Blue rubber bleb nevus syndrome

The Blue -Rubber- bleb nevus syndrome, also known as the German Blue rubber bleb syndrome, or Bean syndrome is characterized by multiple benign tumors of the blood vessels (known as hemangiomas ), which occur in the skin and internal organs. It is a hereditary disease with autosomal dominant inheritance, the exact pathogenesis is currently unknown.

Diagnostic criteria and appearance

The Blue -Rubber- bleb nevus syndrome usually occurs congenitally or in early childhood. It is characterized by multiple deep blue nodes, which can be found anywhere on the skin. The gummy consistency is responsible for the name. Histologically, there is cavernous hemangiomas. These masses may also be visceral, so in the bowels occur. The most commonly affected here is the small intestine, but other organs may be affected.

Spontaneous there is no improvement. Malignant degeneration does not occur. Nevertheless, the patients are often severely limited due to the multiple infestation, CNS manifestations may lead to neurological problems, frequent bleeding lead to chronic anemia, and acute bleeding leading to death before coming.