eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Behcet Disease: Treatment & Medication

Author: Marjan Yousefi, MD, Department of Dermatology, Geisinger Medical Center
Coauthor(s): Tammie Ferringer, MD, Teaching Staff, Departments of Dermatology and Pathology, Geisinger Medical Center; Sungnack Lee, MD, Vice President of Medical Affairs, Professor, Department of Dermatology, Ajou University School of Medicine, Korea; Dongsik Bang, MD, PhD, Vice Director, Severance Hospital; Professor, Department of Dermatology, Yonsei University College of Medicine, Korea
Contributor Information and Disclosures

Updated: Jun 19, 2009

Treatment

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.
    • 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.32,33
    • A patient with Beh ç et disease presenting with oral ulcers resistant to prednisone, azathioprine, colchicine, dapsone, and cyclosporin responded well to lenalidomide.34   
    • A case of Beh ç et disease resistant to prednisone, cyclosporin, azathioprine, infliximab with methotrexate, and colchicine has been successfully treated with anakinra.35
    • 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.36
      • Case reports describe treatment of patients with recalcitrant disease or those in whom conventional immunosuppressive agents have failed.37,38,39,40
      • Pediatric case responding to infliximab has been reported.41
      • Infliximab has resulted in responses after etanercept failed.42
      • 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.38
      • Several patients not responding to infliximab have been treated with adalimumab.43
      • Etanercept has been used at 25 mg twice a week.44

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.

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

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Corticosteroids have been used for all of the various clinical manifestations of Behçet disease. Anti-inflammatory and immunosuppressive agents are used to treat mucocutaneous lesions and arthritis associated with this disease. Anticoagulants are administered to patients with thromboses.

Corticosteroids

These agents modify the body's immune response to diverse stimuli and therefore have anti-inflammatory properties. In addition, they cause profound and varied metabolic effects. Corticosteroids are immunosuppressive and affect the replication, movement, and activity of virtually all cells involved with inflammation.


Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Immunosuppressant for treatment of autoimmune disorders. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

Adult

0.05-2 mg/kg/d PO divided bid/qid; not to exceed 80 mg/d; taper as condition improves; single morning dose is safer for long-term use, but divided doses have more anti-inflammatory effect

Pediatric

4-5 mg/m2/d PO; alternatively, 0.05-2 mg/kg PO divided bid/qid; if used > 3 wk, taper over 2 wk as symptoms resolve

Coadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur


Hydrocortisone (Solu-Cortef)

Decreases inflammation by suppressing migration of PMN leukocytes and reversing increased capillary permeability.

Adult

15-240 mg IV/IM q12h

Pediatric

1-5 mg/kg/d or 75-300 mg/m2/d PO divided q12-24h

Clearance may decrease with estrogens; may increase digitalis toxicity secondary to hypokalemia

Documented hypersensitivity; viral, fungal, or tubercular skin infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hyperthyroidism, osteoporosis, peptic ulcer, cirrhosis, nonspecific ulcerative colitis, diabetes, and myasthenia gravis

Nonsteroidal anti-inflammatory drugs

Although most NSAIDs are used primarily for their anti-inflammatory effects, they are effective analgesics and used to treat mild to moderate pain.


Ibuprofen (Ibuprin, Advil, Motrin)

DOC for mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.

Adult

400 mg PO q4-6h, 600 mg q6h, or 800 mg PO q8h while symptoms persist; not to exceed 3.2 g/d

Pediatric

6 months to 12 years: 4-10 mg/kg/dose PO tid/qid
>12 years: Administer as in adults

May decrease effects of loop diuretics with coadministration; coadministration with anticoagulants may increase PT (monitor and watch for signs of bleeding); may increase serum lithium levels and risk of methotrexate toxicity; probenecid may increase toxicity of NSAIDs

Documented hypersensitivity; avoid in peptic ulcer disease, recent GI bleeding or perforation, renal insufficiency, and high risk of bleeding

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Category D in third trimester of pregnancy; caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in coagulation abnormalities or during anticoagulant therapy

Antibiotics

May be immunomodulatory.


Tetracycline (Sumycin)

Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s). Has anti-inflammatory activity.

Adult

Dissolve the contents of a 250-mg cap in 5 mL water or flavored syrup and hold solution in mouth for 2 min before swallowing; repeat qid

Pediatric

<8 years: Not recommended
>8 years: 25-50 mg/kg/d (10-20 mg/lb) PO qid

Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy; can increase hypoprothrombinemic effects of anticoagulants

Documented hypersensitivity; severe hepatic dysfunction

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; use during tooth development (last half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines

Antiulcer agents

Topical treatment for aphthae.


Amlexanox (Aphthasol)

Paste at 5% concentration. Topical mucosal anti-inflammatory agent. Exact mechanism of action remains unknown. Begin treatment as soon as patient notices symptoms.

Adult

Apply topically to each mouth ulcer qid

Pediatric

Not established

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

No serious reactions have been reported; less serious adverse effects of burning, transient pain, and contact dermatitis/mucositis have been reported


Sucralfate (Carafate, Sulcrate)

Forms viscous adhesive substance that protects GI lining against pepsin, peptic acid, and bile salts. Use for short-term management of ulcers.

Adult

1 g PO qid

Pediatric

Not established; suggested dose, 40-80 mg/kg/d PO divided q6h

May decrease effects of ketoconazole, ciprofloxacin, tetracycline, phenytoin, warfarin, quinidine, theophylline, and norfloxacin

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Caution in renal failure and conditions that impair excretion of absorbed aluminum

Immunosuppressive agents

Indicated to treat autoimmune diseases.


