Behçet disease

Last revised by Assoc Prof Frank Gaillard on 08 Oct 2022

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 diagnosis of Behçet disease relies on fulfilling a number of criteria as no single diagnostic test is available 10,11.

The International criteria for Behcet’s disease assigns one or more ports to a number of criteria, and the likelihood of a diagnosis of Behçet disease increases as the score increases 11.

  • ocular lesions: 2 points

  • genital aphthosis: 2 points

  • oral aphthosis: 2 points

  • skin lesions: 1 point

  • neurological manifestations: 1 point

  • vascular manifestations: 1 point

  • positive pathergy test: 1 point

A simple cut off score of 4 or more is sufficient for the diagnosis in the correct clinical setting, however, a more probablistic interpretation of the score is also available 11.

  • 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

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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