Behçet’s Disease

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  • Idiopathic multisystem disease, recurrent episodes with urogenital ulcers and vasculitis (small, medium, and large arteries and veins)
  • Association with HLA-B51
  • Frequently affects patients from the eastern Mediterranean regions and Japan
  • Peak incidence in the 3rd decade of life
  • More common in men than women

Clinical Presentation

  • Recurrent oral ulcers.
  • Gastrointestinal ulcers.
  • Recurrent genital ulcers.
  • Ocular inflammations:
    • Simultaneous systemic and ocular manifestations are unusual
    • Typically bilateral eye involvement
    • Acute anterior uveitis (usually a white eye with a mobile hypopyon, no fibrin!), retinitis, retinal vasculitis (of veins and arteries), vitritis, optic atrophy in the end stage; also possible manifestations include conjunctivitis, episcleritis, scleritis, and ophthalmoplegia
  • Skin lesions: Erythema nodosum, folliculitis, papulopustular lesions, positive pathergy test (= pustule formation 24-48 hours after skin puncture)
  • Vasculitis with secondary aneurysm formation, venous thrombosis, cardiomyopathy, etc.
  • Arthritis

Treatment of Posterior Uveitis:

  • Systemic steroids; usually not sufficient as a sole therapy.
  • In addition: Azathioprine/Ciclosporin/subcutaneous interferon-alpha / biologics.


  • EyeWiki Behcet Disease
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)