Port-wine stain

Definition

A port-wine stain is a vascular (containing vessels) birthmark made of enlarged capillaries in the skin, which produce a reddish-purplish discoloration of the skin.

Causes, incidence, and risk factors

Port-wine stains (PWS) are present at birth. The rate 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.

PWS may cause emotional and social problems for the affected person because of their 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, perhaps as part of a syndrome such as Sturge-Weber syndrome or Klippel-Trenaunay-Weber syndrome.

Symptoms

• Usually present at birth
• Is usually a flat pink-to-purplish lesion on skin in infants
• Most commonly seen on face and neck (but may occur on trunk or extremities)
• Is usually slightly more reddish-to-purplish lesion, flat to slightly raised in older children
• Is usually darkly red-to-purplish lesion in adults

Signs and tests

Your doctor can usually diagnose a port wine stain based entirely upon its appearance. In unusual cases, a skin biopsy may be needed to confirm the diagnosis. Depending on the location of the birthmark and other symptoms, your doctor may order a measurement of intraocular pressure or x-ray of the skull.

Treatment

Many treatments have been tried for port-wine stains including freezing, surgery, radiation, and tattooing. Lasers have made the biggest impact on treatment, because they are the sole method of destroying the tiny blood vessels in the skin without significant damage to the overlying skin.

The flashlamp pumped dye laser, a yellow light laser, has been the most successful at destroying stains in infants and young children. Two other yellow light lasers -- the copper vapor and krypton laser -- have been used successfully in adults. The neodymium-YAG laser is used to treat thick, nodular, deep purple port-wine stains.

Support Groups

National Congenital Port-Wine Stain Foundation, 125 East 63rd Street, New York, NY 10021.

Expectations (prognosis)

Treatment of infants with the flashlamp pumped dye laser generally produces marked improvement in appearance. Stains on the face respond better than those on the trunk or limbs. Older stains may be more difficult to treat.

Complications

Hypertrophy (increased tissue mass) of the stains may occasionally produce deformity and increasing disfigurement.

Calling your health care provider

All birthmarks should be evaluated by the health care provider during a routine examination.

Illustrations

Port wine stain on a child's face

Sturge-Weber syndrome - legs

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