DOI: 10.36347/sjds.2020.v07i05.001

Pages: 74-77

Tuberous sclerosis is a neurocutaneous syndrome with an autosomal dominant inheritance. Tuberous sclerosis has an approximate incidence of one in ten thousand to fifty thousand. Tuberous sclerosis complex Syndrome (TSCs) is a dominantly inherited disorder affecting multiple organs; caused by mutations of either the TSC1 or TSC2 gene encoding hamartin and tuber in respectively. It is characterized by the development of benign tumors affecting different body systems. The most common oral manifestations of TSC are fibromas (angiofibromas), gingival hyperplasia and enamel hypoplasia and the formation of hamartomas in multiple organ systems leading to morbidity and mortality. It is important to make an early diagnosis of TSC so that lifelong monitoring, early recognition of complications and proactive treatment can lower the morbidity and mortality rates.