Abstract
Objective: To perform a literature review on the correlation between port-wine stain (PWS) and Sturge-Weber Syndrome (SWS). Methods: This is an integrative review study through the selection of journals in the PubMed electronic database between 2002 and 2019. We used the keywords “weber sturge syndrome”, “stain port wine” and “dermatology”. Results/Discussion: SWS is a rare, sporadic, congenital neurocutaneous syndrome characterized by cutaneous capillary malformations, cerebral venous capillary malformations and glaucoma. PWS results from somatic mutations in the GNAQ gene during embryonic development. It was found that PWS in the “forehead” area had greater significance for SWS development. In addition, the wider distribution of PWS, being unilateral extending to the maxilla and mandible, as well as bilateral presentation, are considered risk factors. Final considerations: The characteristics of the dermatological presentation of PWS determine whether there is a greater predisposition to SWS. Therefore, early clinical diagnosis is required due to compliance of PWS associated with magnetic resonance imaging results. This practice alerts professionals about the neurological symptoms and complications of patients with the syndrome.