Blue rubber bleb nevus syndrome

Blue rubber bleb nevus syndrome
Blue rubber bleb nevus syndrome
Classification and external resources

The cutaneous vascular malformations of blue rubber bleb nevus syndrome.
ICD-10 D18
ICD-9 228.0
ICD-O: 9121/0
OMIM 112200
eMedicine derm/56

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.

It was characterized by William Bean in 1958.[1][2][3]

Contents

Presentation

BRBNS is a venous malformation,[4] formerly, though incorrectly, thought to be related to the hemangioma. It carries significant potential for serious bleeding.[5] Lesions are most commonly found on the skin and in the small intestine and distal large bowel. It usually presents soon after birth.[6] Although no genetic link has yet been determined, it has been suggested that it may represent a manifestation of familial venous malformation, and may be associated with TEK tyrosine kinase.[7][8]

Causes

The causes of this syndrome are unknown. 152 cases have been described worldwide.

Diagnosis

Visceral hemangiomas seen in blue rubber bleb nevus syndrome. The hemangiomas chiefly affect the gut (image below), are fragile, and bleed easily.

See also

References

  1. ^ synd/740 at Who Named It?
  2. ^ Bean, William Bennett (1958). Vascular spiders and related lesions of the skin. Springfield, Illinois: Charles C.Thomas. pp. 178–85. 
  3. ^ Walshe MM, Evans CD, Warin RP (Oct 1966). "Blue rubber bleb naevus". Br Med J 2 (5519): 931–2. doi:10.1136/bmj.2.5519.931. PMC 1944156. PMID 5920397. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1944156. 
  4. ^ Dobru D, Seuchea N, Dorin M, Careianu V (Sep 2004). "Blue rubber bleb syndrome: case report and literature review". Rom J Gastroenterol 13 (3): 237–40. PMID 15470538. http://www.jgld.ro/32004/32004_9.html. 
  5. ^ Ertem D, Acar Y, Kotiloglu E, Yucelten D, Pehlivanoglu E (Feb 2001). "Blue rubber bleb nevus syndrome". Pediatrics 107 (2): 418–20. doi:10.1542/peds.107.2.418. PMID 11158481. http://pediatrics.aappublications.org/cgi/pmidlookup?view=long&pmid=11158481. 
  6. ^ Kassarjian A, Fishman SJ, Fox VL, Burrows PE (Oct 2003). "Imaging characteristics of blue rubber bleb nevus syndrome". AJR Am J Roentgenol 181 (4): 1041–8. PMID 14500226. http://www.ajronline.org/cgi/pmidlookup?view=long&pmid=14500226. 
  7. ^ Online 'Mendelian Inheritance in Man' (OMIM) BLUE RUBBER BLEB NEVUS -112200
  8. ^ Gallione CJ, Pasyk KA, Boon LM et al. (Mar 1995). "A gene for familial venous malformations maps to chromosome 9p in a second large kindred". J. Med. Genet. 32 (3): 197–9. doi:10.1136/jmg.32.3.197. PMC 1050316. PMID 7783168. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1050316. 

External links

v · d · eVascular tissue neoplasm (ICD-O 9120–9179) (C49+C46/D18, 171+176/215)
Blood
Hemangioma/hemangiosarcoma · Blue rubber bleb nevus syndrome · Hemangioendothelioma (Infantile hemangioendothelioma · Endovascular papillary hemangioendothelioma · Epithelioid hemangioendothelioma · Spindle cell hemangioendothelioma · Composite hemangioendothelioma · Proliferating angioendotheliomatosis · Retiform hemangioendothelioma) · Hemangiopericytoma · Angiokeratoma · Capillary hemangioma (Hemangioblastoma) · Cavernous hemangioma · Venous lake
Kaposi's sarcoma: African cutaneous Kaposi sarcoma · African lymphadenopathic Kaposi sarcoma · AIDS-associated Kaposi sarcoma · Classic Kaposi sarcoma · Immunosuppression-associated Kaposi sarcoma · Kaposiform hemangioendothelioma
Angioma serpiginosum · Cherry angioma · Glomeruloid hemangioma · Microvenular hemangioma · Spider angioma · Targetoid hemosiderotic hemangioma · Tufted angioma · Universal angiomatosis

Pyogenic granuloma
Lymphatic
Multifocal lymphangioendotheliomatosis · Acquired progressive lymphangioma · Lymphangiomatosis
Either
Angioma/angiosarcoma · Angiofibroma

M: VAS

anat(a:h/u/t/a/l,v:h/u/t/a/l)/phys/devp/cell/prot

noco/syva/cong/lyvd/tumr, sysi/epon, injr

proc, drug(C2s+n/3/4/5/7/8/9)



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