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OBJECTIVE. The purpose of this review is to describe the role of imaging and associated findings in the diagnosis of blue rubber bleb nevus syndrome, Proteus syndrome, Klippel-Trénaunay syndrome, and Kasabach-Merritt syndrome.

CONCLUSION. Blue rubber bleb nevus, Proteus, Klippel-Trénaunay, and Kasabach-Merritt syndromes are a diverse group of vascular malformation and hemangiomatosis syndromes. Both cutaneous and visceral vascular lesions are associated with these disorders. Accurate diagnosis of these syndromes is important because they can be associated with serious complications, including life-threatening hemorrhage.