What are port wine stains?
A port wine stain (sometimes called a capillary malformation) is a birthmark caused by abnormal development of blood vessels in the skin. They are usually a flat, red or purple mark on the skin that is present at birth.
Very occasionally, over time, the port wine stain may become thicker, darken and develop a ‘cobblestone’ appearance with raised bumps and ridges.
Port wine stain occurs early in pregnancy while the baby is developing in the womb. This change in the gene is not inherited (passed on from one generation to the next) and is not known to be related to anything that happened during pregnancy.
Port wine stains can appear anywhere on the body, in most cases on one side of the body only, but occasionally on both sides. About 65 per cent of port wine stains are on the head and neck. About three in every 1,000 children has a port wine stain. Girls are twice as likely to have a port wine stain as boys, but we do not know why.
Port wine stains are clearly noticeable and quite different to other types of birthmark so no special diagnostic tests are usually needed. Generally, port wine stains do not need any special treatment.
However, they do need protection from the sun. The patient should use a high factor sun cream on all areas of exposed skin, and use a hat to protect the patient’s face and/or an umbrella over the buggy or pushchair.
Port wine stains involving the upper part of the face can be linked to the following conditions:
Glaucoma: Patients with a port wine stain around the eye have an increased risk of glaucoma. Glaucoma is raised pressure within the eye, which can lead to blindness if it is not treated. Treatment is usually by eye drops and occasionally an operation. A specialist eye doctor (ophthalmologist) should examine the patient’s eyes to check for glaucoma.
When comparing to the normal eye, the eye on the port wine side may look different. If the pupil looks larger, the eyelids are open further or the eye itself looks larger or more prominent than the other, the patient’s eyes should be checked by a specialist eye doctor.
Sturge-Weber Syndrome: If the port wine stain is on the skin around the eye, forehead or scalp, there is a chance that the patient may have a condition called Sturge-Weber Syndrome. As well as the port wine stain affecting the skin, it may also involve blood vessels over the surface of the brain, which can cause seizures (fits or convulsions). If there is any suspicion the patient is at risk of Sturge-Weber Syndrome, they will need to be reviewed by a neurologist.
Klippel Trenaunay Syndrome: A large port wine stain on the arm or leg might be associated with extra growth of that limb and is referred to as Klippel Trenaunay syndrome. This may need a multidisciplinary review by dermatologists, and general, orthopaedic and vascular surgeons.
Patient eligibility criteria
There are two options for treatment of port wine stain:
- Camouflage makeup
- Laser treatment.
Skin camouflage products effectively cover the affected area. Support charity, Changing Faces, provides education by trained volunteer practitioners on the use and application of cosmetic camouflage creams and powders, and people may self-refer. The patient’s GP can prescribe camouflage makeup for patients.
Cosmetic camouflage creams can be used on any part of the body. The aim is to provide natural-looking cover. They are waterproof, and may remain on the body for up to four days, and on the face for 12-18 hours. Three brands of camouflage product, in a range of shades, are included in the Birmingham, Sandwell and Solihull APC Formulary, and the prescription must be endorsed ‘ACBS’.
- Covermark classic foundation (10 shades) and Covermark finishing powder
- Dermacolor camouflage cream (100 shades) and Dermacolor fixing powder
- Keromask masking cream (9 shades) and Keromask finishing powder.
Laser treatment for a port wine stain, may lighten the affected area of skin. The pulse dye laser is generally considered safe, although long term outcomes are not well studied. The laser passes through a fibre optic cable. On the end of the cable is a device that looks like a pen.
It’s gently held against the surface of the skin and a button is pressed, which sends a beam of light to the skin. Short term adverse effects are common which vary from pain, skin crusting, bruising and blistering. How effective the treatment is will depend on how prominent and dark the affected area is. The best results are often seen in marks that are already smaller and lighter.
Patients with port wine stains involving the upper part of the face should be appropriately referred for further investigation to ensure complications of port wine stain, as outlined above, are identified and managed appropriately.
Laser treatment is not usually funded by your local NHS commissioning organisation for the treatment of port wine stains.
This is because there is insufficient clinical evidence to support the use of laser treatment as an effective intervention for port wine stain, further research is warranted, particularly around long term outcomes, quality of life and cost effectiveness. This means that the CCG will ONLY fund the treatment if an Individual Funding Request (IFR) application proves exceptional clinical need and this is supported by the CCG.
Advice and guidance
- For more information, search for ‘birthmarks’ at www.nhs.uk
Choosing Wisely UK is part of a global initiative aimed at improving conversations between patients and their doctors and nurses.
Treatment policy for patients covered by NHS Birmingham and Solihull CCG