Behçet disease

Radswiki et al.

Behçet disease is a multi-systemic 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 region, Middle East and East Asia. The highest incidence has been reported in Turkey. Males are affected 2-5 times more often than females.

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), Turkish dermatologist.

On imaging consider:

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Article information

rID: 11128
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Behcet syndrome
  • Behçet's disease
  • Behçet's disease

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Cases and figures

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    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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