eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses
Behcet Disease: Treatment & Medication
Updated: Jun 19, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
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
None reported
Documented hypersensitivity
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
Documented hypersensitivity
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
None reported
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
Documented hypersensitivity
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
Documented hypersensitivity
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
None reported
Documented hypersensitivity
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
None reported
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 |
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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
