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Port-wine stain

Nevus flammeus

 

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-stage port-wine stains are usually flat and pink. 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

 

The health care provider can usually diagnose a port wine stain by looking at the skin.

In a few cases, a skin biopsy is needed. Depending on the location of the birthmark and other symptoms, the provider 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.

Krakowski AC, Eichenfield LF. Port wine stain ('nevus flammeus'). In: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson I. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 193.

Martin KL. Vascular disorders. In: Kliegman RM, Stanton BF, St. Geme, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 650.

 
  • Port wine stain on a child's face

    Port wine stain on a child's face - illustration

    Port wine stains are always present at birth. In an infant, they are flat, pink, vascular lesions. Common locations include the face and neck, but they may be present anywhere on the body. Port wine stains may appear in association with other syndromes.

    Port wine stain on a child's face

    illustration

  • Sturge-Weber syndrome - legs

    Sturge-Weber syndrome - legs - illustration

    Sturge-Weber syndrome is a disease that affects the skin and nervous system (neurocutaneous) and is associated with Port Wine Stain, red vascular markings on the face and other parts of the body (shown here on the legs). This is an unusual case, due to the large size of the lesion (extensive involvement). Occasionally seizures or learning disorders are also associated with Sturge-Weber syndrome.

    Sturge-Weber syndrome - legs

    illustration

    • Port wine stain on a child's face

      Port wine stain on a child's face - illustration

      Port wine stains are always present at birth. In an infant, they are flat, pink, vascular lesions. Common locations include the face and neck, but they may be present anywhere on the body. Port wine stains may appear in association with other syndromes.

      Port wine stain on a child's face

      illustration

    • Sturge-Weber syndrome - legs

      Sturge-Weber syndrome - legs - illustration

      Sturge-Weber syndrome is a disease that affects the skin and nervous system (neurocutaneous) and is associated with Port Wine Stain, red vascular markings on the face and other parts of the body (shown here on the legs). This is an unusual case, due to the large size of the lesion (extensive involvement). Occasionally seizures or learning disorders are also associated with Sturge-Weber syndrome.

      Sturge-Weber syndrome - legs

      illustration

    A Closer Look

     

      Self Care

       

        Tests for Port-wine stain

         

           

          Review Date: 4/14/2015

          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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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