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.

Associations
Distribution

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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