Blue Rubber Bleb Nevus syndrome

National Organization for Rare Disorders, Inc.

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It is possible that the main title of the report Blue Rubber Bleb Nevus syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.


  • Bean syndrome

Disorder Subdivisions

  • None

General Discussion

Blue rubber bleb nevus syndrome (BRBNS) is a rare blood vessel (vascular) disorder that affects the skin and internal organs of the body. Multiple distinctive skin lesions are usually characteristic of this disorder and are often present at birth or present during early childhood. Lesions in the gastrointestinal tract frequently become apparent during childhood or early adulthood. The lesions are multifocal venous malformations, resulting from abnormal embryonic blood vessel development.


BRBNS is characterized by soft, elevated lesions on the skin or just under the skin that are dark blue, red, purple-red or black in color. The venous malformations may be tender, contain blood and be easily compressed and are usually located on the upper limbs, trunk and soles of the feet but can occur anywhere. The lesions increase in size and become more apparent over time but have not been reported to become cancerous. The organ system most commonly affected by BRBNS is the gastrointestinal (GI) tract, particularly the small intestine. The lesions in the GI tract often bleed and can lead to mild or severe anemia. Iron replacement and/or frequent blood transfusions may be required. The GI lesions can also cause an obstruction or blockage (intussusception) of part of the bowel. Skeletal abnormalities and venous malformations in muscle are sometimes associated with BRBNS.


Blue rubber bleb nevus syndrome is sporadic. Although families have been described in which the condition follows autosomal dominant inheritance, these families actually have other multifocal venous malformations.

Affected Populations

Blue Rubber Bleb Nevus Syndrome affects males and females in equal numbers. Approximately 150 cases have been reported in the medical literature.

Standard Therapies


BRBNS is diagnosed by physical examination and a procedure in which the GI tract is illuminated and visualized (endoscopy). Genetic testing for BRBNS is available on a research basis only.


Iron therapy and blood transfusions are used to conservatively manage BRBNS. The skin lesions associated with BRBNS can be treated with laser therapy, injection of chemicals that collapse the lesion (sclerotherapy) or surgical removal. Lesions in the gastrointestinal system are usually not removed unless bleeding leads to anemia and necessitates repeated blood transfusions. Gastrointestinal lesions can be safely removed surgically, but one or several lengthy operations may be required.

Investigational Therapies

Argon plasma coagulation and octreotide acetate have been described in individual cases to reduce GI bleeding, but there are no cases of prolonged durable eradication of bleeding.

Information on current clinical trials is posted on the Internet at All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222

TTY: (866) 411-1010


For information about clinical trials sponsored by private sources, contact:

Contact for additional information about blue rubber bleb nevus syndrome:

Steven J. Fishman, MD

Stuart and Jane Weitzman Family Chair in Surgery

Co-Director, Vascular Anomalies Center

Department of Surgery

Children's Hospital Boston

617-355-3040 (office)

617-730-0752 (FAX)



Fishman SJ, Mulliken JB. Blue Rubber Bleb Nevus Syndrome. In: The NORD Guide to Rare Disorders, Philadelphia: Lippincott, Williams and Wilkins; 2003:160.


Fishman SJ, Smithers CJ, Folkman J, et al: Blue rubber bleb nevus syndrome: surgical eradication of gastrointestinal bleeding. Ann Surg. 2005;5241(3): 523-8.

Apak H, Celkan T, Ozkan A, et al: Blue rubber bleb nevus syndrome associated with consumption coagulopathy: treatment with interferon. Dermatology. 2004;208:345-8.

Ng WT, Kong CK: Argon plasm coagulation for blue rubber bleb nevus syndrome in a female infant. Eur J Pediatr Surg. 2003; 13: 137-9.

Andersen JM. Blue Rubber Bleb Nevus Syndrome. Curr Treat Options Gastroenterol. 2001;4(5):433-440.

Ertem D, Acar Y, Kotiloglu E, et al: Blue rubber bleb nevus syndrome. Pediatrics. 2001; 107(2):418-20.

Gonzalez D, Elizondo BJ, Haslag S, et al: Chronic subcutaneous octreotide decreases gastrointestinal blood loss in blue rubber-bleb nevus syndrome. J Pediatr Gastroenterol Nutr. 2001;33(2): 183-8.

Bak YT, Oh CH, Kim JH, Lee CH: Blue rubber bleb nevus syndrome: endoscopic removal of the gastrointestinal hemangiomas. Gastrointest Endosc. 1997;45(1):90-2.

Morris L, Lynch PM, Gleason WA Jr, et al: Blue rubber bleb nevus syndrome: laser photocoagulation of colonic hemangiomas in a child with microcytic anemia. Pediatr Dermatol. 1992;9(2): 91-4.


Cherpelis BS. Blue Rubber Bleb Nevus Syndrome. eMedicine. Updated April 9, 2010. Accessed February 14, 2012.


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NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

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Genetic and Rare Diseases (GARD) Information Center

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National Organization of Vascular Anomalies

PO Box 38216

Greensboro, NC 27438-8216



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see