Overview

A port wine stain is a type of capillary vascular malformation. The appearance of a port wine stain resembles a maroon-coloured wine spill on the skin, and is a relatively common condition, affecting 1 in 300 babies.¹ They are caused by abnormal development of blood vessels in the skin. At birth, they start out light pink in colour, gradually becoming darker as you grow older. They usually appear on the face, but can affect other areas of the body. A port wine stain can be flat or slightly raised, and has a clear border of where the birthmark starts and stops. They will only fade with treatments such as laser therapy because they are permanent birthmarks.

Symptoms and causes

Why do I have a port wine stain?

The exact cause of a port wine stain is unknown. However, a port wine stain is a type of  vascular birthmark, and has been linked to the formation of abnormal blood vessels near the skin surface during foetal development, which explains their pink and red appearance. While they may be caused due to a genetic condition, port wine stains are generally sporadic, and are therefore not hereditary.

What do they look like?

Port wine stains are always present at birth. Early-stage port wine stains are usually flat and pink. Over time, the stain will become thicker, darken to a deeper red or purple colour, and develop a ‘cobblestone’ appearance with raised bumps and ridges.² They can range in size from a few millimetres to several centimetres, and they can appear anywhere on the body, but are most commonly found on the head and neck.

How common are they?

It is a very common skin condition, where about three in every 1000 children has a port wine stain. Girls are twice as likely to acquire a port wine stain than boys, although the reason behind this is unclear.²

Diagnosis and tests

As they are very noticeable and distinguishable, no special diagnostic tests are needed. However, if doctors suspect that other tissue groups are affected, they can be clinically investigated using an ultrasound scan. In more complicated cases, an MRI or angiography will be performed to help plan any necessary treatments.³

Are there any long-term problems associated with port wine stains?

Port wine stains are noncancerous and they don’t cause any physical discomfort to the individual.⁴ Although, for many children and teenagers, port wine stains may impact their self-esteem and social skills because they may find their birthmark embarrassing.

Most vascular birthmarks are not associated with other abnormalities. However, in very rare cases, port wine stains involving the upper part of the face can be associated with several health conditions such as:²

• Glaucoma

Glaucoma are common eye conditions where the connections to the optic nerves become damaged. Children with a port wine stain around the eye have an increased risk of glaucoma because the birthmark creates extra pressure inside the eye. This can lead to blindness if not treated

• Sturge-Weber syndrome

Port wine stains around the eye, forehead or scalp mean there are abnormal levels of blood vessels. This can lead to an extra layer of blood vessels over the surface of brain, with some extra vessels found in the lining of the eye. This leads to a condition known as Sturge-Weber syndrome, and is caused by a change in the GNAQ gene during pregnancy

• Klippel-Trenaunay syndrome

A large port wine stain with extra growth on the arm or leg might be associated with Klippel-Trenaunay syndrome. It will need to be investigated by dermatologists, laser specialists and vascular surgeons.

Management and treatment

Treatment

Port wine stains that are not linked to other medical conditions do not require medical treatment. But some people may choose to treat their birthmark in order to:

• Improve the individual’s self-esteem and socialisation levels
• Reduce the risk of infection if the birthmark bleeds
• Prevent potential disfigurement later in life

Laser therapy

Laser therapy is the most common treatment when managing the appearance of port wine stains. Treatment often starts in infancy and uses intense light to shrink the blood vessels and improve the appearance of the birthmark by causing discoloration to the area. The success of treatment depends on the size, location, and depth of the stain, and may require multiple treatments over time.⁵

Early treatment is usually more effective, because the port wine stain is relatively smaller and the baby’s skin is thinner, however this doesn’t prevent the stain from darkening over time.¹ This treatment is not exclusive to infancy, and can help teenagers and adults too. Although, it may be harder to successfully treat due to the larger size and depth of the birthmark.

The specific laser used in the treatment of port wine stains is the pulsed dye laser. The laser targets the red pigmentation in the blood vessels to reduce the appearance of the birth mark.⁶ These treatments are very brief, as they last for less than 10 minutes. 

After treatment, the area may be irritated and bruised. Blistering and crusting may also appear temporarily but the skin should heal itself between 7 and 10 days. If you need multiple treatments, it can be done every 6-8 weeks to allow the skin to fully recover.  

Although this is deemed as the ‘gold standard’ treatment for reducing the appearance of birthmarks, a follow up study identified that 10 years after the initial laser treatment, their port wine stains were significantly darker, which is important to note.

In certain cases, surgical interventions may be considered to address specific challenges associated with the birthmark.

Skin camouflage

Another great treatment option is skin camouflaging. Skin camouflage is less invasive than laser therapy and can be used at home. It is a special type of highly pigmented water resistant make up which will be matched to the colour of your skin (like concealer) which allows your birthmark to blend in with your skin naturally. To obtain skin camouflage, it needs to be prescribed to you by your GP.

Coping strategies to manage having a port wine stain

Coping with a visible birthmark involves not only medical interventions, but emotional support too. Support groups and communities provide a safe space for individuals to share their thoughts and experiences. Emotional well-being, education and awareness initiatives play an important role in allowing these individuals to feel accepted within society.

Here are a few links to support groups and communities within the UK for managing birthmarks:

• Birthmark Support Group
  • Tel: 07825 855 888
• Changing Faces
  • Tel: 0300 012 0275
• British Association of Skin Camouflage
  • Email: info@skin-camouflage.net

Conclusion

If you are one of the 1 in 300 people who have been affected by port wine stains, you are not alone. They are commonly prevalent in society and through the utilisation of support groups, you may feel more accepted within society. For those who do not suffer from port wine stains, through the understanding of the medical aspects, treatment options and fostering empathy and awareness are crucial steps towards supporting individuals with port wine stains. As Fred Meijer says, learn to enjoy and respect each other's differences.