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| ==Background==
| | #REDIRECT[[Behçet's disease]] |
| * Chronic small-vessel vasculitis
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| * Mucocutaneous, ocular, CV, renal, GI, pulmonary, urologic, musculoskeletal, CNS involvement
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| * Aphthous oral ulcers + ≥ 2 of following: Genital aphthae, cutaneous lesions, and neurological, oral or rheumatologic manifestations
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| ==Diagnosis==
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| ===Clinical Features===
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| * Age 20-30s MC
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| * Turkey, Japan, Middle East
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| * Triad: Recurrent oral aphthous ulcers, genital ulcers, and uveitis
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| * Skin: Subcutaneous nodules, pyoderma gangrenosum, cutaneous thrombophlebitis, pustular acne-like folliculitis
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| * Ocular: Uveitis, iritis, optic neuritis, vision loss, hypopyon
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| * Neurologic: Brainstem and corticospinal tract syndromes, aseptic meningoencephalitis, increased ICP, cerebral sinus thrombosis, optic nerve ischemia
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| * GI: Ulcers, obstruction, ileocecal perforation
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| * Inflammatory oligoarthritis
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| * Vasculopathy: Aneurysm, vasculitis, superficial thrombophlebitis, thrombosis
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| * Cardiac: Myocarditis, endocarditis, pericarditis
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| * Renal: Glomerulonephritis, amyloidosis
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| * Pulmonary: Pleural effusions, pulmonary HTN, pulmonary aneurysm
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| ===Classification===
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| * International Study Group Criteria
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| * Recurrent oral aphthae (at least 3 times in 1 year) + 2 of the following in absence of other systemic diseases:
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| ** Recurrent genital aphthae
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| ** Eye lesions
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| ** Skin lesions
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| ** A positive pathery test
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| ==Workup==
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| * Clinical diagnosis
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| * ESR/CRP elevated
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| ==Differential Diagnosis==
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| * [[HSV]], [[lichen planus]], [[pemphigus vulgaris]], [[pemphigoid]], IVD, [[SJS]], celiac dz, [[SLE]], auto-inflammatory disorders
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| * [[Sjogren's]], MS, [[sarcoidosis]], [[syphillis]], [[TB]], malignancy, reactive arthritis, [[HIV]], vasculitides
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| ==Management==
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| * Rheumatology consult +/- multidisciplinary consults
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| * Oral and genital ulcers: Topical steroid
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| * Severe mucocutaneous disease: [[Prednisone]] 1mg/kg, low-dose thalidomide, or methotrexate
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| * Systemic disease: Steroid +/- cyclophosphamide or azathioprine
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| * Ocular: Prednisone + azathioprine and rapid ophthalmologist referral
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| * Cerebral [[venous sinus thrombosis]]- Heparinization
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| ==Sources==
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| <references/>
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| *Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1540.
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| *Smith EL, et al. Clinical manifestations and diagnosis of Behcet's disease. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014.
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| * Alnaimat FA, et al. (2014, Dec 16). Behcet Disease. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/329099-overview
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| [[Category:Rheum]]
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