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Updated: Apr 18 2020

Behcet Syndrome

  • Snapshot
    • A 35-year-old Middle Eastern man presents to his dermatologist for painful oral and genital ulcers. He reports that he also is fatigued, has joint pain and stiffness in the morning. He is currently sexually active with two women but reports that all parties had sexually transmitted infection testing prior to engaging in any sexual activities. Physical exam reveals multiple aphthous ulcers and genital ulcers. He also has palpable purpura on his lower extremities that is concerning for vasculitis. He is sent for additional laboratory workup and given topical corticosteroids and colchicine.
  • 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
    • 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
          • hyper-reactivity of the skin that occurs in response to minimal trauma
          • formation of skin lesions and ulcers
      • cranial nerve palsies may occur
  • 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
    • Making the diagnosis
      • most cases are clinically diagnosed
  • Differential
    • Herpes genitalis
    • Syphilis
    • Inflammatory bowel disease
  • 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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