Blue rubber bleb nevus syndrome
| Blue rubber bleb nevus syndrome | |
| ICD-10 | D18 |
|---|---|
| ICD-9 | 228.0 |
| ICD-O: | 9121/0 |
| eMedicine | derm/56 |
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Overview
Blue rubber bleb nevus syndrome (or "BRBNS", or "blue rubber bleb syndrome, or "blue rubber-bleb nevus", or "Bean syndrome") is a rare disorder that consists mainly of abnormal blood vessels affecting the gastrointestinal tract.
Presentation
BRBNS is a venous malformation, formerly, though incorrectly, thought to be related to the hemangioma. It carries significant potential for serious or fatal bleeding. Lesions are most commonly found on the skin and in the small intestine and distal large bowel.
Causes
The causes of this syndrome are unknown. Not more than a few hundred cases have been described worldwide.
History
It was first described by Gascoyen in 1860.[1] In 1958 William Bennett Bean described the lesions further and came up with the term BRBNS, chosen because the 'cutaneous hemangiomas have the look and feel of rubber nipples'.[2]
References
- ↑ Gascoyen GG. Case of naevus involving the parotid gland and causing death from suffocation: naevi of the viscera. Trans Pathol Soc London 1860;11:267.
- ↑ Bean WB. Bleeding from the butt in rare disorders with diagnostic lesions of the skin and mucous membranes. In: Bean WB, editor. Rare diseased and lesions: their contributions to clinical medicine. Springfield (IL): Charles C. Thomas; 1967. p. 3-29.
Additional Resources
- Moodley M, Ramdial P (1993). "Blue rubber bleb nevus syndrome: case report and review of the literature". Pediatrics. 92 (1): 160–2. PMID 8516068
- Dobru D, Seuchea N, Dorin M, Careianu V (2004). "Blue rubber bleb nevus syndrome: case report and literature review". Rom J Gastroenterol. 13 (3): 237–40. PMID 15470538
- Dwivedi M, Misra SP (2002). "Blue rubber bleb nevus syndrome causing upper GI hemorrhage: a novel management approach and review". Gastrointest Endosc. 55 (7): 943–6. PMID 12024162