Glomangioma (Glomuvenous Malformation)

• 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)

  Arises in children and adolescents; may be sporadic or inherited (autosomal dominant with incomplete penetrance; defect in glomulin gene)
• If solitary lesion (glomus tumor), onset typically in adulthood with subungual location
• Presents as soft pink to deep blue papules or nodules in segmental distribution; tender to palpation, ± attacks of pain with pregnancy or menstruation
• Histology: resembles vascular malformation but vessels lined with one or more rows of cuboidal glomus cells