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