Revision 35 for 'Behçet disease'

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Behçet disease

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

Epidemiology

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.

Clinical presentation

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.

Pathology

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

Associations
Distribution

Primarily involves four main systems:

Treatment and prognosis

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

History and etymology

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

Differential diagnosis

On imaging consider:

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