Port-wine stain: symptoms, causes & treatment port-wine stain on skin

A port-wine stain is a congenital, benign vascular abnormality characterized by over-expanded blood vessels in the skin. The medical name for a port-wine stain is ‘nevus flammeus’. A port-wine stain is a congenital and normally benign skin defect. Due to an excessive growth of blood vessels on the surface of the skin, a reddish purple discoloration of the skin occurs. A port-wine stain does not spontaneously regress, as a ‘stork bite’ does. Treatment of a port wine stain consists of camouflage and/or laser therapy. In the past, various methods have been used to remove a wine stain, but removing a wine stain by laser has proven to be the most effective.

  • What is a port wine stain on the skin?
  • Synonyms
  • Prevent
  • Causes of a port wine stain
  • Symptoms of a port wine stain
  • Skin
  • Scope
  • Colour
  • Bleed
  • Syndrome
  • Complications
  • Examination and diagnosis
  • Diagnosis à vue
  • Follow-up research
  • Differential diagnosis
  • Treatment of a wine stain
  • Camouflage
  • Laser treatment
  • Self-care
  • Prognosis
  • Prevention

What is a port wine stain on the skin?

A port-wine stain is a congenital (innate), benign skin defect, which consists of dilated or dilated blood vessels in the skin and is visible as a sharply defined, initially light red and later wine-red to even purple macula. The term ‘macula’ is used in dermatology for a change in color of the skin, without the skin showing any abnormalities. Sometimes the affected area can be thicker or more raised than the surrounding skin from an early age. This is called ‘hypertrophy’.

Port-wine stain in an adult: Mikhail Gorbachev / Source: Bernd vdB, Wikimedia Commons (CC BY-SA-1.0)

The spot grows in size as the child grows; the spot grows with the skin. The spot can occur anywhere, but is usually localized in the head and neck region. A birthmark such as nevus flammeus can put a heavy social-emotional burden on the person in question, especially if it is of a considerable size and is clearly visible. In addition, nowadays strong emphasis is placed on appearance.

Synonyms

A port-wine stain is also known by the following names:

  • nevus flammeus
  • port wine stain
  • nevus vinosus
  • nevus telangiectaticus
  • haemangioma planum

Prevent

A port-wine stain occurs in 1:300 of newborns worldwide. There is an equal distribution between the two sexes.

A well-known person with a wine stain is Mikhail Gorbachev (see photo), president of the Soviet Union (USSR) from 1990 to 1991 and known for the terms ‘perestroika’ and ‘glasnost’, which mean ‘reform’ and ‘openness’ respectively. .

Causes of a port wine stain

A port-wine stain is a disease of the capillaries, which are dilated. The cause is not yet well known in 2023. The condition is not hereditary. A mutation in the GNAQ gene is described in the literature, as is an association with RASA1. In a port-wine stain, the nerves that control blood vessels don’t work properly, so they are permanently dilated. The result is that the skin looks red. Usually, a port-wine stain is located somewhere on the face, neck, scalp, or upper torso.

Port-wine stain on a baby’s face / Source: ArturroD, Wikimedia Commons (CC BY-SA-3.0)

Symptoms of a port wine stain

Skin

A port-wine stain can appear on the entire body, but it usually occurs on one side on the face or neck. However, a port-wine stain can also appear on your hand, arm, leg or foot. The underlying skin shows no abnormalities. A port-wine stain only involves a change in color of the skin.

Scope

The size of a nevus flammeus is different in every child. It may be a small spot, but it may also cover half of the face. The spot grows with the skin, but does not grow itself.

Colour

The skin abnormality does not involute and therefore remains present throughout life. Initially they are often flat and usually clearly defined. The lesions may change from pink in childhood to (wine) red in early adulthood to deep purple during middle age. As the years progress, the nevus not only becomes more purple, but also becomes increasingly thicker and therefore more difficult to treat.

Bleed

Occasionally a port-wine stain may bleed spontaneously, often as a result of a granuloma pyogenicum. A ‘granuloma pyogenicum’ is a wart-like bulge or skin growth that is caused by rapid growth of capillaries and can bleed very quickly if touched.

Syndrome

A port-wine stain usually occurs in isolation, but can also be part of a congenital syndrome, such as Sturge-Weber syndrome or Klippel-Trenaunay syndrome.

Complications

About 1 in 100 babies born with a port-wine stain on the face suffer from eye or brain problems. If 1 in 300 babies have a port-wine stain and 1 in 100 have complications, then you can imagine how rare these problems are: they only affect 1 in 30,000 babies.

  • Eye problems : These can develop if the port wine stain is on the eyelid area. If a child has a port-wine stain next to one eye, an eye specialist will monitor the child regularly until he or she reaches adulthood.
  • Brain abnormalities : are an unusual association with port-wine stains on the face. This is due to extensive blood vessel abnormalities in the brain (the Sturge Weber syndrome). Epilepsy and other problems can then develop.
  • Spinal abnormalities and varicose veins can be other additional problems.

The vast majority of children with port-wine stains do not have these complications.

Examination and diagnosis

Diagnosis à vue

There is no specific test to diagnose a port wine stain. The diagnosis is made by eye. A biopsy is normally not necessary. A port wine stain should not be confused with a ‘stork bite’ that almost half of babies have, on their neck. This fades after about a year and is completely harmless.

Follow-up research

A port-wine stain is sometimes (though very rarely) a sign of another medical condition or syndrome. For example, port-wine stains on or near the eye or on the forehead should be checked. That’s because they can be associated with Sturge Weber syndrome which causes problems such as seizures, developmental delays and learning disabilities. Spots on the eyelids can lead to glaucoma, which can affect vision and lead to blindness if left untreated.

Differential diagnosis

Similar syndromes are:

  • nevus of Unna (stork bite);
  • nevus telangiectaticus;
  • angioma serpiginosum (nevus vasculosus tardus).

Treatment of a wine stain

Treatment can consist of camouflage and/or laser therapy. Many treatments have been tried in the past for port-wine stains, including freezing, surgery and radiation. Laser therapy has proven to be the most successful treatment so far, with the fewest drawbacks. It is the only method that can destroy the small blood vessels in the skin without causing significant damage to the skin.

Camouflage

If desired, a person with a port-wine stain can use camouflage cream. These are available in a wide range of colors and can be matched to the person’s skin tone. The cream is usually removed at the end of the day with a cleanser.

Laser treatment

The type of laser used to remove a port-wine stain depends on the age of the person and the particular characteristics of the port-wine stain. Older spots are not more difficult to treat, as is sometimes thought. Extensive research within the plastic surgery department of the Academic Medical Center or AMC in Amsterdam has shown that treating the port-wine stain in childhood does not lead to a better result than treatment in adulthood. Spots on the face generally respond better to laser therapy than those on the arms, legs, or torso.

Self-care

Port wine stains can sometimes become very dry. It is therefore important to use a moisturizer. Also, be sure to call the doctor if your child’s port-wine stain starts bleeding, hurts, itches, or becomes infected. If the port wine stain has been treated with laser surgery, do not rub the area and clean it gently with lukewarm water. The doctor may prescribe an ointment to aid recovery and healing and to help prevent infection.

Prognosis

A port-wine stain is a benign abnormality that remains on the skin throughout life. The port-wine stain grows in proportion to the growth of the child and shows no tendency for spontaneous healing or recovery. The color usually darkens and transforms into purple or deep red. The skin of a port-wine stain also often thickens and may feel pebble-like instead of soft.

Prevention

Port wine stains cannot be prevented; they are not caused by what a mother did during pregnancy.

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