Rehman S U, Hazir T, Nisar Y B, Krishin J, Matloob Azam, Hassan M.
Blue Rubber Bleb Nevus Syndrome: associated with Severe GI Bleeding requiring one hundred blood transfusions.
J Pak Med Assoc Jan ;53(11):570-2.
The patient is an eight years old girl, the product of a full term, uncomplicated pregnancy. At birth parents noticed two bluish macules over the anterior chest. With increasing age, similar new lesions appeared all over the body. These, lesions were soft, non-tender and of variable sizes. At three years of age, parents noticed an obvious change in the colour of her stools which turned tarry black. Patient gradually became anemic. She was put on Iron supplements and also required blood transfusions, and continued to have blood colored stools. At the time of admission, she had received about 100 blood transfusions. She was investigated and her upper GI endoscopy and colonscopy showed multiple characteristic bluish, bled like lesions in the hypopharynx, distal esophagus, stomach, duodenum and in the rectum, sigmoid and descending colon. Majority of these lesions were solitary, with a shiny surface. Histopathology of skin lesions showed dilated vascular channels in the dermis lined by endothelial cells, supported by collagen fibers.
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