![Age progression of cutaneous PWB. Birthmark is usually apparent from birth as a light pink patch that can get darker with age, corresponding to progressive dilation of affected capillaries in the dermis. Patients in adulthood may develop soft tissue hypertrophy or nodularity in the absence of clinical intervention. Images created using BioRender.

Van Trigt WK, Kelly KM, Hughes CCW. GNAQ mutations drive port wine birthmark-associated Sturge-Weber syndrome: A review of pathobiology, therapies, and current models. Frontiers in Human Neuroscience. 2022;16. doi:https://doi.org/10.3389/fnhum.2022.1006027](attachment:5d7b8306-b5af-4df7-8c94-0d21442871fd:fnhum-16-1006027-g001.jpg)

Age progression of cutaneous PWB. Birthmark is usually apparent from birth as a light pink patch that can get darker with age, corresponding to progressive dilation of affected capillaries in the dermis. Patients in adulthood may develop soft tissue hypertrophy or nodularity in the absence of clinical intervention. Images created using BioRender.

Van Trigt WK, Kelly KM, Hughes CCW. GNAQ mutations drive port wine birthmark-associated Sturge-Weber syndrome: A review of pathobiology, therapies, and current models. Frontiers in Human Neuroscience. 2022;16. doi:https://doi.org/10.3389/fnhum.2022.1006027

![Photographs of a participant with the Sturge–Weber syndrome (Patient 36), obtained at birth, show a facial port-wine stain with a left-sided V1 distribution (Panels A and B). The child began having seizures at 7 months of age. An isolated port-wine stain birthmark on the left shoulder from a participant without the Sturge–Weber syndrome (Patient 10) shows a birthmark that is flat and red without evidence of hypertrophy or cobblestoning or any other associated vascular or lymphatic anomaly (Panel C). Axial CEMR scans of the brain in a participant with the Sturge–Weber syndrome (Panels D, E, and F; Patient 36 at 17 months of age) show left-sided hemispheric leptomeningeal enhancement (yellow arrows), an enlarged and enhancing left-sided choroid plexus (red arrow), and left hemispheric brain atrophy (white arrows).

Shirley MD, Tang H, Gallione CJ, et al. Sturge–Weber Syndrome and Port-Wine Stains Caused by Somatic Mutation inGNAQ. New England Journal of Medicine. 2013;368(21):1971-1979. doi:https://doi.org/10.1056/nejmoa1213507‌](attachment:77279186-46f9-4ae8-a5e9-e9b12142e3e8:nejmoa1213507_f1.jpg)

Photographs of a participant with the Sturge–Weber syndrome (Patient 36), obtained at birth, show a facial port-wine stain with a left-sided V1 distribution (Panels A and B). The child began having seizures at 7 months of age. An isolated port-wine stain birthmark on the left shoulder from a participant without the Sturge–Weber syndrome (Patient 10) shows a birthmark that is flat and red without evidence of hypertrophy or cobblestoning or any other associated vascular or lymphatic anomaly (Panel C). Axial CEMR scans of the brain in a participant with the Sturge–Weber syndrome (Panels D, E, and F; Patient 36 at 17 months of age) show left-sided hemispheric leptomeningeal enhancement (yellow arrows), an enlarged and enhancing left-sided choroid plexus (red arrow), and left hemispheric brain atrophy (white arrows).

Shirley MD, Tang H, Gallione CJ, et al. Sturge–Weber Syndrome and Port-Wine Stains Caused by Somatic Mutation inGNAQ. New England Journal of Medicine. 2013;368(21):1971-1979. doi:https://doi.org/10.1056/nejmoa1213507‌

Klippel-Trenaunay syndrome: Geographic stain on the lateral thigh, knee, and lower leg of an 18-year-old male with the Klippel-Trenaunay syndrome (patient 2). Swelling of the foot dorsum, wide first web space, varicose veins, and hypertrophy of the leg were also present. White arrows, varicosities. | Sung, H. M., Chung, H. Y., Lee, S. J., Lee, J. M., Huh, S., Lee, J. W., Choi, K. Y., Yang, J. D., & Cho, B. C. (2015). Clinical Experience of the Klippel-Trenaunay Syndrome. Archives of plastic surgery, 42(5), 552–558. https://doi.org/10.5999/aps.2015.42.5.552