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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 .
Sturge-Weber syndrome
Sturge-Weber syndrome is a rare disorder that is present at birth. A child with this condition will have a port-wine stain birthmark (usually on the...
Klippel-Trenaunay-Weber syndrome
Klippel-Trenaunay syndrome is a rare condition that is typically present at birth. The syndrome often involves port wine stains, excess growth of bo...
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 .
Intraocular pressure
Tonometry is a test to measure the pressure inside your eyes. The test is used to screen for glaucoma.
x-ray of the skull
A skull x-ray is a picture of the bones surrounding the brain, including the facial bones, the nose, and the sinuses.
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 .
Glaucoma
Glaucoma is a group of eye conditions that can damage the optic nerve. This nerve sends the images you see to your brain. Most often, optic nerve da...
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.
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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
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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 - 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 - 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: 4/14/2015
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.