Azathioprine (Imuran)

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

Adult

1 mg/kg/d PO for 6-8 wk; increase by 0.5 mg/kg q4wk until response or dose reaches 2.5 mg/kg/d

Pediatric

Initial dose: 2-5 mg/kg/d PO/IV
Maintenance dose: 1-2 mg/kg/d PO/IV

Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine

Documented hypersensitivity, deficiency of thiopurine methyltransferase

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Increases risk of neoplasia; caution in liver disease and renal impairment; hematologic toxicities may occur; monitor liver and hematologic laboratory values periodically; evaluate thiopurine methyltransferase levels prior to initiating therapy


Chlorambucil (Leukeran)

Alkylates and cross-links strands of DNA, inhibiting DNA replication and RNA transcription.

Adult

0.1-0.2 mg/kg/d PO or 3-6 mg/m2/d PO for 3-6 wk; adjust dose depending on blood counts

Pediatric

0.1-0.2 mg/kg/d PO for 5-15 wk

Documented hypersensitivity; previous resistance to this medication

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Caution in seizure disorders and bone marrow suppression


Tacrolimus (Prograf, Tacrine, FK506)

Suppresses humoral immunity (T lymphocyte) activity.

Adult

0.05 mg/kg/d IV or 0.15-0.3 mg/kg/d PO divided bid

Pediatric

0.1 mg/kg/d IV or 0.3 mg/kg/d PO

Levels may increase with diltiazem, nicardipine, clotrimazole, verapamil, erythromycin, ketoconazole, itraconazole, fluconazole, bromocriptine, grapefruit juice, metoclopramide, methylprednisolone, danazol, cyclosporine, cimetidine, or clarithromycin; levels may decrease with rifabutin, rifampin, phenobarbital, phenytoin, or carbamazepine

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Do not administer simultaneously with cyclosporine; tonic clonic seizures may occur


Cyclosporine (Sandimmune, Neoral)

Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs. For children and adults, base dosing on ideal body weight.

Adult

2-10 mg/kg/d IV divided q8-12h

Pediatric

Administer as in adults

Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin

Documented hypersensitivity; uncontrolled hypertension or malignancies; do not administer concomitantly with PUVA or UVB radiation in psoriasis because may increase risk of cancer

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Evaluate renal and liver function often by measuring BUN, serum creatinine, serum bilirubin, and liver enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO; monitor blood pressure

Immunomodulators

Anti-inflammatory agents that modulate the immune system through a variety of mechanisms.


Thalidomide (Thalomid)

Immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.

Adult

100-300 mg/d PO qd with water, preferably hs and at least 1 h pc
<50 kg (110 lb): Start at low end of dose regimen

Pediatric

Not established

May increase sedation of alcohol, barbiturates, chlorpromazine, and reserpine; due to teratogenic effects, women must use 2 additional methods of contraception or abstain from intercourse

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Perform pregnancy test within 24-h period prior to initiating therapy (weekly during the first month, followed by monthly tests with regular menstrual cycles or q2wk with irregular menstrual cycles); bradycardia may occur; use protective measures (eg, sunscreens, protective clothing) against exposure to sunlight or UV light (eg, tanning beds); prescribing physician must enter STEPS program established by manufacturer


Infliximab (Remicade)

Neutralizes cytokine TNF-alpha and inhibits its binding to TNF-alpha receptor. Mix in 250 mL NS for infusion over 2 h. Must use with low protein-binding filter (>1.2 µm) Has been used off label for treating BD.

Adult

5-10 mg/kg IV infusion at 0, 2, and 6 wk as induction regimen; then, 5 mg/kg q8wk for maintenance
IV infusion must be administered over at least 2 h; must use infusion set with inline, sterile, nonpyrogenic, low protein-binding filter (>1.2 µm); regimen approved for psoriatic arthritis; case reports describe slightly different intervals; one reported administration day 1, day 30, and then every 8 wk for total of 20 mo and 16 mo of continuous treatment in 2 different patients

Pediatric

Not available

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

TNF-alpha modulates cellular immune responses; anti-TNF therapies (eg, infliximab) may adversely affect normal immune responses and allow development of superinfections; more cases of lymphoma were observed in TNF alpha-blockers compared with control groups; may increase risk of reactivation of tuberculosis in patients with particular granulomatous infections; may worsen congestive heart failure; demyelination has been reported with TNF-alpha inhibitors


Etanercept (Enbrel)

Soluble p75 TNF receptor fusion protein (sTNFR-Ig). Inhibits TNF binding to cell surface receptors, which, in turn, decreases inflammatory and immune responses. Off label use for Behçet disease.

Adult

25 mg SC 2 times/wk or 50 mg SC 2 times/wk for 3 mo; then maintenance dose of 50 mg/wk (administration is for psoriatic arthritis)

Pediatric

0.8 mg/kg SC; maximum single dose 25 mg

Documented hypersensitivity, sepsis, concurrent live vaccination.

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Serious infections may develop, in which case therapy should be discontinued; possible adverse effects include injection site pain, redness, swelling, and headaches; rare cases of lupuslike symptoms and heart failure have been reported (discontinue treatment if symptoms develop); in some clinical trials, increased incidence of lymphomas, other malignancies, and demyelinating disorders has been reported

More on Behcet Disease

Overview: Behcet Disease
Differential Diagnoses & Workup: Behcet Disease
Treatment & Medication: Behcet Disease
Follow-up: Behcet Disease
Multimedia: Behcet Disease
References
Further Reading

References

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Keywords

Behcet disease, Behcet's disease, Behçet disease, Behcet's disease, Behcet syndrome, Behcet's syndrome, oral aphthous ulcer, genital ulcer, uveitis, oral aphthae, oral ulcers, canker sores

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, Teaching Staff, 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, and American 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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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.

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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