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.