Brbns is a rare syndrome and should be considered in the presence of multifocal hemangiomas preferably in the skin and gastrointestinal tract.
Blue rubber nevus syndrome skin.
Blue rubber bleb nevus syndrome brbns sometimes called bean syndrome is a rare congenital vascular anomaly in which malformed veins or blebs appear on the skin and surfaces of internal organs.
The malformed blood vessels appear as a spot or lesion called a nevus.
It was first described by gascoyen in 1806 but in 1958 william bennet bean coined the term blue rubber nevus syndrome for its color and consistency 1 2 only about 200 hundred cases were.
The tendency to the syndrome is passed on to half of one s children.
Brbns is an uncommon disorder that is characterized by cutaneous and gi hemangiomas with.
Blue rubber bleb nevus syndrome brbns also called bean s syndrome is a rare disease associated with multiple venous malformations in the skin and gastrointestinal gi tract.
Dermatological lesions which are the first clinically visible manifestations appear as skin colored compressible protub.
Bean first used the term blue rubber bleb nevus syndrome brbns which was derived from cutaneous hemangiomas having the feeling and look of rubber nipples.
Lesions in the gastrointestinal tract frequently become apparent.
This condition was initially discovered in 1860 by gascoyen.
The blue rubber bleb naevus syndrome or bean syndrome is a rare form of venous malformation in which there are skin and gastrointestinal tract lesions.
Given the clinical and imagiological features we established the diagnosis of a sporadic type of blue rubber bleb nevus syndrome brbns.
These small purple lesions are particularly common in the gastrointestinal gi tract.
There is frequently a family history with autosomal dominant inheritance i e.
The disease is characterized by the presence of fluid filled blisters as visible circumscribed chronic lesions nevus it was described by william bean in 1958.
Multiple distinctive skin lesions are usually characteristic of this disorder and are often present at birth or present during early childhood.
Blue rubber bleb nevus syndrome brbns is a rare condition that is characterized by numerous malformations of the venous system that significantly involve the skin and visceral organs.
The underlying blood vessel malformations are present from birth even though the nevus may not be visible until later in life.
Gascoyen first reported the association of cutaneous nevi and intestinal lesions with gastrointestinal gi bleeding in 1860.
However it was later made famous in 1958 by william bennett bean for which the disease has been termed bean syndrome later referenced as.
Blue rubber bleb nevus syndrome is a condition in which the blood vessels do not develop properly in an area of the skin or other body organ particularly the intestines.
