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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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| ==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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| *Classically painful ulcer with necrotic center and red rim
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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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| ==Differential Diagnosis==
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| *[[HSV]]
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| *[[lichen planus]]
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| *[[pemphigus vulgaris]]
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| *[[pemphigoid]]
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| *IVD
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| *[[SJS]]
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| *celiac disease
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| *[[SLE]]
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| *auto-inflammatory disorders
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| *[[Sjogren's]]
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| *[[MS]]
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| *[[sarcoidosis]]
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| *[[syphillis]]
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| *[[TB]]
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| *malignancy
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| *reactive arthritis
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| *[[HIV]]
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| *vasculitides
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| ==Evaluation==
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| *Clinical diagnosis
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| *ESR/CRP elevated
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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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| ==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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| ==References==
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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:Rheumatology]]
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