Port Wine Stain

Alternative names:

flat hemangioma

Definition:

A vascular birthmark (nevus) consisting of superficial and deep dilated capillaries in the skin which produce a reddish to purplish discoloration of the skin.

Causes, incidence, and risk factors:

Port wine stains (PWS) are present at birth. The incidence is 3 out of 1,000 people. Port wine stains occur most often on the face but can appear anywhere on the body. Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish color. Over time, a PWS may hypertrophy (increased tissue mass) becoming raised, nodular, and grape-like in appearance. Significant hypertrophy may produce distortion of the facial features.

The presence of PWS can cause emotional and social problems for the affected person because of their cosmetic appearance. Port wine stains that involve the upper and lower lids (trigeminal distribution) may be associated with the development of glaucoma.

PWS may be one of a group of symptoms and signs in which case it is considered to be part of a syndrome. Several of these syndromes may include:

• Sturge-Weber syndrome
• Klippel-Trenaunay-Weber syndrome
• Rubinstein-Taybi syndrome