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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 are caused by an abnormal formation of tiny blood vessels in the skin.

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 stain grows with the child and the color may deepen to dark red or purple. Port-wine stains 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 of the eye 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 removing port-wine stains. It is the only method that can destroy the tiny blood vessels in the skin without causing much damage to the skin. The exact type of laser used depends on the person's age, skin type, 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

Complications may include:

  • Deformity and increasing disfigurement
  • Emotional and social problems related to their appearance
  • Development of glaucoma in people with port-wine stains involving upper and lower eyelids
  • Neurologic problems 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 provider during a routine examination.

References

Cheng N, Rubin IK, Kelly KM. Laser treatment of vascular lesions. In: Hruza GJ, Tanzi EL, Dover JS, Alam M, eds. Lasers and Lights: Procedures in Cosmetic Dermatology. 3rd ed. Philadelphia, PA: Elsevier Saunders; 2018:chap 2.

Habif TP. Vascular tumors and malformations. In: Habif TP, ed. Clinical Dermatology. 6th ed. Philadelphia, PA: Elsevier; 2016:chap 23.

Moss C, Browne F. Mosaicism and linear lesions. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier Saunders; 2018:chap 62.

  • 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


     

    Review Date: 4/16/2019

    Reviewed By: Michael Lehrer, MD, Clinical Associate Professor, Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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