Port-wine stain

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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.

Who gets 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.

Predisposing Factors

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

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.

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