Behçet’s Syndrome

Suspect mainly in patients with ancestry from the Silk Road
  • esp. Turkey
  • Iraq, Iran, Arabia, China, Korea, Japan
  • Usually presents 20-40 yrs. old
Oral / Genital Ulcers are sine qua non
  • Painful
  • Outbreaks lasting 1-3 weeks, frequent recurrences
Arthritis: non-erosive
  • Knees, ankles, wrists
  • Usually during ulcer flares
Skin Lesions common
  • Acneiform pustules, erythema nodosum, others
Diagnosis:  Clinical  (findings not due to other conditions)
  • Oral ulcers ≥3 times/yr, PLUS 2 of Following:
  • Genital ulcers, idiopathic, recurrent
  • Uveitis (Dx’d by slit lamp)
  • Skin lesions
  • Pathergy: Insert 20 g needle obliquely 5 mm into forearm → >2 mm papule at 24-48 hrs.
Complications
  • Uveitis [can cause blindness]
  • CNS lesions
  • Thromboses & vasculitis

See posting Monoarticular / Oligoarticular Arthritis.