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For further information, see CMDT Part 20-27: Behçet Syndrome

Key Features

Causes recurrent attacks of oral aphthous ulcers, genital ulcers, uveitis, and skin lesions

Onset usually in young adults, aged 25–35 yr

Blindness, CNS abnormalities, and thrombosis or rupture of large vessels are the most serious complications

Clinical Findings

  • Recurrent oral and genital ulcers

  • Erythema nodosum–like lesions

  • Follicular rash

  • Eye abnormalities include

    • Keratitis

    • Retinal vasculitis

    • Anterior uveitis (often with hypopyon, or pus in the anterior chamber)

  • Seronegative arthritis occurs in about two-thirds of patients, most commonly affecting the knees and ankles

  • CNS abnormalities include

    • Cranial nerve palsies

    • Convulsions

    • Encephalitis

    • Mental disturbances

    • Spinal cord lesions

  • Aphthous ulcerations of the ileum and cecum and other forms of gastrointestinal involvement develop in ∼25% of patients

  • Large vessel vasculitis can lead to pulmonary artery aneurysms and life-threatening pulmonary hemorrhage

  • Clinical course may be chronic but is often characterized by remissions and exacerbations

Diagnosis

  • Clinical diagnosis

  • CNS lesions may mimic multiple sclerosis radiologically

  • Differential diagnosis

    • Inflammatory bowel disease

    • Systemic lupus erythematosus

    • Recurrent aphthous ulcers

    • Herpes simplex infection

    • Ankylosing spondylitis

    • Reactive arthritis

    • Syphilis

    • Sarcoidosis

    • HIV infection

Treatment

  • Colchicine (0.6 mg once to three times daily orally) and thalidomide (100 mg/day orally) help ameliorate the mucocutaneous findings

  • Apremilast

    • A selective phosphodiesterase-4 inhibitor

    • Effective for the treatment of oral ulcers

  • Corticosteroids (prednisone 1 mg/kg/day orally) are a mainstay of initial therapy for severe disease manifestations

  • Azathioprine (2 mg/kg/day orally) may be an effective steroid-sparing agent

  • Infliximab, cyclosporine, or cyclophosphamide is indicated for severe ocular and CNS complications of Behçet disease

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