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Blue Rubber Bleb Nevus Syndrome Treatment & Management

  • Author: Basil S Cherpelis, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jul 14, 2016
 

Medical Care

The literature suggests no standard of care or systemic treatment regimen is available for blue rubber bleb nevus syndrome (BRBNS). One report of BRBNS 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.[26] Another report describes long-term subcutaneous octreotide therapy as successful as decreasing GI bleeding in BRBNS.[27]  One report describes successful treatment with oral sirolimus.[28]

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Surgical Care

Surgical treatment of cutaneous lesions in blue rubber bleb nevus syndrome (BRBNS)

The treatment of cutaneous lesions is not usually required. Treatment is rendered for cosmetic purposes or for the management of symptomatic lesions. Malignant transformation of BRBNS lesions has not been reported.

Methods to remove lesions include electrodesiccation and curettage, liquid nitrogen, and excision. These treatment options can lead to recurrent skin lesions and abnormal healing, such as hypertrophic scars. 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.[29]

Surgical treatment of extracutaneous lesions in BRBNS

Bleeding from GI lesions is usually 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. Other therapeutic management modalities for BRBNS include Nd:YAG laser, bipolar or argon plasma coagulation, band ligation, sclerotherapy and snare resection.[30, 31]

One study even reported the successful treatment of mucocutaneous lesions of BRBNS with NdYag.[32]

The literature suggests treatment options include glubran 2 injections, which have been successfully used in the management of gastric varices.

Other treatments described include endoscopic obliteration of cavernous hemangiomas with n-butyl-2-cyanoacrylate (Histoacryl).[30]

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.[33]

In one report, an aggressive surgical approach to the treatment of blue rubber bleb nevus syndrome 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.[34]

Multiple hemangiomas of the terminal ileum and colon were treated endoscopically in an adult female with the argon plasma coagulator.[35]

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Consultations

Patients with blue rubber bleb nevus syndrome (BRBNS) should be referred to a gastroenterologist for management of GI manifestations.

Orthopedics should be consulted to assist in the care management of bone or joint related complications.

Additional referrals should be made to the appropriate specialists and treatment teams depending on the extent and nature of organ system involvement.

Genetic testing and counseling should be considered in cases with familial occurrence.

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Long-Term Monitoring

The formation of new GI lesions in blue rubber bleb nevus syndrome (BRBNS) may continue to occur; therefore, periodic GI and hematologic surveillance studies should be instituted.

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Contributor Information and Disclosures
Author

Basil S Cherpelis, MD Associate Professor, Chief of Dermatologic Surgery, Department of Dermatology and Cutaneous Surgery, Associate Professor, Department of Oncologic Sciences, University of South Florida College of Medicine; 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 Surgery, American Society for Dermatologic Surgery, Association of Professors of Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Neil Alan Fenske, MD Chairman, Department of Dermatology and Cutaneous Surgery, Professor, Department of Dermatology and Cutaneous Surgery, Department of Pathology and Cell Biology, Department of Oncologic Sciences, Medical Director, Health Cosmetic and Laser Center, University of South Florida College of Medicine

Disclosure: Received none from Abbvie for speaking and teaching; Received none from Valeant for speaking and teaching.

Hoka Lisa Nyanda, MD Academic Chief Resident, Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine

Hoka Lisa Nyanda, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, American Medical Student Association/Foundation, Florida Medical Association, Southern Medical Association, Society for Pediatric Dermatology, Florida Chapter of The American Academy of Pediatrics, Florida Pediatric Society, Student National Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Van Perry, MD Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Multiple scattered blue to black rubbery papules and nodules involving the mid-chest region.
Purple to blue/black papules involving the upper and lower lips.
Multiple blue to black pigmented, rubbery, blood-filled sacs, which are easily compressible involving the GI tract.
Lower extremity cutaneous lesions described in blue rubber bleb nevus syndrome, consisting of blue rubbery papules and nodules with an easily compressible smooth surface.
Histopathology reveals blood-filled vessels, composed of single layers of endothelium, surrounded by connective tissue.
 
 
 
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