Preferred Label : Blue rubber bleb naevus syndrome;
ICD-11 definition : Blue rubber bleb nevus (BRBNS) is a rare vascular malformation disorder with cutaneous
and visceral lesions frequently associated with serious, potentially fatal bleeding
and anaemia. Multifocal venous malformations most commonly involve the skin and gastrointestinal
tract but can also affect the brain, kidneys, lungs, eyes, bones and other organs.
Consumption coagulopathy may occur. The lesions, which are generally present in childhood
but can develop later in life, are usually blue coloured and easily compressible with
light palpation. The main clinical manifestation is acute or chronic bleeding from
the multiple GIT vascular malformations.;
ICD-11 synonym : Bean syndrome (MIM 112200); Bean syndrome;
Origin ID : 1112312815;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Blue rubber bleb nevus (BRBNS) is a rare vascular malformation disorder with cutaneous
and visceral lesions frequently associated with serious, potentially fatal bleeding
and anaemia. Multifocal venous malformations most commonly involve the skin and gastrointestinal
tract but can also affect the brain, kidneys, lungs, eyes, bones and other organs.
Consumption coagulopathy may occur. The lesions, which are generally present in childhood
but can develop later in life, are usually blue coloured and easily compressible with
light palpation. The main clinical manifestation is acute or chronic bleeding from
the multiple GIT vascular malformations.
