Behçet disease

Last revised by Henry Knipe on 3 Nov 2024

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

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

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

The International Criteria for Behcet’s Disease assigns one or more ports to several 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 points 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 point: almost certainly not Behçet disease

  • 2 points: very unlikely

  • 3 points: possible but unlikely

  • 4 points: probable

  • 5 points: highly likely

  • ≥6 points: almost certainly Behçet disease

The classic clinical triad of Behçet disease consists of the following:

  • oral ulceration

  • genital ulceration

  • ocular manifestations

Additional clinical manifestations include the involvement of:

  • skin and joints

  • gastrointestinal and genitourinary tracts

  • central nervous system

  • cardiovascular and respiratory systems

  • 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.

Behçet disease primary involves five main systems:

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

This condition is named after Hulusi Behçet (1889-1948), a Turkish dermatologist, who first described it in 1937 ref.

On imaging consider:

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