Behçet disease

Last revised by Dr Yuranga Weerakkody on 24 Feb 2021

Behçet disease is a multisystemic and chronic inflammatory vasculitis of unknown etiology.

The mean age at which Behçet disease occurs is 20-30 years. The disease is most prevalent in the Mediterranean region, Middle East and East Asia. The highest incidence has been reported in Turkey. Males are affected 2-5 times more often than females.

The classic clinical triad of Behçet disease consists of:

  1. oral ulceration
  2. genital ulceration
  3. ocular manifestations

Additional clinical manifestations include skin, joints, gastrointestinal tract, genitourinary tract, central nervous system, cardiovascular system, and lung involvement. Vascular complications develop in ~30% (20-40%) of patients with Behçet disease.

The underlying pathologic process in Behçet disease is vasculitis, and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs.

Primarily involves four main systems:

The disease may recur, progress, or resolve (either spontaneously, within a few weeks to several months, or with corticosteroid therapy).

It is named after Hulusi Behçet (1889-1948), a Turkish dermatologist.

On imaging consider:

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Cases and figures

  • Case 1: with CNS involvement
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  • Case 2: with thoracic involvement
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  • Case 3: pulmonary artery aneurysms
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  • Case 4: with aortitis
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