Hypocomplementaemic urticarial vasculitis associated with Jaccoud's syndrome.

We report a 33-year-old Japanese man diagnosed as having hypocomplementaemic urticarial vasculitis at the age of 21, who subsequently developed Jaccoud's syndrome. Although Jaccoud's syndrome has been most frequently seen in patients with systemic lupus erythematosus, an association with other diseases has occasionally been described. Jaccoud's syndrome clinically shows joint deformities similar to rheumatoid arthritis, and needs to be differentiated from it. Patients with hypocomplementaemic urticarial vasculitis may develop Jaccoud's syndrome.