Blue rubber bleb nevus syndrome

Last revised by Daniel J Bell on 28 Sep 2019

Citation, DOI, disclosures and article data

Citation:

Weerakkody Y, Bell D, Glick Y, et al. Blue rubber bleb nevus syndrome. Reference article, Radiopaedia.org (Accessed on 19 Apr 2024) https://doi.org/10.53347/rID-44141

DOI:

https://doi.org/10.53347/rID-44141

Permalink:

https://radiopaedia.org/articles/44141

rID:

44141

Article created:

9 Apr 2016, Yuranga Weerakkody ◉

Disclosures:

At the time the article was created Yuranga Weerakkody had no recorded disclosures.

View Yuranga Weerakkody's current disclosures

Last revised:

28 Sep 2019, Daniel J Bell ◉

Disclosures:

At the time the article was last revised Daniel J Bell had no recorded disclosures.

View Daniel J Bell's current disclosures

Revisions:

12 times, by 7 contributors - see full revision history and disclosures

Systems:

Vascular

Sections:

Syndromes

Tags:

cases, snippet title

Synonyms:

Bean syndrome
Bean's syndrome
Blue rubber bleb nevus syndrome
Blue rubber bleb nevus syndrome (BRBNS)
BRBNS

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.

Differential diagnosis

hereditary hemorrhagic telangiectasia
- multiple telangiectasias, pulmonary arteriovenous malformations
- epistaxis
- hereditary, positive family history
Maffucci syndrome
- multiple enchondromas
Klippel-Trénaunay syndrome
- port wine stain capillary hemangiomas
- varicosities
- localized gigantism

History and etymology

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.