Gorlin syndrome associated with basal cell carcinoma of the vulva: A case report.

Abstract

Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is a hereditary condition transmitted as an autosomal dominant trait with high penetrance and variable expressivity. The syndrome is characterized by numerous manifestations: basal cell carcinomas (BCCs) and odontogenic keratocysts (OKCs) are the leading ones. In this article a typical Gorlin syndrome case associated with basal cell carcinoma of the vulva is described.