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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 (formerly Reiter syndrome)

    • 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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