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| ==Background==
| | #REDIRECT[[Behçet's disease]] |
| [[File:Behcet's.png|thumb|]] | |
| *Chronic small-vessel [[vasculitis]]
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| *Mucocutaneous, ocular, cardiovascular, 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 most commonly
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| *More common in 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 oligo[[arthritis]]
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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 hypertension]], 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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| *[[Sjogren's]]
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| *[[MS]]
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| *[[Sarcoidosis]]
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| *[[Syphilis]]
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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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| *Other [[vasculitis syndromes]]
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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 steroids]]
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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]]- [[heparin]]
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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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