Blue rubber bleb naevus syndrome

Abstract

An autopsy case of blue rubber bleb naevus syndrome (BRB-NS) is reported. There was the usual occurrence of cavernous haemangioma in the skin and intestine and cardiac involvement by a haemangiomatous lesion might have directly led to the patient's death. By light and electron microscopy, all the haemangiomatous lesions examined were cavernous with the exception of the cardiac tumour which was a mixed-vessel (capillary and cavernous) type of a haemangioma. These findings indicate that any vascular tumour-like lesions may occur in BRBNS. The principal combination of haemangiomas in the skin and intestine in BRBNS regardless of their type is the typical feature of this syndrome.