Blue Rubber Bleb Nevus Syndrome 1예

Abstract

Blue Rubber Bleb Nevus syndrome, or Bean's syndrome is a very rare disease characterized by an association of 1) hemangioma cutis in which the nevi feel like rubber blebs; 2) hemangiomas of the digestive organs; 3) iron-deficiency anemia due to hemorrhage from the digestive tract. Sometimes, the bleeding is so severe and massive that the patient needs blood transfusions and/or emergency operations. We present a 29-year-old man with Blue Rubber Bleb Nevus syndrome. This patient was admitted to out hospital due to recently aggrevated dizziness and intermittent rectal bleeding. Multiple bluish tumors were noted on the palm, sole and glans penis. Laboratory examination revealed severe iron-deficiency anemia. Barium studies revealed multiple polypoid masses in the stomach, small bowel and colorectum. On esophagogastroduodenoscopy and colonoscopy, three or four dozens of hemangiomas with variable size and shape were noted in the stomach, duodenum at or around the ampulla of Vater and colorectum. This patient was of particular interest because rectal bleeding occurred from rectal lesion which protruded out of anus and caused bleeding during defecation, and which showed as a 2.0 cm Yamada type III polypoid lesion. Endoscopic ultrasonography confirmed us that this lesion was confined to the mucosa and submucosa without extension into muscle layer. Endoscopic polypectomy of this lesion was performed because which considered as the main focus of current rectal bleeding. He was discharged without any complication and 4 months later, he was well without rectal bleeding and his hemoglobin level increased upto 13.5 g/dl. To our knowledge, this is the first . case of Blue Rubber Bleb Nevus syndrome in Korea.