A vascular birthmark known as a port-wine stain is commonly associated with which syndrome?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

A port-wine stain is a type of vascular birthmark that typically appears as a flat, pinkish-red lesion on the skin. It is associated with Sturge-Weber Syndrome, which is a neurocutaneous disorder. This syndrome is characterized by a facial birthmark, often resembling a port-wine stain, that occurs in the distribution of the trigeminal nerve.

Patients with Sturge-Weber Syndrome may also exhibit neurological manifestations such as seizures, developmental delay, and other neurological deficits due to leptomeningeal angiomatosis, where abnormal blood vessels are present in the brain. The presence of a port-wine stain is an important clinical clue that helps in diagnosing this condition.

The association of the port-wine stain with Sturge-Weber Syndrome underscores the need for comprehensive evaluation of patients with such birthmarks, as they may have underlying neurological complications and require interdisciplinary management.

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