Blue rubber bleb nevus syndrome
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| Blue rubber bleb nevus syndrome Classification and external resources | |
| 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
External links
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
