Jaccoud's arthritis and panvasculitis in the hypocomplementemic urticarial vasculitis syndrome

We describe a woman who developed hypocomplementemic urticarial vasculitis syndrome, and who demonstrated 2 rare features. As well as vasculitis of the small cutaneous vessels, vasculitis affected the femoral, cystic and renal arteries. Her arthritis led to subluxation of the metacarpophalangeal joints, without radiological evidence of rheumatoid-like erosions. This deformity could be passively corrected, and therefore conformed to the pattern described by Jaccoud.