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Updated: Dec 28 2021

Behcet Syndrome

Images disease oral ulcer.jpg
  • Snapshot
    • A 40-year-old East Asian man presents for painful oral and genital ulcers. He reports that he has had these ulcers for about a week. About a year ago, he moved from Malaysia to the United States. He reports that he is currently not sexually active and has had a test for HIV half a year ago, which was negative. He is in a 3-year monogamous relationship. Physical exam shows 3 aphthous ulcers in his oral mucosa. Genital exam reveals multiple erythematous ulcers along his penis. Additionally, there are soft subcutaneous nodules along his bilateral shins. Labs reveal increased inflammatory markers. He is given topical corticosteroids.
  • Introduction
    • Clinical definition
      • a systemic small and large vessel vasculitis
  • Epidemiology
    • Incidence
      • most common in Asia, Mediterranean regions, and the Middle East
    • Demographics
      • male > female
      • 20-40 years of age
    • Risk factors
      • geographic area
      • family history
  • etiology
    • Pathogenesis
      • thought to be an autoimmune process that is triggered by an environmental or infectious source, especially in a genetically predisposed person
      • mechanism involves increased cytokines, proliferation of T-cells, and hyperactive neutrophils
    • Associated conditions
      • erythema nodosum
  • Presentation
    • Symptoms
      • arthritis
      • enthesitis
      • can have vascular thrombosis or aneurysms from vasculitis
    • Physical exam
      • recurrent oral aphthous ulcers
      • genital ulcers
      • bilateral ocular lesions
        • slit lamp testing
          • nongranulomatous uveitis
          • anterior uveitis
      • skin lesions
        • papules and pustules
        • acneiform nodules
        • erythema nodosum
        • cutaneous ulcers
        • pathergy is common
          • papules or pustules in areas of previous trauma
      • cranial nerve palsies may occur
  • Imaging
    • Magnetic resonance imaging (MRI) of head
      • indication
        • used if neurologic symptoms are present
    • Computed tomography (CT) of chest
      • indication
        • used if pulmonary involvement (pulmonary artery occlusion or aneurysm) is suspected
  • Studies
    • Labs
      • ↑ inflammatory markers
        • erythrocyte sedimentation rate
        • C-reactive protein
        • leukocytes
      • autoantibodies typically absent
    • Biopsy
      • indications
        • used to confirm diagnosis
        • can differentiate Behcet syndrome from Crohn disease
      • findings
        • leukocytoclastic vasculitis or lymphocytic vasculitis
        • thrombosis
  • Differential
    • Herpes genitalis
    • Syphilis
    • Inflammatory bowel disease
    • Making the diagnosis
      • most cases are clinically diagnosed
  • Treatment
    • Management approach
      • based on affected organs and discussion of side effects with patients
    • Medical
      • topical corticosteroids
        • indication
          • isolated oral or genital ulcers
      • colchicine
        • indications
          • erythema nodosum
          • arthritis
      • other systemic immunosuppression
        • indications
          • other organ involvement, including ocular, gastrointestinal, and neurologic
          • drugs
            • corticosteroids
            • cyclosporine
            • infliximab
            • cyclophosphamide
            • azathioprine
  • Complications
    • Blindness from ocular disease
    • Thrombosis
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