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Port-wine stain

Definition

A port-wine stain is a birthmark in which swollen blood vessels create a reddish-purplish discoloration of the skin.

Causes

Port-wine stains occur in about 3 out of 1,000 people.

In rare cases, port-wine stains are a sign of Sturge-Weber syndrome or Klippel-Trenaunay-Weber syndrome.

Symptoms

Early port-wine stains are usually flat and pink in appearance. As the child gets older, the color may deepen to a dark red or purplish color. They occur most often on the face but can appear anywhere on the body. Over time, the area can become thickened and take on a cobblestone-like appearance.

Exams and Tests

Your doctor can usually diagnose a port wine stain by looking at the skin.

In unusual cases, a skin biopsy may be needed. Depending on the location of the birthmark and other symptoms, your doctor may want to do an intraocular pressure test or x-ray of the skull.

An MRI or CT scan of the brain may also be done.

Treatment

Many treatments have been tried for port-wine stains, including freezing, surgery, radiation, and tattooing.

Laser therapy is most successful in eliminating port-wine stains. It is the only method that can destroy the tiny blood vessels in the skin without significantly damaging the skin. The exact type of laser used depends on the person's age and particular port-wine stain.

Outlook (Prognosis)

Stains on the face respond better to laser therapy than those on the arms, legs, or middle of the body. Older stains may be more difficult to treat.

Possible Complications

Some stains may occasionally cause deformity and increasing disfigurement.

People with port-wine stains may have emotional and social problems related to their appearance.

Port-wine stains that involve the upper and lower eyelids may be associated with the development of glaucoma.

Neurologic problems are present when port-wine stain is associated with a disorder such as Sturge-Weber syndrome.

When to Contact a Medical Professional

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

References

Enjolras O. Vascular malformations. In: Bolognia JL, Jorizzo JL, Schaffer JV, eds. Dermatology. 3rd ed. Philadelphia, Pa: Elsevier Saunders; 2012:chap 104.

Metz BJ, Eichenfield LF. Port wine stains. In: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson I. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 3rd ed. Philadelphia, Pa: Elsevier Saunders; 2009:chap 187.

Morelli JG. Vascular disorders. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 642.


Review Date: 5/15/2013
Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
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