[Blue rubber bleb nevus (author's transl)]

Hereditary dysembryoplasia, the B. R. B. N. (Bean, 1958) is a variety of bluish nevus of the skin, associated with angioma of the gastrointestinal tract with serious bleeding, and sometimes of liver, spleen, bones, brain, marrow. The angiomas of the skin may occur in 3 forms: large cavernous angiomas; blood sac looking like a blue rubber nipple, they can be emptied; irregular blue mark, sometimes with puncted blackish spots, they may not blanch on pressure. They are painful.

glomus-like angioma cavernous angioma. Ultrastructure: cavity with endothelial cells and one to four coats of smooth muscle cells. The angiomas arose in the submucous cellular tissue, projected into the cavity of the bowel, sometimes bleeding. Hematemesis and melena may be observed as in the Rendu-Osler disease. They nver spontaneously involve but they don't degenerate. The prognosis depends essentially on the bleedings and anemia. It is possible to cut out the skin angiomas.