blue rubber bleb nevus

Blue rubber bleb nevus syndrome

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.

Presentation

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.

Causes

The causes of this syndrome are unknown. Not more than a few hundred cases have been described worldwide.

References

External links

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