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 features

gastrointestinal 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 features

cutaneous 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.