PORT WINE STAIN

By Gary M. White, MD

port wine stain A port wine stain on the cheek of an infant.


The port wine stain (PWS) is a congenital, stable, low-flow vascular malformation of the dermal capillaries and post-capillary venules. It occurs in 3-5 children per 1000 live births. It is a type of capillary malformation (CM) with the other main CM being the nevus simplex. Occasionally, a lesion that appears to be a PWS at birth will proliferate into an infantile hemangioma (IH).

Clinical

At birth, the child presents with a fixed, vascular patch whose color has been likened to port wine. Sometimes, in the first week or two of life, the distinction between PWS and IH is difficult. Palpation is key as the hemangioma will at some point be palpably thickened. Sometimes, waiting a week or two is needed to verify the diagnosis.

If untreated, lesions in adulthood may become thickened with multiple vascular nodules. There is a variant of PWS that is acquired in adult life.

Classification

The ISSVA (International Society for the Study of Vascular Anomalies) is a good reference for classification of vascular lesions in infants. Their classification for vascular anomalies may be found here.

Risk of Sturge-Weber Syndrome

The old teaching was risk for SWS is for anyone with involvement with V1. Recently, however, it has been determined that the risk SWS is only for those with forehead involvement [Br J Dermatol. 2014 Oct;171:861-7].

Treatment

Pulsed dye laser treatment is quite helpful in reducing the color of the lesion although multiple treatments are needed (e.g. 5-15) and usually, some slight vascular blush remains. Other lasers may be beneficial if the PDL is not. In general with regard to the face, smaller lesions respond better than large ones, lesions on the lateral face and forehead do better than those on the central face and patients treated at a younger age do better than those treated at an older age. (Once beyond one year, the age of treatment is less critical). Histologic analysis shows that the vessels that comprise the PSWs are located more superficial laterally, whereas in the central regions they extend deeper into the dermis and the subcutaneous tissue [JAAD 2016;74;527].

Features that make a PWS more treatment-resistant
Age over 1 year
Larger lesion (e.g. > 40 cm2)
Central face
Hypertrophic or nodular
Large vessels on pathology

Topical rapamycin may improve the efficacy of laser therapy by 10-15% but an issue of bias of the study has been raised (lateral vs central face concerns) [JAAD 2015 Jan;72:151]. Concerns however about systemic absorption and its potential effects in the very young may need to be addressed.

The addition of timolol gel for preventing neoangiogenesis failed to significantly improve the efficacy of pulsed dye laser treatment of PWS [British Journal of Dermatology 170 6:1350–1353, June 2014]

Additional Pictures

port wine stain port wine stain

Congenital PWS on the sole of a woman.
port wine stain

In an adult, PWSs may thicken and develop vascular papules.
port wine stain port wine stain

Here is a dramatic case of vascular nodularity forming later in life.
port wine stain in adult life causing nodularity

Port wine stain on the leg
Port wine stain on the leg

A congenital vascular stain over the spine always brings up the question of possible spinal dysraphism.
port wine stain port wine stain

References

Rarely, diffuse modularity may occur in adult life. Indian Dermatol Online J 2015;6:467-8


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