Dermatologic Aspects of Behcet Disease Treatment & Management

  • Author: Marjan Yousefi, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Dec 23, 2010
 

Medical Care

Although multiple therapeutic modalities have been used, treatment is far from satisfactory. Treatment of Behçet disease seems to be symptomatic and empiric. Therapeutic efficacy has been difficult to evaluate because of the variable course of the disease and the limited number of cases for clinical investigation.

  • Local therapy
    • Tetracycline remains the drug of choice for aphthous stomatitis and oral ulcers of Behçet disease.
    • The patient dissolves the contents of a 250-mg tetracycline capsule in 5 mL of water or flavored syrup and holds the solution in the mouth for approximately 2 minutes before swallowing. This is repeated 4 times daily.
    • Topical corticosteroids are effective for oral or genital ulcerations if they are applied during the prodromal stage of ulceration.
    • Other useful drugs include lidocaine gel (2%), sucralfate suspension, and 5% amlexanox.
    • Twice daily usage of topical 0.2% hyaluronic acid gel improved inflammation and healing periods and reduced oral ulcers.[31]
  • Systemic therapy
    • No single drug has proven effective.
    • Corticosteroids are the mainstay of treatment for all the various clinical manifestations. Although they have a beneficial effect on acute manifestations, no definite evidence indicates they are effective for controlling progression.
    • The adverse effects of long-term steroid therapy must be considered.
    • Mucocutaneous lesions and arthritis have been treated with nonsteroidal anti-inflammatory drugs, zinc sulfate, levamisole, colchicine, dapsone, or sulfapyridine and thalidomide (use is strictly limited because of its teratogenicity). Immunosuppressive therapy with azathioprine, chlorambucil, or cyclophosphamide has been used.
    • Uveitis and central nervous system involvement is treated with systemic corticosteroids, azathioprine, or cyclosporine. However, in patients with central nervous system involvement, risks and benefits of cyclosporine need to be considered given the medication may have neurological side effects.[32, 33, 34, 35]
    • Anticoagulants are given to patients with thromboses.
    • Other therapeutic approaches have included cyclosporine, IFN alfa, IFN gamma, acyclovir, high-dose corticosteroids or cyclophosphamide pulse therapy, and FK506 (tacrolimus, an immunosuppressive agent similar to cyclosporine).
    • FK506 (tacrolimus) has been particularly noteworthy. The Japanese FK506 study group reported that FK506 was effective in treating refractory uveitis in a dosage-dependent manner. Adverse effects were renal impairment (28.3%), neurologic symptoms (20.8%), gastrointestinal symptoms (18.9%), and hyperglycemia (13.2%). The study group also noted the need for further clinical investigations on FK506 before more widespread application.
    • Subcutaneous IFN alfa-2a therapy has resulted in reduced ulcers and eye disease. Flulike symptoms were the most common adverse effect. Leukopenia, hair loss, and development of antinuclear and antithyroid antibodies were reported less commonly.[36, 37]
    • A patient with Behçet disease presenting with oral ulcers resistant to prednisone, azathioprine, colchicine, dapsone, and cyclosporin responded well to lenalidomide.[38]
    • In a single patient previously diagnosed with Behcet syndrome with recurrent oral aphthous ulcers, quadruple therapy (proton pump inhibitor, bismuth, tetracycline, and metronidazole), for histologically positive H pylori determined by upper gastrointestinal endoscopy, reduced the development of new ulcers and frequency of recurrent attacks for less than one month. Evaluation of breath test for H pylori was negative in the first month after discontinuation of therapy.[39]
    • A case of Behçet disease resistant to prednisone, cyclosporin, azathioprine, infliximab with methotrexate, and colchicine has been successfully treated with anakinra.[40]
    • With the possible role of TNF-alpha in the pathogenesis of Behçet disease, infliximab, a chimeric monoclonal immunoglobulin G antibody that inhibits TNF-alpha, and etanercept, a TNF receptor blocker, have steroid-sparing effects and have decreased the frequency of attacks in patients with Behçet disease.[41]
      • Case reports describe treatment of patients with recalcitrant disease or those in whom conventional immunosuppressive agents have failed.[42, 43, 44, 45]
      • Pediatric case responding to infliximab has been reported.[46]
      • Infliximab has resulted in responses after etanercept failed.[47]
      • Infliximab infusions of 5-10 mg/kg have been used with variable dosing schedules.
      • Tuberculosis was a reported adverse effect of infliximab infusion in one Behçet disease patient.[43]
      • Several patients not responding to infliximab have been treated with adalimumab.[48]
      • Etanercept has been used at 25 mg twice a week.[49]
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Surgical Care

Surgical therapy becomes necessary in serious conditions, including the following:

  • Gastrointestinal perforation
    • Enterocutaneous fistula formation
    • Spontaneous arterial aneurysm formation
    • Thrombotic obstruction in large-caliber vessels
    • Cardiac involvement
  • Proper timing for surgical treatment is important.
  • Delayed wound healing or inflammation at operative sites may be related to pathergy.
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Consultations

  • Dermatologist - For evaluation of mucocutaneous lesions (ie, oral ulcer, genital ulcer, skin lesions)
  • Ophthalmologist - For evaluation of eye involvement
  • Rheumatologist or orthopedic surgeon - For evaluation of joint involvement
  • Neurologist or psychiatrist - For evaluation of CNS involvement
  • Internal medicine specialist - For evaluation of gastrointestinal, pulmonary, renal, or endocrine involvement
  • General surgeon - For evaluation of gastrointestinal involvement
  • Chest surgeon or cardiologist - For evaluation of cardiovascular involvement
  • Ear, nose, and throat specialist or dentist - For evaluation of oral cavity
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Contributor Information and Disclosures
Author

Marjan Yousefi, MD  Department of Dermatology, Geisinger Medical Center

Marjan Yousefi, MD is a member of the following medical societies: American Academy of Dermatology and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Tammie Ferringer, MD  Dermatopathology Section Head, Dermatopathology Fellowship Director, Departments of Dermatology and Pathology, Geisinger Medical Center

Tammie Ferringer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, and International Society of Dermatopathology

Disclosure: Nothing to disclose.

Sungnack Lee, MD  Vice President of Medical Affairs, Professor, Department of Dermatology, Ajou University School of Medicine, Korea

Sungnack Lee, MD is a member of the following medical societies: American Dermatological Association

Disclosure: Nothing to disclose.

Dongsik Bang, MD, PhD  Vice Director, Severance Hospital; Professor, Department of Dermatology, Yonsei University College of Medicine, Korea

Disclosure: Nothing to disclose.

Specialty Editor Board

David P Fivenson, MD  Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan

David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Warren R Heymann, MD  Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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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Minor aphthous ulcer.
Major aphthous ulcer.
Herpetiform oral ulcer.
A characteristic genital ulcer on scrotum.
A single ulcer on vulva.
Erythema nodosum–like lesions on skin.
Papulopustular eruptions.
Sweet syndrome–like lesion.
Typical positive pathergy reaction at injection site.
Ocular involvement showing posterior uveitis.
 
 
 
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