Port-wine stain

Alternative names
Nevus flammeus

Definition

A port-wine stain is a vascular birthmark 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.

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 such as Sturge-Weber syndrome or Klippel-Trenaunay-Weber syndrome.

Symptoms

     
  • lesion is usually present at birth  
  • newborn infant with a flat pink to purplish lesion on skin  
  • most commonly seen on face and neck (but may occur on trunk or extremities)  
  • older child with a slightly more reddish to purplish lesion, flat to slightly raised  
  • adult with darkly red to purplish lesion

Signs and tests

Your physician 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 associated symptoms, your physician may choose to 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 cutaneous capillaries 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.

Johns Hopkins patient information

Last revised: December 5, 2012
by David A. Scott, M.D.

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