ACase of Sjögren’s Syndrome with Cryoglobulinemia and Cutaneous Vasculitis

Abstract

We report a case of Sjögrenʼs syndrome with cryoglobulinemia and cutaneous vasculitis. A 55-year-old female was diagnosed with Sjögrenʼs syndrome because she had dry eyes and mouth, positive anti-SS-A and SS-B antibodies, keratitis, and hypofunction of the salivary glands. Indurative purpuras and ulcers had appeared on her face, ears, hands, feet, and lower legs, and they worsened with cold exposure during winter for two years before the first visit. Histopathological examination of the purpura on her lower leg showed leukocyteclastic vasculitis around the small vessels in the dermis. The immunofluorescence technique showed granular deposits of IgM and C3 around the small vessels. She was diagnosed with immunocomplex vasculitis due to type II mixed cryoglobulinemia from the detection of monoclonal IgA-κ and polyclonal IgG-κ, λ cryoglobulin by immunoelectrophoresis. Treatment with 30 mg/day prednisolone was effective, resulting in disappearance of the purpuras and ulcers. She has been gradually tapered off the prednisolone by avoiding the cold exposure.