Port-wine stain

Overview
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. It is part of the family of disorders known as vascular malformations.

Background
Port-wine stains are present at birth and persist throughout life. The area of skin affected grows in proportion to general growth. 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. In adulthood, thickening of the lesion or the development of small lumps may occur.

The presence of PWS can cause emotional and social problems for the affected person because of their cosmetic appearance (see disfigurement).

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. Hypertrophy (increased tissue mass) of the stains may occasionally produce deformity and increasing disfigurement. Nevertheless, all birthmarks should be evaluated by the health care provider during a routine examination.

Epidemiology
The incidence is 3 out of 1,000 people.

Diagnosis
A physician can usually diagnose a port wine stain based entirely upon the history and 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.

Treatment
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. The neodymium YAG laser is used to treat the nodules that may develop in some adult port-wine stains.

Treatment of infants with the flashlamp pumped dye laser generally produces marked improvement in appearance though complete disappearance is rare. Unfortunately, in a small percentage of cases (approximately 20%) there may be no improvement at all. Stains on the face respond better than those on the trunk or limbs. Older stains may be more difficult to treat.