port-wine stain; capillary malformations

Port-Wine Stain / Capillary Malformations - Symptoms

PWS usually appear as pink or pale purple patches on one side of the scalp, face, neck or limbs.

Although they can occur on any part of the skin, they occur most commonly on the head, neck and limbs.

They can vary in size from a few millimetres to a big patch covering almost half of the face or limb.

These patches grow in proportion to the growth of the child.

Although, in the initial six months of life, there might be an apparent lightening of the initial colour, this must not be mistaken as a sign of resolution. True PWS do not resolve without treatment. With age, the colour can darken and the affected skin may become thicker and lumpy.

Uncomplicated PWS do not usually cause any symptoms. However, they can affect the quality of life of the affected individual and their family due to their appearance.

Eczema can develop over a PWS, which may become itchy or sore.

Rarely, PWS can occur in association with other abnormalities depending on the location:

PWS occurring on the forehead or around the eye may be associated with eye abnormalities, including increased pressure (glaucoma), requiring treatment by a paediatric ophthalmologist.
PWS on the upper face / forehead can be associated with brain abnormalities, like fits and delayed development (Sturge-Weber-Syndrome). This may require further investigations with scans or referral to a paediatric neurologist.
PWS on the central back overlying the spine can be linked to an underlying spinal defect (spinal dysraphism). This may require referral to a paediatric neurosurgeon.
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.