Blue Rubber Bled Nevus syndrome: three cases

M. Muñoz-Navas, I. Fernández-Urién, E. Espinet, M. Betés, C. Carretero and J. C. Súbtil

Service of Digestive Diseases. Clínica Universitaria de Navarra. Pamplona, Spain

 

In recent years, three cases of Blue Rubber Bleb Nevus Syndrome (BRBNS) have been diagnosed in our center. In the younger patients (18 and 49-year old), endoscopic examinations were indicated because of the presence of iron deficiency anemia (IDA); in the older patient (77-year old), endoscopy was performed because of hematochezia without laboratory or clinical involvement. In all patients, we observed nodular, bluish, tender, wrinkled or slightly depressed lesions at the skin and gastrointestinal (GI) tract. Most severe cutaneous lesions were observed on the upper limbs of the older patient (Fig. 1). On the other hand, endoscopic examinations of patients with IDA showed many of these lesions throughout the GI tract, and the greater ones were located in the colon (Figs. 2 and 3). Because of the symptoms of these three patients were mild, they were treated with conservative measures. Outcome was favorable in all cases. BRBNS, also known as Bean's syndrome, is a rare and probably inherited disorder characterized by the presence of cutaneous hemangiomas and vascular tumors of the GI tract (1,2). The lesions in the GI tract are usually multiple and may involve any portion of the GI tract; however, the small bowel and the distal colon are most commonly affected. Moreover, skin lesions (single or multiple) usually develop on the trunk, face and upper extremities (3). Although most patients remain asymptomatic, they may suffer from chronic blood loss and therefore IDA (3,4). The diagnosis, when GI involvement is suspected, must include endoscopic techniques. Most patients respond to supportive therapy such as iron supplementation and blood transfussion when required (3,4). For chronic and recurrent bleeding, medical, endoscopic and surgical therapies have been used (3-5).

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