All issues > Volume 29(12); 1986

Case Report

J Korean Pediatr Soc. 1986;29(12):1372-1376. Published online December 31, 1986.

A Case of Blue Rubber Bleb Nevus Syndrome.

Kwang Soo Oh¹, Soon Kook Choi¹, San Ho Kim¹, Bock Keun Kee¹, Hyung Sun Sohn², Seok Don Park²

¹Department of Pediatrics, Won Kwang University School of Medicine, Iri、Korea
²Department of Dermatology, Won Kwang University School of Medicine, Iri、Korea

Abstract

The Blue Rubber Bleb Nevus Syndrome is defined as rarely occuring hemangioma of the skin and gastrointestinal tract, with intestinal hemorrhage. Bean in 1958 gave the name Blue Rubber Bleb Nevus (BRBN) to a variant of bluish hemangiomas of the gastrointestinal tract which causes bleeding, which had been described for the first time by Gascoyen. We experienced a case of Blue Rubber Bleb Nevus Syndrome in a 2 month old male who was admitted to our Hospital because of pallor and multiple hemangiomas on the general body surface. This skin lesion had been present since birth and had grown progressively in size and numbers and turned into blue color, but some lesions regressed spontaneously. The histopathologic findings of red hemangioma revealed dilated vascular spaces containing erythrocytes in papillary dermis and many large, irregular, vascular spaces lined by single layer of endothelial cells in mid reticular dermis. The sweat glands were in close proximity to many of the vascular structures. The radiologic examination revealed numerous tiny- polypoid filling defect in small and large intestine suggesting hemangiomas. The review of literature was made briefly.

Keywords :Blue rubber bleb nevus syndrome, Hemangioma of the skin and gastrointestinal tract