Behçet disease

David Goldemund M.D.
Updated on 22/02/2024, published on 16/02/2024

  • Behçet disease is characterized by the inflammation of blood vessels (vasculitis) of multiple organ systems
    • etiology remains unclear;  genetic predisposition (HLA-B51), environmental factors, and immune system dysregulation are thought to be involved
  • usual onset between 20-30 years of age, more common in males (2-5:1)
  • characterized by recurrent ulceration in the mouth and external genitalia, as well as involvement of the eyes (uveitis), joints, skin, large vessels, and the nervous system
  • the disease is more common in the Mediterranean, Middle East, and East Asia, with the highest incidence reported in Turkey; it is rarely reported in Europe

Clinical Presentation

General symptoms

  • recurrent uveitis (anterior and posterior) and keratitis leading to blindness
  • oral and genital ulcers (mainly affecting the scrotum)
    • initial presentation in ~60% of cases
  • skin involvement (nodular erythema)
    • pathergy reaction – pimple or blister at the site of sterile needle puncture in 24-48 hours- can be used as a diagnostic test  Positive pathergy reaction - a pimple or blister at the injection site occuring within 24-48 hours
  • joint involvement (>50%)
    • arthritis affecting ankles, knees, wrists, and elbows
    • lasts for a limited period of several weeks and rarely results in permanent joint damage
  • gastrointestinal and genitourinary tracts – mucosal ulcers
  • cardiovascular manifestations (5-30% of cases)
    • venous thrombosis (including visceral veins)  Caput medusae in a patient with visceral thrombosis
    • thickening of the aorta and superior vena cava (vasculitis)
    • pericarditis (most common cardiac manifestation)
    • pulmonary arterial aneurysms
Ulcerations and skin lesions in Behcet disease

CNS involvement (neuro-Behçet)

Parenchymal involvement

  • meningoencephalitis, typically involving the brainstem
  • occurrence up to one month after cutaneous manifestation
  • remittent course, acute or gradual onset
    • may clinically mimic MS
    • absence of OCB, MS-atypical lesions on MRI
  • lesions characteristics:
    • T1 – hypointense
    • T2 – hyperintense with vasogenic edema
    • T1C+ – patchy enhancement
    • DWI – isointense or slightly hyperintense
    • MRS – decreased NAA + elevated lipid and choline/creatine ratio
  • lesions localization:
    •  brainstem, BGG, and hypothalamus on T2 images  Behcet disease - MRI shows lesions typically localized in the brainstem and basal ganglia
      • do not correspond to vascular territories
      • subcortical white matter is less commonly affected
      • lesions often resolve after therapy
      • atrophy is common in chronic course
    • myelitis is less common (~ 10% of cases)
      • myelitis can be the first manifestation of the disease; a history of recurrent uveitis or ulcerations can help to establish the correct diagnosis
      • typical T2 hyperintense lesions extend over 2 segments on MRI
      • pleocytosis is found in the cerebrospinal fluid
      • increased erythrocyte sedimentation rate (ESR) indicates systemic inflammation

Vascular Behçet’s disease (less common)

  • arterial infarctions (1-5%)
    • Behçet’s disease is classified as a systemic vasculitis, although CNS vasculitis is rare
    • involvement of large arteries is atypical for Behçet’s disease
    • the role of microembolization due to a prothrombotic state is also considered
  • aneurysms formation
  • cerebral venous thrombosis (11-35%)
    • non-specific symptoms can lead to delayed diagnosis
    • usually involves multiple sinuses

Combination of parenchymal and vascular involvement

Diagnostic evaluation

  • it can take several years to confirm the diagnosis by meeting internationally accepted criteria
  • there are no specific laboratory findings
    • the pathergy reaction test is positive in only about 60% of patients   Patergická reakce - pupen nebo puchýř v místě vpichu sterilní jehlou za 24-48 hodin
  • CT+CTA or MR+MRA are used to assess vascular and parenchymal involvement
The International Criteria for Behçet’s Disease (ICBD)  (Davatchi, 2014)
  • ocular lesions (uveitis): 2 points
  • genital ulcers: 2 points
  • oral ulcers: 2 points
  • skin lesions: 1 point
  • neurological manifestations: 1 point
  • vascular manifestations: 1 point
  • positive pathergy test: 1 point


  • 0 or 1 = almost certainly not Behçet disease
  • 2 = very unlikely
  • 3 = possible but unlikely
  • 4 = probable
  • 5 = highly likely
  • ≥6 = almost certainly Behçet disease


  • medications to control inflammation
    • corticosteroids – very effective in controlling inflammation
      • systemic and topical (treatment of ulcers)
    • immunosuppressants (azathioprine, cyclosporine A and cyclophosphamide)
    • colchicine – treatment of joint manifestations and nodular erythema
      • for more severe cases
  • antithrombotic drugs
    • ASA 100 mg/d is sufficient for most stroke patients
    • anticoagulants are used in combination with immunosuppressants to treat venous thrombosis


  • the course of Behçet’s disease is variable, with periods of exacerbation and remission
  • while it is a lifelong condition, many patients can achieve significant improvement in their symptoms and quality of life with appropriate management
  • the overall activity often decreases over time

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Behcet disease