FaCD Online Syndrome Fact Sheet
Last updated: 03 Jul 2009
Name: Blue Rubber Bleb Nevus syndrome
Synonym: Bean syndrome, BRBNS
Mode of Inheritance: AD/ spor
Tumor featuresgastrointestinal polypoid hemangiomas hepatic hemangioma joint capsules, hemangioma in lung hemangioma splenic hemangioma subcutaneous hemangioma
Tumor features (possible)leukemia, chronic lymphocytic (CLL) medulloblastoma osteomas renal cell cancer
Non-tumor featurescutaneous nevi , hemangiomatous (blue rubber blebs)
Comment
The hallmarks of this disease are the presence of multiple bladder-like hemangiomas (blue rubber blebs) on the skin, especially on the trunk and arms. The intestinal polypous hemangiomas may cause severe bleeding and obstruction. Hemangiomas may be found in a wide range of tissues (spleen, lungs, joint capsules etc.)[1-3,6-8].
Chronic lymphocytic leukemia (at age 66) and renal cell cancer (at age 57) was reported in a male with this disorder[4]. Medulloblastoma was reported in a 21-year old affected woman[5]. Osteoid osteomas have been reported in a BRBNS patient as well[9].
This condition may be identical to Familial Venous Malformation Syndrome
Links
eMedicine text on Blue Rubber Bleb nevus syndrome 24 1 08
References
[1] Laugier P. [Blue rubber bleb nevus (author's transl)]. Ann Dermatol Venereol 1978; 105(8-9):729-731.
[2] Munkvad M. Blue rubber bleb nevus syndrome. Dermatologica 1983; 167:307-309.
[3] Morris SJ, Kaplan SR, Ballan K, Tadesco FJ. Blue rubber-bleb nevus syndrome. JAMA 1978; 239(18):1887.
[4] Hoffman T, Chasko S, Safai B. Association of blue rubber bleb nevus syndrome with chronic lymphocytic leukemia and hypernephroma. Johns Hopkins Med J 1978; 142:91-94.
[5] Rice JS, Fischer DS. Blue rubber-bleb nevus syndrome. Arch Dermatol 1962; 86:503-511.
[6] Deng ZH, Xu CD, Chen SN. Diagnosis and treatment of blue rubber bleb nevus syndrome in children. World journal of pediatrics : WJP 2008; 4(1):70-3.
[7] Fishman SJ, Smithers CJ, Folkman J, Lund DP, Burrows PE, Mulliken JB, Fox VL. Blue rubber bleb nevus syndrome: surgical eradication of gastrointestinal bleeding. Annals of surgery 2005; 241(3):523-8.
[8] Feingold RM. The blue rubber nevus syndrome. J Insur Med. 2009;41(1):67-71
[9] Palleschi GM, Torchia D, Fabbri P. Blue rubber-bleb nevus syndrome: report of a case associated with osteoid osteomas. The Journal of dermatology 2005; 32(7):589-93.
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