Síndrome de blue rubber bleb nevus ou síndrome de Bean. Causa rara de hemorragia digestiva.

Abstract

A 71-year-old male who had previously suffered from chronic alcoholism was admitted to the Internal Medicine Service of Coimbra Hospital Center in January 1996 due to asthenia, loss of weight, icterus and abdominal pain, clinical features that had begun six months before admittance to hospital. A physical examination revealed that, in addition to icterus, the patient presented multiple hemangiomas of 1 to 5 cm in diameter, located in the oral cavity, neck, breast and left axilla. These lesions were bluish, elevated and with a rubber-nipple consistency, and had been developing for about 15 years. Subsequent examination revealed normocytic normochromic anemia, cholestatic icterus and the existence of a gastrointestinal hemangioma located in the esophagus. Excisional biopsy of an element proved that it was cavernous hemangioma. A subsequent angio-scintigraphy indicated other aspects suggestive of deep hemangiomas located in the legs, face and cervical region. The authors had the opportunity of examining other members of the patient's family, who apparently did not exhibit similar lesions. They concluded that it was a case of blue rubber bleb nevus syndrome (BRBNS), probably in its sporadic form. Treatment was essentially conservative and the patient is well.