Venous Malformations

Capillary Malformation (Nevus Flammeus, Port-Wine Stain, PWS) (Figure 2.17B)
  • 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)
    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