What is it?

A port wine stain (PWS) is a congenital capillary malformation. There are several syndromes that are associated with PWS, including Sturge-Weber syndrome and overgrowth syndromes (Diffuse capillary malformation with overgrowth (DCMO), Klippel-Trenaunay syndrome, Megalencephaly-capillary malformation (MCAP), CLOVES syndrome, Proteus syndrome and PTEN Hamartoma syndrome). Facial or large PWS should prompt a work-up to rule out an associated syndrome.

What does it look like?

PWS are usually present at birth as well-demarcated, bright or deep red macules and patches. They are most commonly unilateral and often occur on the face. PWS grow in proportion with the child. Over time PWS can become darker in colour and develop skin thickening and nodules. These changes are most common in facial PWS and rarely seen in those on the trunk or extremities.

How is it diagnosed?

PWS do not spontaneously resolve or involute. Many patients want treatment due to the cosmetic appearance of these lesions and they can have a significant psychosocial impact. Treatment with a vascular laser is very effective although it does require multiple treatment sessions. If a PWS is associated with Sturge-Weber syndrome or an overgrowth syndrome, these patients require multidisciplinary care by the family physician, specialist pediatricians, and dermatology.