A 40-year-old man presents with recurrent oral ulcers (>3 episodes/year), genital ulcers, a hypopyon in the left eye, and erythema nodosum-like skin lesions. The pathergy test is positive. What is the most likely diagnosis?
- A Reactive arthritis (Reiter's syndrome)
- B Herpes simplex virus infection
- C Behçet's disease ✓
- D Inflammatory bowel disease-associated vasculitis
Explanation
Behçet's disease is a systemic vasculitis of unknown etiology diagnosed by the International Study Group criteria: recurrent oral ulcers (major criterion) plus at least 2 of the following: genital ulcers, ocular lesions (hypopyon uveitis, the hallmark eye finding), skin lesions (erythema nodosum, pseudofolliculitis), and a positive pathergy test. The pathergy reaction (sterile pustule at a skin prick site) is highly specific for Behçet's. It is more prevalent along the ancient Silk Road. Treatment includes colchicine for mucocutaneous disease and azathioprine or anti-TNF agents for severe ocular or vascular involvement.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.