Don't Be Rash: Advances in Diagnosis and Treatment of Port Wine Capillary Malformations

Welcome back to Don’t Be Rash!

Port wine capillary malformations (PWCMs), previously referred to as port wine stains or port wine birthmarks, are congenital vascular lesions caused by genetic changes that result in abnormal blood vessels within the skin. Although typically flat and pink, red, or purple at birth, these lesions can evolve over decades, with some developing vascular nodules, skin thickening, or enlargement of underlying structures. Their variable depth, vessel size, and flow make treatment response difficult to predict.

In this episode, Kristen Kelly, MD, chair of dermatology at the University of California, Irvine, and host Andrew Krakowski, MD, pediatric dermatologist and chair of dermatology at St. Luke’s University Health Network, discussed current approaches to PWCM management. She emphasized that pulsed dye laser (PDL) remains the gold-standard treatment, with a long safety record and established efficacy. Importantly, treatment can be initiated very early, potentially within the first weeks of life.

Early Treatment Benefits

“There is evidence that we may get a better result if we start early,” Kelly said. “You have a higher chance of getting 90% or more resolution or improvement by the time that a child is one year of age."

Early intervention may offer several advantages, including the presence of fetal hemoglobin as an additional laser target, smaller and more superficial vessels, and lower melanin levels that allow laser energy to reach the blood vessels more effectively. Early treatment may also help reduce the psychosocial impact of a visible birthmark before children enter school.

"It's probably more acceptable for a 1- or 2-year-old to be bruised up than a kid who's trying to integrate back into the classroom and not have to answer a bunch of questions that he or she's just not comfortable having to deal with," Krakowski noted.

Alternative Therapies and Recurrence Challenges

Despite these advantages, treatment outcomes remain variable. Facial lesions generally respond better than those on the extremities, while lesions on the lateral face may respond better than those in central facial locations. Revascularization also contributes to recurrence because the underlying genetic alteration remains within some endothelial cells after laser treatment. Kelly noted that clinicians currently cannot reliably predict the number of treatments required for a given patient.

Alternative laser technologies include long-pulsed 532-nm lasers and intense pulsed light, although Kelly generally favors PDL in children. Deeper-penetrating 755-nm and 1064-nm lasers may have a role in adults with thicker lesions or nodules but carry greater safety concerns. Photodynamic therapy, which uses an intravenous photosensitizer followed by light exposure, represents another potential approach and may offer advantages across skin types, although systemic photosensitivity and procedural discomfort limit its practicality.

Future Directions in Research and Patient Care

Research is increasingly focused on the biology underlying PWCMs, including the common GNAQ mutation, and on technologies such as optical coherence tomography to characterize vessel depth and density. These advances could eventually improve treatment selection and help predict response.

"The Sturge-Weber Foundation and the Vascular Birthmark Foundation have really done a tremendous amount in terms of getting knowledge out, guiding treatment guidelines, and also helping to promote research,” Kelly said. “I feel pretty confident in the next couple [of] years that we'll know more about the pathway and be able to target it better."

Don’t Be Rash is produced in collaboration with the Society for Pediatric Dermatology. For more information on the society, its programs, and resources, check out their website.

Have a topic you think we should talk about? Email us at [email protected] to join the conversation!