Blue rubber bleb nevus syndrome
(or "BRBNS", or "blue rubber bleb syndrome, or "blue rubber-bleb nevus", or "Bean syndrome") is a rare disorder
that consists mainly of abnormal blood vessels
affecting the gastrointestinal tract
It was characterized by William Bean in 1958.
BRBNS is a venous
malformation, formerly, though incorrectly, thought to be related to the hemangioma
. It carries significant potential for serious bleeding. Lesions
are most commonly found on the skin
and in the small intestine
and distal large bowel
. It usually presents soon after birth. Although no genetic link has yet been determined, it has been suggested that it may represent a manifestation of familial venous malformation
, and may be associated with TEK tyrosine kinase
The causes of this syndrome are unknown. Not more than a few hundred cases have been described worldwide.