SFGH am report pearls: Behcet’s

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
Clinical manifestations
– 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%

Treatment:

– 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.

 

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