Published on 18/03/2015 by admin
Filed under Dermatology
Last modified 18/03/2015
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Sandra A. Kopp and Justin J. Green
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Behçet disease is a chronic inflammatory disorder leading to systemic vasculitis characterized by oral aphthae (at least three episodes in a 12-month period), plus two of the following: genital aphthae, cutaneous lesions (erythema nodosum, pustulosis, acneiform lesions, and pseudofolliculitis), uveitis or retinal vasculitis, or positive pathergy test. Arthritis, gastrointestinal, cardiac and neurologic manifestations may also occur. Many regimens effective for recurrent aphthous stomatitis are used to treat the aphthae of Behçet disease (see chapter on aphthous stomatitis).
In the absence of the multisystem disease, the severity and extent of the mucocutaneous manifestations direct the treatment strategy. First-line therapy for oral and genital aphthae is a high-potency topical corticosteroid in a gel or ointment formulation. Alternatively, intralesional corticosteroids (triamcinolone 5 mg/mL) can be used for major aphthae and severe minor aphthae. Other topical therapies accelerate healing or diminish the pain associated with oral aphthae. These include viscous lidocaine 2–5% applied directly to lesions, amlexanox 5% oral paste, sucralfate, tetracycline suspension and topical tacrolimus 0.1% ointment.
Colchicine 0.6 mg three times daily combined with topical corticosteroid therapy is efficacious in mucocutaneous disease. Dapsone 100–200 mg daily is also effective, but requires more frequent laboratory follow-up.
Those patients who fail the more conservative approaches or have severe mucocutaneous disease may require aggressive therapy. Thalidomide 100–300 mg daily (pediatric dose varies from 1 mg/kg/week to 1 mg/kg daily) is more effective than low-dose methotrexate 7.5–20 mg/week for severe disease. Prednisone taper begun at 1 mg/kg daily can be used for severe mucocutaneous flares, but rebound is a possible complication.
Systemic interferon (IFN)-α2a and anti-tumor necrosis factor (TNF)-α therapies may be best suited for those with severe mucocutaneous lesions and non-ocular systemic manifestations. Etanercept 50 mg weekly by subcutaneous injection, or infliximab 5 mg/kg in single or multiple intravenous infusions, have been shown to be effective in patients with recalcitrant disease and may be used as monotherapy or as an adjunct to conventional immunosuppressive therapy. Notably, infliximab has demonstrated rapid therapeutic effect, which may be useful in patients with vision-threatening posterior uveitis. These patients should be screened for latent tuberculosis infection prior to initiating anti-TNF therapies. Patients with certain extracutaneous signs (e.g., uveitis, aneurysms) may warrant combination therapy with prednisone and an immunosuppressive agent such as cyclosporine, azathioprine, chlorambucil or cyclophosphamide. Mucocutaneous disease alone rarely warrants this therapy; however, these agents have a beneficial effect on skin and mucous membrane lesions.
Pathergy test optional
Culture/polymerase chain reaction of aphthae to exclude herpes simplex virus
Vitamins B1, B2, B6, and B12, folate, zinc, and iron levels
Urinalysis
HLA-B27
Exclude inflammatory bowel disease
Jorizzo JL, Soloman AR, Cavallo T. Arch Pathol Lab Med 1985; 109: 747–51.
This method of pathergy testing may be more sensitive than standard techniques devoid of histologic study.
Ghate JV, Jorizzo JL. J Am Acad Dermatol 1999; 40: 1–18.
A review of investigations that should be carried out in a patient with complex aphthosis is presented.
Herpes simplex virus, nutritional deficiencies, neutropenia, lymphopenia, reactive arthritis, and inflammatory bowel disease may simulate the oral aphthae of Behçet disease.
Alexoudi I, Kapsimali V, Vaiopoulos A, Kanakis M, Vaiopoulos G. Clin Rheumatol 2011; 30: 157–63.
An excellent review and guide to treatments of all aspects of Behçet disease.
Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlian AM, Gul A, et al. Ann Rheum Dis 2009; 68: 1528–34.
In this systematic literature review from 1966 to 2006, systemic medications for Behçet disease were analyzed for treating different aspects of the disease.
Yurdakul S, Mat C, Tuzun Y, Ozyazgan Y, Hamuryudan V, Uysal O, et al. Arthritis Rheum 2001; 44: 2686–92.
A prospective, double-blind, controlled trial of 116 patients treated with colchicine vs placebo. Therapy with colchicine 1–2 mg daily was effective for arthritis and erythema nodosum. Orogenital aphthae were more responsive to treatment in females.
Fontes V, Machet L, Huttenberger B, Lorette G, Vaillant L. Ann Dermatol Venereol 2002; 129: 1365–9.
Fifty-four patients were treated with 1–1.5 mg of colchicine daily for at least 3 months. Twelve patients no longer had aphthae and were in complete remission and 22 patients were significantly improved, as the frequency and duration of the lesions had decreased by at least 50%. Treatment failed or tolerance was poor in 20 patients.
Sharquie KE, Najim RA, Al-Dori WS, Al-Hayani RK. J Dermatol 2006; 33: 541–6.
In a prospective, randomized, double-blind, controlled trial, 27 patients with mucocutaneous and/or joint disease and 30 healthy subjects matched for age and gender were treated with zinc sulfate vs placebo. Zinc sulfate 100 mg three times daily was found to be effective in reducing disease severity, and no adverse effects were reported.
Patients with ocular, neurologic, cardiac, or other systemic manifestations were excluded.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
Topical/intralesional corticosteroid
Topical tacrolimus
Pimecrolimus
Amlexanox 5% paste
Sucralfate
Tetracycline suspension
Chlorhexidine gluconate
Colchicine
Zinc sulfate
