What is Port-wine stain

Port-wine stain is also known as naevus flammeus. It is a vascular malformation, which means it is a birthmark made of enlarged capillaries in the skin. Capillaries are tiny blood vessels in the body that connect the arteries and veins together. They are normally small and tiny. With enlarged capillaries, port-wine stain produces a red to purple discolouration of the skin.

Port-wine stain

Statistics on Port-wine stain

Port-wine stains are present at birth at the rate of 0.1-0.3% of the population. This means it happens from 1-3 births in 1000 births. It can happen anywhere on the body. However it affects one side of the part of body in 85% of the cases.

Risk Factors for Port-wine stain

Port-wine stain is usually not hereditary (ie. not passed down from parents to child), and it does not have any racial or gender predisposition. It affects males and females equally.

Progression of Port-wine stain

Port wine stain appears as a flat birthmark, ranging from light pink to deep purple in colour. It usually grows proportionately with age and become extremely unsightly in some cases. The lesion may become raised or bumpy as the patient achieves puberty. Later in life, the surface may become bumpy and uneven, and it may bleed easily.
The significance of port wine stain is its association. If the port wine stain involves the skin in the face affecting the upper forehead, it may be associated with an uncommon disorder called Sturge-Weber syndrome. In this syndrome, there is other vessel defects in the brain. This can lead to mental retardation, fitting, weakness on one sided of the body or other neurological signs and symptoms later in life. In Sturge-Weber syndrome, the stain always involves the upper part of the face.
If both sides of the face is involved, where the skin of both eyelids has the stain, congenital glaucoma may occur.
Other associations include spinal bone deformities. This must be suspected when a port-wine stain occurs over the lower back.
Otherwise, port wine stain usually presents as an unsightly birthmark and does not fade over time.

Prognosis of Port-wine stain

Port-wine stain does not affect the normal lifespan of an individual. However if its association (like Sturge-Weber syndrome) is found, it might affect the patient’s life and hence shorten the lifespan.

How is Port-wine stain Treated?

If the port-wine stain is suggestive of associations such as Sturge-Weber syndrome or congenital glaucoma, it should be referred to the respected specialists promptly such as a paediatric neurologist or an ophthalmologist. Treatment usually consists of supportive measures for Sturge-Weber syndrome, such as drugs for fitting, special education for the child, etc.
Otherwise, there is no cure for port-wine stain, however it can be treated for cosmetic purposes so that it does not provide a psychological impact in later life to the patient.
Cosmetic treatment of port-wine stain was often unsatisfactory before the development of laser technology. Currently, laser therapy called the pulsed dye laser (PDL) is used to treat port-wine stain. There are studies confirming its effectiveness in adults, children and infants with low side effects. Hence, PDL is currently the first-line treatment for port-wine stain.

Port-wine stain References

[1] Enjolras O, Riche MC, Merland JJ. Facial port-wine stains and Sturge-Weber syndrome. Pediatrics 1985; 76:48-51.
[2] Up to Date: Vascular lesions and congenital naevi in the newborn [online]. 2005. [Cited 2005 October 4th]. Available from: URL: http://www.utdol.com/application/topic.asp?file=ped_derm/4439&type=P&selectedTitle=3~5
[3] Vinciullo C. Pulsed dye laser treatment of port-wine stains: a review of patients treated in Western Australia. MJA 1996; 164: 333-336.