Capillary Malformation (Nevus Flammeus, Port-Wine Stain, PWS)

• Figure 2.17 A: Kaposiform hemangioendothelioma (in Kasabach-Merritt) (Reprint from Laxer RM, ed. The Hospital for Sick Children: Atlas of Pediatrics. Philadelphia, PA: Current Medicine; 2005) B: Port-wine stain (Reprint from Abel-Halim AW. Passing the USMLE. New York, NY: Springer; 2009) C: Glomangiomas (Courtesy of Dr. Michelle B. Bain)

  Presents as a well-demarcated erythematous patch or plaque that grows in proportion to general growth of the body; does not spontaneously recede (unlike “salmon patches” over forehead, glabella, nose/philtrum, nape or eyelid which typically disappear by age 3)
• Facial PWS follows sensory CN V distribution (V1–V3); over time, skin changes from pink to deep purple and thickens with ↑ nodularity and pyogenic granulomas
• GLUT1 negative
• PWS can be seen with combination of epidermal or melanocytic abnormalities: phakomatosis pigmentovascularis (see below)
• Associated syndromes: Sturge–Weber syndrome, Klippel–Trénaunay syndrome, Proteus syndrome