Port-wine Stains

September 14, 2005
Alexander K. C. Leung, MD

,
C. Pion Kao, MD

These lesions are always present at birth. They consist of mature dilated capillaries and represent a permanent developmental defect. They are red to purple, macular, and sharply circumscribed. They are usually unilateral and may occur anywhere on the body, although they tend to favor the face. Unlike salmon patches, port-wine stains do not fade; in fact, they tend to darken and become nodular with age.

These lesions are always present at birth. They consist of mature dilated capillaries and represent a permanent developmental defect. They are red to purple, macular, and sharply circumscribed (A). They are usually unilateral and may occur anywhere on the body, although they tend to favor the face. Unlike salmon patches, port-wine stains do not fade; in fact, they tend to darken and become nodular with age.

If the lesions are present in the trigeminal area (B), they may be associated with a vascular malformation of the ipsilateral meninges and cerebral cortex (Sturge-Weber syndrome). Port-wine stains also occur as a component of Klippel-Trnaunay-Weber syndrome and with moderate frequency in other syndromes, such as Rubinstein-Taybi syndrome, Cobb syndrome, Beckwith-Wiedemann syndrome, and trisomy 13 syndrome.

Treatment with the pulsed tunable dye laser is highly effective and should be considered for all children with port-wine stains if the lesion is bothersome or unsightly. Some patients can achieve significantly improved cosmetic appearance with the use of a tinted, waterproof makeup.