Blue rubber bleb nevus syndrome

Blue rubber bleb nevus syndrome (BRBNS) or Bean syndrome, is a rare sporadic syndrome characterized by multifocal venous anomalies. Patients often have multiple soft blue skin lesions (blueberry muffin syndrome) associated with multiple bowel venous malformations, which could lead to lower gastrointestinal bleeding

The skin and gastrointestinal tract are the primary sites of involvement. Multiple other organ systems can be affected, including the musculoskeletal system, vulva, submandibular region, parotid region, thymus, masticator space, and cranium (sinus pericranii). There are rare reports of involvement of the orbits, thyroid, heart, pericardium, lungs, peritoneal cavity, adrenal glands, and kidneys 3.

The association of “hemangiomas” of the skin and gastrointestinal tract was first reported in 1860 by Gascoyen. The condition was fully characterized by, the American physician and polymath William "Bill" Bennet Bean (1909-1989) 6 in 1958 2.

Vascular tumours and malformations
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Article information

rID: 44141
System: Vascular
Section: Syndromes
Synonyms or Alternate Spellings:
  • Bean syndrome
  • Bean's syndrome
  • Blue rubber bleb nevus syndrome
  • Blue rubber bleb nevus syndrome (BRBNS)
  • BRBNS

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