eMedicine Specialties > Dermatology > Diseases of the Vessels

Blue Rubber Bleb Nevus Syndrome: Differential Diagnoses & Workup

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

Differential Diagnoses

Kaposi Sarcoma
Klippel-Trenaunay-Weber Syndrome
Maffucci Syndrome
Venous Lakes

Other Problems to Be Considered

Arteriovenous malformation
Capillary-lymphatic malformation

Workup

Laboratory Studies

  • Fecal occult blood test: Screen for occult blood loss from GI blue rubber bleb nevus syndrome (BRBNS) lesions.
  • CBC count: Screen for iron deficiency anemia.
  • Urinalysis: Hematuria may be caused by lesions in the bladder.

Imaging Studies

  • Radiographic images may be useful in suspected bone or joint involvement to detect fractures, bony overgrowth, and articular derangement. Radiographic contrast techniques may detect GI lesions, but endoscopy is considered to be superior.
  • MRI has been described as a useful tool for detecting extracutaneous lesions and for screening asymptomatic family members.
  • Technetium Tc-99m – labeled red blood cell imaging may be helpful to localize or delineate the extent of bleeding in patients with BRBNS.9

Procedures

  • Endoscopy
    • GI lesions may be demonstrated by endoscopic examination or by radiographic contrast techniques.
    • Upper GI endoscopy is more sensitive than an upper GI series and colonoscopy more useful than a barium enema.
    • Endoscopy also provides the opportunity to treat and diagnose the lesions.10,11

Histologic Findings

Histopathologic examination of skin lesions reveals vascular tissue with tortuous, blood-filled ectatic vessels, lined by a single layer of endothelium, with surrounding thin connective tissue. Dystrophic calcification may be present.

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
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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].

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Further Reading

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Keywords

blue rubber bleb nevus syndrome, BRBNS, Bean syndrome

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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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