Introduction In his classic monograph in 1958 Bean1 separated from the heterogenous group of vascular lesions of the skin a variety of bluish hemangioma of the skin found in association with hemangiomas of the gastrointestinal tract which cause serious bleeding. To this he gave the name "blue rubber-bleb nevus of the skin and gastrointestinal tract" because the larger hemangiomas "have some of the feel and look of rubber nipples, are compressible and refill fairly promptly from their rumpled compressed state." The skin lesions vary in size, shape, and number. He did not emphasize the microscopic appearance of the lesions although he included a photomicrograph of a section of one of the skin lesions in the case he described. He stated "While much less common than hereditary hemorrhagic telangiectasia, the syndrome of erectile bluish nevi of the skin and angiomatosis of the gut associated with enteric bleeding is definite" . . . "Though