What is Behçet disease?

Updated: Dec 21, 2020
  • Author: Nicole Davey-Ranasinghe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Behçet disease is a rare vasculitic disorder that is characterized by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis. [1]  The systemic manifestations can be variable. Ocular disease has the greatest morbidity, followed by vascular disease generally from active vasculitis. Cutaneous manifestations can occur in up 75% of patients with Behcet disease and can range from acneiform lesions, to nodules and erythema nodosum. [2] (See Presentation.) GI manifestations can be severe; often, differentiating Behçet disease from active inflammatory bowel disease can be clinically difficult.

Behçet disease is unique among vasculitides in that it has the ability to affect small, medium, and large vessels. The disease appears to involve an autoimmune response triggered by exposure to an infectious agent, and it occurs predominantly in people with ancestors along the Silk Road, the ancient network that connected Asia with the Middle East and southern Europe. 

Diagnosis is based on clinical criteria (see Presentation), because specific diagnostic tests are lacking. The criteria most commonly used for diagnosis come from the International Study Group for Behçet's Disease. [3] The treatment approach depends on the individual patient, severity of disease, and major organ involvement (see Treatment and Medication.

For patient education information, see Behcet’s Syndrome.


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