Behcet Disease

Critical

Painful oral aphthous ulcers (well-defined borders with a white yellow necrotic center, often with surrounding erythema, found in 98% to 100% of patients) at least three times per year and two of the following: genital ulcers, skin lesions, positive Behçetine (pathergy) test (formation of a local pustule that appears 48 hours after skin puncture with a needle), and eye lesions. May have other skin findings including erythema nodosum, pseudofolliculitis, palpable purpura, superficial thrombophlebitis, or dermographism.

Other

Other systemic manifestations include arthritis, hemoptysis from pulmonary artery involvement, renal involvement, gastrointestinal disease with bowel ulceration, epididymitis, and neuro-Behçet (e.g., vasculitis, encephalitis, cerebral venous thrombosis, neuropsychiatric symptoms).

Ocular Signs

Anterior: Bilateral hypopyon and anterior chamber reaction; scleritis occasionally reported.
Posterior: Vitritis, retinal vasculitis affecting both arteries and veins, venous obstruction, arterial attenuation, retinal neovascularization, focal necrotizing retinitis, waxy optic nerve pallor, and retinal detachment.

NOTE:

Patients with Behçet disease almost never have fibrin even if the anterior chamber reaction is severe, thus the hypopyon appears mobile (“shifting”) in contrast to HLA-B27-associated uveitis.

Epidemiology

Age 20 to 40 years; especially Japanese, Turkish, or Middle Eastern descent.

Differential Diagnosis ⬆ ⬇

Sarcoidosis: May occasionally present with oral ulcers. See 12.6, SARCOIDOSIS.
HLA-B27: Usually unilateral or bilateral alternating uveitis. Severe fibrinous uveitis. Oral ulcers are less painful and severe. See 12.4, HUMAN LEUKOCYTE ANTIGEN–B27–ASSOCIATED UVEITIS.
ARN: Confluent retinal whitening in the periphery. More pain than Behçet disease. See 12.8, ACUTE RETINAL NECROSIS.
GPA: Nephritis, orbital inflammation, sinus, and pulmonary inflammation.
Syphilis. See 12.12, SYPHILIS.
Systemic lupus erythematosus and other collagen vascular diseases.

Work Up ⬆ ⬇

Workup

Chest radiograph or CT, ACE level, PPD or IGRA, RPR or VDRL, and FTA-ABS or treponemal-specific assay.
Consider HLA-B27 in young men with a positive review of systems suggesting seronegative spondyloarthropathy.
Granular-staining cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) if GPA is suspected.
Consider HLA-B51 and HLA-DR5 testing for Behçet disease (positive and negative predictive values are uncertain).
Consider Behçetine (pathergy) test. The test is considered positive if there is development of a papule of 2 mm or more in size 48 hours after 5-mm deep skin prick with a 20-gauge needle.

Treatment ⬆ ⬇

If untreated, bilateral blindness often develops within 3 to 4 years. Death may result from CNS involvement. Proper referral for immunosuppressive therapy is critical.

Topical corticosteroids (e.g., prednisolone acetate 1% q1–6h depending on severity of inflammation) and cycloplegics (e.g., atropine 1% b.i.d.) for anterior inflammation.
Systemic corticosteroids should be started (prednisone 1 mg/kg p.o. daily or intravenous methylprednisolone sodium succinate 1 g daily for 3 days, followed by prednisone). Steroids delay the onset of blindness but do not alter the long-term outcome. Prior to systemic immunosuppressive therapy, it is important to rule out syphilis, tuberculosis, and hepatitis.
All patients with Behçet disease and posterior uveitis should be referred to a specialist for initiation of immunosuppressive therapy. TNF-antagonists such as infliximab or adalimumab are now considered first-line therapy for Behçet disease. Calcineurin inhibitors (tacrolimus and cyclosporine) and antimetabolites (e.g., mycophenolate mofetil, methotrexate, and azathioprine) can be used, but take 1 to 2 months to achieve the full effect.

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Symptoms ⬇

Signs ⬆ ⬇

Differential Diagnosis ⬆ ⬇

Work Up ⬆ ⬇

Treatment ⬆ ⬇

Follow Up ⬆