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A port-wine stain or naevus flammeus is a vascular birthmark consisting of superficial and deep dilated capillaries in the skin which produce a reddish to purplish discoloration of the skin. They are so called for their colour, resembling that of Port wine.
Port-wine stains 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 (see disfigurement). 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.
The lesion is usually present at birth, presenting as a flat pink to purplish lesion on the newborn's skin. It is most commonly seen on face and neck, but may occur on the trunk or extremities. In an older child, the lesion may be with a slightly more reddish to purplish, and flat to slightly raised. In adults, it may become darkly red to purplish.
A 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, a physician may choose to order a measurement of intraocular pressure or X-ray of the skull.
Many treatments have been tried for port-wine stains including freezing, surgery, radiation, and tattooing; port-wine stains can also be covered with cosmetics. 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.
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.
Hypertrophy (increased tissue mass) of the stains may occasionally produce deformity and increasing disfigurement.
All birthmarks should be evaluated by the health care provider during a routine examination.
The most famous person with a port-wine stain is Mikhail Gorbachev, whose birthmark is located on his forehead.
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