Port-wine stain
Nevus flammeusA 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.
Sturge-Weber syndrome
Sturge-Weber syndrome (SWS) is a rare disorder that is present at birth. A child with this condition will have a port-wine stain birthmark (usually ...
Klippel-Trenaunay-Weber syndrome
Klippel-Trenaunay syndrome (KTS) is a rare condition that is typically present at birth. The syndrome often involves port wine stains, excess growth...
Read Article Now Book Mark ArticleSymptoms
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
Your health care provider can usually diagnose a port-wine stain by looking at your 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.
Intraocular pressure
Tonometry is a test to measure the pressure inside your eyes. The test is used to screen for glaucoma. It is also used to measure how well 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 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
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...
ImageRead Article Now Book Mark Article - 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
Dinulos JGH. Vascular tumors and malformations. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 23.
Kelly KM, Arkin L, Baugh EG, Tran JM. Laser treatment of vascular lesions. In: Tanzi EL, Dover JS, eds. Procedures in Cosmetic Dermatology: Lasers, Lights, and Energy Devices. 5th ed. Philadelphia, PA: Elsevier; 2023:chap 2.
Moss C, Browne F. Mosaicism and linear lesions. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 62.
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
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: 6/7/2023
Reviewed By: Elika Hoss, MD, Assistant Professor of Dermatology, Mayo Clinic, Scottsdale, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
All rights reserved.