Take away pearls:
- oral/genital ulcers + ocular disease – think Behcet’s!
- Behçet’s can involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation
- renal disease and peripheral nervous system disease is RARE in Behcet’s compared to other vasculitides!
Epi: most common age 20-40, middle eastern and Asian populations
– Mucous membranes: apthous ulcer like recurrent, painful oral and genital ulcers (r/o HSV)
– Ocular: 2/3 patients: uveitis>retinal vasculitis
– Skin: PATHERGY (pustule like lesion or papule that forms 48h after skin prick by a 20 gauge needle), many types of lesions including: acneiform lesions, papulo-vesiculo-pustular eruptions, pseudofolliculitis, erythema nodosum, palpable purpura
– GI: nausea, vomiting, often involves the ileocecal valve, ddx chrons
– Vascular (1/3 pts): can see a vascular pathergy-like response after vascular procedures, eg phlebitis or aneurysms; high mortality with pulmonary artery aneurysm
– CNS disease (10-20% pts): wide variety of presentations
– Arthritis: nonerosive, asymmetric, usually nondeforming arthritis in 50%
– For arthritis and mucocutaneous disease: can trial colchicine
– For organ involvement: steroids, and a second immunosuppressive agent: Azathioprine / TNF-alpha inhibitors, cyclosporine, or methotrexate
Barnes CG. Treatment of Behcet’s syndrome. Rheumatology (Oxford) 2006; 45:245.
Sakane T. Behcet’s Disease. NEJM 1999; 341: 1284-1291.