A Case of Gorlin-Goltz Syndrome Presented With Multiple Odontogenic Keratocysts in the Jaw Without Skin Manifestation

Abstract

Gorlin-Goltz syndrome is a hereditary autosomal dominant condition with high penetrance and varied phenotypic expressiveness that can appear spontaneously. It is estimated that between 30% and 50% of people with this disease do not know if any of their family members have had it. Patched (PTCH), a tumor suppressor gene found on the 9q22.3 chromosome, has been identified as the cause of Gorlin-Goltz syndrome. This case emphasizes the necessity of awareness of this uncommon illness in young people who do not have any skin blemishes. Due to the severity of clinical manifestations, early identification of the illness and a long follow-up time are critical. Furthermore, a multidisciplinary team consisting of a dentist, dermatologist, geneticist, and neurologist, is necessary to improve overall survival rates. Gorlin-Goltz syndrome is inherited as an autosomal dominant disease. In almost 50% of cases, many people do not know whether they have a positive family history. It is not always present with basal cell epitheliomas or skin manifestation.