Art of port wine birthmark: a case report on type 2 Sturge–Weber Syndrome

Abstract

Sturge–Weber syndrome is a phakomatosis group of neurocutaneous disorders. They manifest in the dermal, neural, ocular, and oral regions because of a mutation in the guanine nucleotide-binding protein G(q) subunit alpha gene. These lesions are often unilateral, following the course of the trigeminal nerve. Here, we present a case of a 54-year-old woman with a history of glaucoma with a unilateral reddish–purple discoloration of the palate and a port-wine stain limited to the left side of her face. The clinical course of Sturge–Weber syndrome is complex and requires a multidisciplinary approach. In type 2 cases of Sturge–Weber syndrome, patients present with facial angiomas and glaucoma, but without neurological involvement. There is an increased risk of hemorrhage associated with Sturge–Weber syndrome, which makes it essential to carefully plan dental procedures to minimize any potential pre and postoperative bleeding risks. This case report aims to highlight the importance of recognizing similar patterns to enable prompt diagnosis.