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Port wine stain

PORT WINE STAIN

What are the aims of this leaflet?

This leaflet has been written to help you understand more about Port Wine Stains. It tells you what this condition is, what it is caused by, what can be done about it, and where you can find out more about it.

What is a Port Wine Stain?

A Port Wine Stain is a collection of abnormally formed blood vessels (capillaries) in the skin, which results in a red mark that may have the colour of port wine. Although most Port Wine Stains are present at birth, it has been reported in few cases to develop later in life (‘acquired Port Wine Stain’).

What causes a Port Wine Stain?

The cause of Port Wine Stains is not known. During the development of an affected baby in the mother’s womb, abnormal small blood vessels are formed near the surface of the skin.

Acquired Port Wine Stains may appear without known cause, as part of an illness or in previously inflamed or injured skin.

Is a Port Wine Stain hereditary?

Port Wine Stains do not often run in families; however, they are relatively common, affecting about 1 in 300 babies, equally in both sexes. Port Wine Stains are not contagious or cancerous.

What are the symptoms of a Port Wine Stain?

Uncomplicated Port Wine Stains do not usually cause any physical symptoms. However, they can upset the quality of life of the affected individual and their family.

Eczema can develop over a Port Wine Stain, which may then be itchy or sore.

A Port Wine Stain may rarely be part of more widespread abnormalities, some of which are listed below:

  • A Port Wine Stain in the skin around the eye, may be associated with increased pressure in the eye (glaucoma). A referral to an eye-specialist may be required.
  • Rarely a Port Wine Stain on the upper face can be linked to abnormalities within the brain (called Sturge-Weber-Syndrome). This can be investigated by the neurology specialists with scans.
  • A Port Wine Stain on the central back overlying the spine can be linked to an underlying spine defect called spina bifida (Latin for ‘split spine’).
  • Klippel-Trenaunay-Syndrome occurs when there is enlargement of the limb affected by the port wine stain, which may also develop enlarged deeper varicose-type veins.

What does a Port Wine Stain look like?

Most Port Wine Stains affect the face, but they may involve any area of the skin.

The appearance of a Port Wine Stain tends to change during life. A flat faint red, purple or pink mark is usually seen at birth, which may become temporarily darker when the baby cries, has a temperature or is teething. Although the Port Wine Stain usually does not get any larger, it does grow in proportion with the child.

Port Wine Stains often turn darker red or purple in adults and the skin becomes thicker. Lumps can form (a cobblestone-like appearance), which may bleed readily. The lip or nose, if involved, may in some people become slightly swollen.

How is a Port Wine Stain diagnosed?

A dermatologist can diagnose a Port Wine Stain by taking a history about the skin changes and looking at the appearance of the skin.

Can a Port Wine Stain be cured?

Unfortunately, a Port Wine Stain does not go away on its own and usually cannot be cured, although it may become far less noticeable after treatment.

How can a Port Wine Stain be treated?

Various lasers are in use for treatment of Port Wine Stains and may be available on the NHS. Early treatment is possibly more effective, as the baby’s skin is thinner and the Port Wine Stain relatively small. However, even after successful early treatment, the remaining areas of Port Wine Stain may darken again later in life.

The Pulsed Dye Laser is most commonly used. It emits a beam of special light, which reacts with the red colour in blood. Adults do not usually require an anaesthetic, apart from cooling the skin. Children may need a general anaesthetic. The treatment causes immediate dark bruising and the skin is more sensitive to rubbing. Other possible temporary side effects include blistering and crusting. Scarring is rare. A course of laser treatment is usually required, with a few months between repeated treatments. The aim of treatment is to make the Port Wine Stain paler, as it is often not possible to make it fade completely. If the Port Wine Stain has developed bleeding areas, these can be successfully treated by laser.

In addition to the Pulsed Dye Laser, several other lasers and light-based treatments are in use, and research is on-going for treatment-resistant Port Wine Stains.

Skin Camouflage can be very useful. Camouflage is a type of special, water resistant make-up matched to the colour of the normal skin. You can get a prescription for a special type of camouflage make-up that covers up the birthmark. The Charity ‘Changing Faces’ may offer camouflage advice in your area (see below for further information).

You can also read more about skin camouflage on the websites of the British Association of Skin Camouflage

Where can I get more information about a Port Wine Stain?

Web links to detailed leaflets:

http://www.dermnetnz.org/vascular/vascular-malformation.html

http://www.patient.co.uk/doctor/Port-wine-Stain.htm

Links to patient support groups:

Birthmark Support Group

Tel: 07825 855 888
Email: info@birthmarksupportgroup.org.uk   
Web: http://www.birthmarksupportgroup.org.uk/

Changing Faces

Tel: 0300 012 0275 (for support and advice)
Tel: 0300 012 0276 (for the Skin Camouflage Service)
Email: skincam@changingfaces.org.uk 
Web: www.changingfaces.org.uk

British Association of Skin Camouflage (NHS and private practice)  

Tel: 01254 703 107
Email: info@skin-camouflage.net 
Web: www.skin-camouflage.net

For details of source materials used please contact the Clinical Standards Unit (clinicalstandards@bad.org.uk).

This leaflet aims to provide accurate information about the subject and is a consensus of the views held by representatives of the British Association of Dermatologists: individual patient circumstances may differ, which might alter both the advice and course of therapy given to you by your doctor.

This leaflet has been assessed for readability by the British Association of Dermatologists’ Patient Information Lay Review Panel

BRITISH ASSOCIATION OF DERMATOLOGISTS
PATIENT INFORMATION LEAFLET
PRODUCED AUGUST 2013
UPDATED DECEMBER 2016
REVIEW DATE DECEMBER 2019

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