Blue rubber bleb nevus syndrome

Blue Rubber Bleb Nevus Syndrome, also known as blue rubber-bleb nevus syndrome, is an independent syndrome. Blue rubber bleb nevus syndrome is mainly characterized by the coexistence of hemangiomas in the skin and intestines, with onset in childhood and a tendency not to regress spontaneously. It commonly occurs on the trunk and extremities. The skin lesions are blue subcutaneous nodules, soft like rubber, tender, generally 3-4 cm in diameter, and may be single or multiple in number. Gastrointestinal involvement presents with melena, which can cause anemia. Histopathology resembles cavernous hemangioma; intestinal lesions are more common in the small intestine and may be located beneath the mucosa. Skin lesions generally do not affect health, but surgical treatment is indicated for those with significant intestinal bleeding. It was first described by Bean in 1958, hence the name Bean syndrome. This syndrome results from abnormal differentiation and development during the embryonic period and is an autosomal dominant hereditary disease.