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Disease
- 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.
Sources
- 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)