A case of urticarial vasculitis in a patient with Sjögren's syndrome

The patient was a 58-year-old woman who was diagnosed to have Sjögren's syndrome in 1998. During the course of observation, urticarial erythema and purpura appeared on both legs, which eventually spread to the trunk and upper limbs. The patient was hospitalized for lab tests in November 2000. The histopathological picture included findings of leukocytoclastic vasculitis, while direct immunofluorescence revealed IgG, IgM and C3 deposits on the blood vessel walls in the papillary layer. IgG was 2880mg/dl, IgA 1450mg/dl, and the patient tested positive for cryoglobulins, IgA κ-type M proteins were observed with immunoelectrophoresis, but the urine was negative for Bence Jones protein and no abnormalities were found in a marrow biopsy. From the above, the patient was diagnosed to have urticarial vasculitis secondary to Sjögren's syndrome. During treatment the patient demonstrated steroid resistance, but DDS (lectizole) had a marked effect. Regular γ-globulin tests will be needed in the future, while close attention should be paid to a possible transition toward multiple myeloma or other related diseases.