The Journal of the Arkansas Medical Society Med Journal Feb 2019 Final 2 | Page 10

CASE STUDY A Rare Case of “Blue Pseudo-polyposis” Abhilash Perisetti,MD 1 ; Saikiran Raghavapuram, MD 2 ; Nayana George, MD 2 ; Debdeep Banerjee, MD 3 ; Benjamin Tharian, MD 2 Hospital Medicine Division, Department of Family and Community Medicine, Texas Tech University, Lubbock, Texas 2 Department of Gastroenterology, UAMS 3 Department of Medicine, University of Florida College of Medicine, Gainesville, Florida 1 A 72-year-old Caucasian male with a history of hyperten- sion presented with fatigue and bleeding per rectum. He denied history of hematemesis, melena, or weight loss. Past medical, surgical and family histories were unremarkable. No history of alcohol abuse or use of over-the-counter medications. Physical examina- tion showed conjunctival pallor without any skin or oral lesions. Digital rectal examination was normal. Laboratory testing showed hemoglobin of 7.1 g/dL with microcytic hypochromic anemia. Platelets, coagulation and liver function tests were within normal limits. CT abdomen did not show evidence of visceral hemangiomas, mass lesions, or ascites. Esophago- gastroduodenoscopy (EGD) was normal. Colonoscopy revealed blue, soft, rubbery, polypoid and easily compressible lesions, throughout the colon, with no evidence of fresh or altered blood (Fig. 1-4, Video). They varied in size from 0.5 to 2cm across, sparing the small bowel. The diagnosis is Blue Rubber Bleb Nevus Syndrome (BRBNS). It is a rare congenital disorder characterized by vascular malformations affecting the skin, soft tissue, and gastrointestinal tract but can involve any tissue with vascular supply. William Bean first described it in 1958 (also known as “Bean syndrome”) as a triad of bluish vascular nevi on the skin, gastro- intestinal (GI) bleeding and hemangiomas of the graft. 1 BRBNS has been noted in Caucasians more than in other races. Most cases are sporadic, though autosomal dominant inheritance has been reported. It is predom- Figure 1. 178 • THE JOURNAL OF THE ARKANSAS MEDICAL SOCIETY Figure 2. inantly a disease of childhood and adolescence, with only 4% noted in adults. 2 Malignant transformation has not been reported. Gastrointestinal involvement is noted mostly as adults. It can affect the entire GI tract, from the oral cavity to the anal canal. Chronic blood loss can result in iron-defi- ciency anemia rarely needing blood transfusion. 2 Though skin lesions are Figure 3. VOLUME 115