Catalog

Blue rubber bleb naevus syndrome

No MMS code

Link to WHO Definition

Blue rubber bleb naevus syndrome – http://id.who.int/icd/entity/1112312815

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.