Behçet's disease
Background
- Chronic small-vessel vasculitis
- Mucocutaneous, ocular, cardiovascular, renal, GI, pulmonary, urologic, musculoskeletal, CNS involvement
- Aphthous oral ulcers + ≥ 2 of following: Genital aphthae, cutaneous lesions, and neurological, oral or rheumatologic manifestations
Clinical Features
- Age 20-30s most commonly
- More common in Turkey, Japan, Middle East
- Triad: Recurrent oral aphthous ulcers, genital ulcers, and uveitis
- Classically painful ulcer with necrotic center and red rim
- Skin: Subcutaneous nodules, pyoderma gangrenosum, cutaneous thrombophlebitis, pustular acne-like folliculitis
- Ocular: Uveitis, iritis, optic neuritis, vision loss, hypopyon
- Neurologic: Brainstem and corticospinal tract syndromes, aseptic meningoencephalitis, increased ICP, cerebral sinus thrombosis, optic nerve ischemia
- GI: Ulcers, obstruction, ileocecal perforation
- Inflammatory oligoarthritis
- Vasculopathy: Aneurysm, vasculitis, superficial thrombophlebitis, thrombosis
- Cardiac: Myocarditis, endocarditis, pericarditis
- Renal: Glomerulonephritis, amyloidosis
- Pulmonary: Pleural effusions, pulmonary hypertension, pulmonary aneurysm
Differential Diagnosis
- HSV
- Lichen planus
- Pemphigus vulgaris
- Bullous pemphigoid
- IVD
- SJS
- celiac disease
- SLE
- Sjögren Syndrome
- MS
- Sarcoidosis
- Syphilis
- TB
- Malignancy
- Reactive arthritis
- HIV
- Other vasculitis syndromes
Evaluation
- Clinical diagnosis
- ESR/CRP elevated
Classification
- International Study Group Criteria
- Recurrent oral aphthae (at least 3 times in 1 year) + 2 of the following in absence of other systemic diseases:
- Recurrent genital aphthae
- Eye lesions
- Skin lesions
- A positive pathery test
Management
- Rheumatology consult +/- multidisciplinary consults
- Oral and genital ulcers: Topical steroids
- Severe mucocutaneous disease: Prednisone 1mg/kg, low-dose thalidomide, or methotrexate
- Systemic disease: Steroid +/- cyclophosphamide or azathioprine
- Ocular: Prednisone + azathioprine and rapid ophthalmologist referral
- Cerebral venous sinus thrombosis- heparin
References
- Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1540.
- 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.
- 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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