eMedicine Specialties > Dermatology > Diseases of the Vessels

Blue Rubber Bleb Nevus Syndrome: Treatment & Medication

Author: Basil S Cherpelis, MD, Assistant Professor, Chief of Dermatologic Surgery, Department of Dermatology and Cutaneous Surgery, Assistant Professor, Department of Oncologic Sciences, University of South Florida; Consulting Staff, Moffitt Cancer Center, Tampa General Hospital and James A Haley Veterans Affairs Medical Center
Coauthor(s): Neil A Fenske, MD, Director of Dermatology and Cutaneous Surgery, Professor, Departments of Internal Medicine and Pathology, University of South Florida College of Medicine
Contributor Information and Disclosures

Updated: Oct 23, 2008

Treatment

Medical Care

No systemic therapy is currently considered a standard of care for blue rubber bleb nevus syndrome (BRBNS). One report indicated the successful use of interferon-beta to treat the manifestations of disseminated intravascular coagulation in one patient with disseminated skin and GI venous malformations.12

Surgical Care

  • Cutaneous lesions
    • Cutaneous lesions do not need to be treated, unless they are cosmetically unacceptable or functionally troublesome. Malignant transformation of BRBNS lesions has not been reported.
    • Methods to remove lesions include electrodesiccation and curettage, liquid nitrogen, and excision. These modalities often lead to recurrence or healing with a hypertrophic scar. Successful treatment without scarring has been reported with the use of the carbon dioxide laser. Treatment of 225 lesions without recurrence was reported with the use of the carbon dioxide laser.
    • The Medscape Dermatologic Surgery Resource Center may be of interest.
  • Extracutaneous lesions
    • Bleeding from GI lesions usually is managed conservatively with iron supplementation and blood transfusions when necessary. Endoscopic coagulation or removal is an effective modality for repeated bleeding. Experience with endoscopic sclerotherapy suggests that it is ineffective and complicated by the development of ulcerations and strictures. Endoscopic laser (Nd:YAG) photocoagulation has been used successfully for lesions in the alimentary tract.13 When traditional methods fail and the vascular lesions are confined to a segment of the GI tract, resection of the involved segment of gut may be indicated. This approach should be used with caution because recurrences may occur after excision. Diffuse and scattered lesions or those where operation is not feasible should be treated conservatively.
    • Orthopedic lesions are managed with orthotics and supportive measures, unless impaired function necessitates surgery.
    • An 8-month old infant with bleeding hemangiomas of the rectum and colon was successfully treated with argon plasma coagulation. This treatment resulted in long-term success.14
    • In one report, an aggressive surgical approach to treatment was attempted in 10 patients. GI venous malformations were identified endoscopically and then treated by wedge resection, polypectomy, suture ligation, segmental bowel resection, or band ligation. Only one patient developed recurrent GI bleeding.15

Consultations

  • Patients should be referred to a gastroenterologist for management of GI manifestations as needed.
  • Referral to an orthopedist can assist in the care of patients with bone or joint involvement.
  • Symptomatic patients should be referred to the appropriate specialist depending on the organ system involved.
  • In cases with familial occurrence, patients should be referred for genetic counseling.

More on Blue Rubber Bleb Nevus Syndrome

Overview: Blue Rubber Bleb Nevus Syndrome
Differential Diagnoses & Workup: Blue Rubber Bleb Nevus Syndrome
Treatment & Medication: Blue Rubber Bleb Nevus Syndrome
Follow-up: Blue Rubber Bleb Nevus Syndrome
Multimedia: Blue Rubber Bleb Nevus Syndrome
References

References

  1. Park CO, Park J, Chung KY. Blue rubber bleb nevus syndrome with central nervous system involvement. J Dermatol. Sep 2006;33(9):649-51. [Medline].

  2. Fleischer AB Jr, Panzer SM, Wheeler CE. Blue rubber bleb nevus syndrome in a black patient: a case report. Cutis. Feb 1990;45(2):103-5. [Medline].

  3. Shannon J, Auld J. Blue rubber bleb naevus syndrome associated with cortical blindness. Australas J Dermatol. Aug 2005;46(3):192-5. [Medline].

  4. Starr BM, Katzenmeyer WK, Guinto F, Pou AM. The blue rubber bleb nevus syndrome: a case with prominent head and neck findings. Am J Otolaryngol. Jul-Aug 2005;26(4):282-4. [Medline].

  5. Mejía-Rodríguez S, Valencia-Herrera A, Escobar-Sánchez A, Mena-Cedillos C. Dermoscopic features in Bean (blue rubber bleb nevus) syndrome. Pediatr Dermatol. Mar-Apr 2008;25(2):270-2. [Medline].

  6. McCarthy JC, Goldberg MJ, Zimbler S. Orthopaedic dysfunction in the blue rubber-bleb nevus syndrome. J Bone Joint Surg Am. Feb 1982;64(2):280-3. [Medline].

  7. Garen PD, Sahn EE. Spinal cord compression in blue rubber bleb nevus syndrome. Arch Dermatol. Jul 1994;130(7):934-5. [Medline].

  8. Giordano C, Battagliese A, di Gioia CR, Campagna D, Benedetti F, Travaglini C, et al. Blue rubber bleb nevus syndrome and pulmonary hypertension: an unusual association. Cardiovasc Pathol. Nov-Dec 2004;13(6):317-22. [Medline].

