected individuals to multiple neoplasms of skin and brain (medulloblastoma) in an early age; life expectancy in GGS is not significantly altered, but morbidity from complications can be substantial. Dentist plays a crucial role in early diagnosis, which prevents recurrence and provides better survival rates from the existent diseases.

We are reporting a rare case of GGS in a 14-year-old girl who visited our institution with characteristic clinical, radiological and histological features.