Port Wine Stain (Nevus Flammeus)

A port wine stain, or nevus flammeus (NEE-vus fla-MEE-us), is a vascular birthmark or a malformation of the capillaries that is present at birth. It usually appears on the face, neck, scalp, arms, or legs.

What Is Port Wine Stain?

A port wine stain, also called nevus flammeus, is a vascular birthmark or a malformation of the capillaries that is present at birth. It is a flat pink mark on the skin when the baby is born. As the child grows the color changes to a dark red or purple color, the color of grape juice or a type of red wine called port. The size of the birthmark varies, but it usually grows as the child grows. In adulthood, the birthmark may develop a thickened and raised cobblestone-like texture. The birthmark commonly appears on the face, neck, scalp, arms or legs.




Port-wine stain shown on the side of the face, under the ear. It is a birthmark in which swollen blood vessels create a reddish-purplish discoloration of the skin.





Port-wine stain shown on the side of the face, under the ear. It is a birthmark in which swollen blood vessels create a reddish-purplish discoloration of the skin.

How Common Is Port Wine Stain?

Although uncommon, port wine stain is the most common type of blood vessel malformation observed. Approximately 1 of every 300 babies has a port wine stain birthmark. In the United States, less than one-half of 1 percent of newborns are born with capillary malformation; global incidence is similarly rare.

What Is the Cause of Port Wine Stain?

Famous people who have a birthmark:

How Is Port Wine Stain Diagnosed and Treated?

Diagnosis

After getting a complete health history, the healthcare provider makes the diagnosis of port wine stain based on information obtained and observation of the skin. An important part of evaluating a person with port wine stain is to determine if there are any associated complications. For example, port wine stains located on the upper and lower eyelids are sometimes associated with the development of glaucoma * . Testing the eyes for glaucoma by checking the intraocular pressure * would be an appropriate procedure for a person with a port wine stain on the eyelids.

In some cases, a skin biopsy * may be done to determine if the discoloration is related to some other skin problem. In some cases, an x-ray, computed tomography (CT) * scan, or magnetic resonance imaging (MRI) * may be done if there is concern that the port wine stain is associated with a rare neurological condition called Sturge-Weber syndrome * .

One of the most important things to determine is the psychological effect the birthmark may have on the child or adult. For many children the port wine stain is a part of who they are and is of little or no concern. For some children, the birthmark is concerning, especially if it is on the face. How people react to the birthmark may affect how the child feels about himself or herself.

Treatment

Various treatments have been tried to remove port wine stains, including freezing, surgery, radiation, and tattooing. The most common and most successful treatment has been removal of the birthmark with laser therapy. The laser treatment destroys the tiny blood vessels that create the birthmark without damaging the skin. Laser treatment is more effective if it is started early, before the port wine stain grows and darkens with age. Some clinicians recommend treatment with laser therapy be started as soon as one to two weeks after birth.

For women who have port wine stains on the face, learning how to use cosmetics to cover or camouflage the birthmark can be helpful.

Can Port Wine Stain Be Prevented?

There is no prevention for port wine stain. Port wine stain is not caused by something the mother did or did not do during pregnancy. For example, having a desire for a specific food throughout pregnancy does not cause port wine stain.

See also Glaucoma • Pregnancy • Skin Conditions: Overview • Vascular Diseases: Overview

Resources

Books and Articles

Goodman, Brenda. “Cause of Port-Wine Stain Birthmarks Pinpointed.” WebMD.com . May 8, 2013. http://www.webmd.com/skinproblems-and-treatments/news/20130508/researchers-pinpointcause-of-port-wine-stain-birthmarks (accessed April 1, 2016).

Wolff, Klaus, Richard Johnson, and Arturo Saavedra. Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology, 7th ed. New York: McGraw-Hill Education, 2013.

Websites

MedlinePlus. “Port-Wine Stain.” U.S. National Library of Medicine, National Institutes of Health. https://www.nlm.nih.gov/medlineplus/ency/article/001475.htm (accessed April 1, 2016).

Morales, Tatiana. “Living with a Birthmark.” http://www.cbsnews.com/news/living-with-a-birthmark (accessed April 1, 2016).

Organizations

Birthmark Support Group. BM The Birthmark Support Group, London, WC1N3XX, UK. Telephone: 44-0-7825-855-888. Website: http://www.birthmarksupportgroup.org.uk (accessed April 1, 2016).

Vascular Birthmarks Foundation. PO Box 106, Latham, NY 12110. Toll-free: 877-823-4646. Website: https://birthmark.org (accessed April 1, 2016).

* glaucoma (glau-KO-ma) is a group of disorders that cause pressure to build within the eye, which may result in loss of vision.

* intraocular pressure (in-traAWK-q-lar) is the fluid pressure inside the eye and is an important aspect in the evaluation of patients at risk from glaucoma.

* biopsy (BI-op-see) is a test in which a small sample of skin or other body tissue is removed and examined for signs of disease.

* computed tomography (kom-PYOO-ted toe-MAH-gruh-fee), or CT, is a technique in which a machine takes many x-rays of the body to create a three-dimensional picture. Formerly called computerized axial tomography (CAT).

* magnetic resonance imaging, or MRI, uses magnetic waves, instead of x-rays, to scan the body and produce detailed pictures of the body's structures.

* Sturge-Weber syndrome is a neurological disease present at birth characterized by a port wine stain, seizures, and delay in development.

Disclaimer:   This information is not a tool for self-diagnosis or a substitute for professional care.

(MLA 8th Edition)