  9. Yarlagadda R, Menda Y, Graham MM. Tc-99m red blood cell imaging in a patient with blue rubber bleb nevus syndrome. Clin Nucl Med. May 2008;33(5):374-6. [Medline].

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

  11. Gallo SH, McClave SA. Blue rubber bleb nevus syndrome: gastrointestinal involvement and its endoscopic presentation. Gastrointest Endosc. Jan-Feb 1992;38(1):72-6. [Medline].

  12. Apak H, Celkan T, Ozkan A, Yildiz I, Aydemir EH, Ozdil S, et al. Blue rubber bleb nevus syndrome associated with consumption coagulopathy: treatment with interferon. Dermatology. 2004;208(4):345-8. [Medline].

  13. Morris L, Lynch PM, Gleason WA Jr, Schauder C, Pinkel D, Duvic M. Blue rubber bleb nevus syndrome: laser photocoagulation of colonic hemangiomas in a child with microcytic anemia. Pediatr Dermatol. Jun 1992;9(2):91-4. [Medline].

  14. Ng WT, Kong CK. Argon plasma coagulation for blue rubber bleb nevus syndrome in a female infant. Eur J Pediatr Surg. Apr 2003;13(2):137-9. [Medline].

  15. Fishman SJ, Smithers CJ, Folkman J, Lund DP, Burrows PE, Mulliken JB, et al. Blue rubber bleb nevus syndrome: surgical eradication of gastrointestinal bleeding. Ann Surg. Mar 2005;241(3):523-8. [Medline].

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

  17. Carr MM, Jamieson CG, Lal G. Blue rubber bleb nevus syndrome. Can J Surg. Feb 1996;39(1):59-62. [Medline].

  18. Ertem D, Acar Y, Kotiloglu E, Yucelten D, Pehlivanoglu E. Blue rubber bleb nevus syndrome. Pediatrics. Feb 2001;107(2):418-20. [Medline].

  19. Gonzalez D, Elizondo BJ, Haslag S, Buchanan G, Burdick JS, Guzzetta PC, et al. Chronic subcutaneous octreotide decreases gastrointestinal blood loss in blue rubber-bleb nevus syndrome. J Pediatr Gastroenterol Nutr. Aug 2001;33(2):183-8. [Medline].

  20. Hofhuis WJ, Oranje AP, Bouquet J, Sinaasappel M. Blue rubber-bleb naevus syndrome: report of a case with consumption coagulopathy complicated by manifest thrombosis. Eur J Pediatr. May 1990;149(8):526-8. [Medline].

  21. Jorizzo JR, Amparo EG. MR imaging of blue rubber bleb nevus syndrome. J Comput Assist Tomogr. Jul-Aug 1986;10(4):686-8. [Medline].

  22. Lee S, Rafii AA, Sykes J. Advances in scalp reconstruction. Curr Opin Otolaryngol Head Neck Surg. Aug 2006;14(4):249-53. [Medline].

  23. Moodley M, Ramdial P. Blue rubber bleb nevus syndrome: case report and review of the literature. Pediatrics. Jul 1993;92(1):160-2. [Medline].

  24. Munkvad M. Blue rubber bleb nevus syndrome. Dermatologica. 1983;167(6):307-9. [Medline].

  25. Oksüzoglu BC, Oksüzoglu G, Cakir U, Bayir T, Esen M. Blue rubber bleb nevus syndrome. Am J Gastroenterol. Apr 1996;91(4):780-2. [Medline].

  26. Olsen TG, Milroy SK, Goldman L, Fidler JP. Laser surgery for blue rubber bleb nevus. Arch Dermatol. Jan 1979;115(1):81-2. [Medline].

  27. Oranje AP. Blue rubber bleb nevus syndrome. Pediatr Dermatol. Sep 1986;3(4):304-10. [Medline].

  28. Paules S, Baack B, Levisohn D. Tender bluish papules on the trunk and extremities. Blue rubber-bleb nevus syndrome. Arch Dermatol. Nov 1993;129(11):1505-6, 1508-9. [Medline].

  29. Place RJ. Blue rubber bleb nevus syndrome: a case report with long-term follow-up. Mil Med. Aug 2001;166(8):728-30. [Medline].

  30. Rice JS, Fischer DS. Blue rubber-bleb nevus syndrome. Generalized cavernous hemangiomatosis or venous hamartoma with medulloblastoma of the cerebellum: case report and review of the literature. Arch Dermatol. Oct 1962;86:503-11. [Medline].

Further Reading

Keywords

blue rubber bleb nevus syndrome, BRBNS, Bean syndrome

Contributor Information and Disclosures

Author

Basil S Cherpelis, MD, Assistant Professor, Chief of Dermatologic Surgery, Department of Dermatology and Cutaneous Surgery, Assistant Professor, Department of Oncologic Sciences, University of South Florida; Consulting Staff, Moffitt Cancer Center, Tampa General Hospital and James A Haley Veterans Affairs Medical Center
Basil S Cherpelis, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American Society for Dermatologic Surgery, and Association of Professors of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Neil A Fenske, MD, Director of Dermatology and Cutaneous Surgery, Professor, Departments of Internal Medicine and Pathology, University of South Florida College of Medicine
Disclosure: Nothing to disclose.

Medical Editor

Bernice R Krafchik, MBChB, FRCPC, Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto
Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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