A 38 year-old man with abdominal pain and 20 pound weight loss. Clinical findings: cutaneous hemangiomas, anemia, hepatosplenomegaly and thrombocytopenia. Small bowel series and barium enema showed coarse nodularity of the ileum and colon. MR scan suggested diffuse hemangiomas involving skin, bowel, spleen, scrotum and pelvic soft tissues, extending into the thigh.
Upper endoscopy demonstrated esophageal varices (top left photo) and gastric fundic varices (top right photo), consistent with portal hypertension. Fiberoptic sigmoidoscopy revealed diffuse hemangiomas involving the rectum and sigmoid colon (bottom photo).
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