A 48-year-old Caucasian man was found to have a rare neoplastic process of vascular endothelial origin. The clinical presentation was that of plum-colored cutaneous plaques of the lower extremities. The histopathology, natural history, and treatment of this case are discussed and compared with previously reported cases. It is suggested that this disease entity represents a malignant state with systemic involvement and a poor prognosis.
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