ACase of Sjögren’s Syndrome with Cryoglobulinemia and Cutaneous Vasculitis

DOI Web Site 6 References
  • Nakano Yumiko
    Department of Dermatology, Kanazawa University Graduate School of Medical Science
  • Echigo Takeshi
    Department of Dermatology, Kanazawa University Graduate School of Medical Science
  • Tomita Ikuyo
    Department of Dermatology, Kanazawa University Graduate School of Medical Science
  • Oishi Naoto
    Department of Dermatology, Kanazawa University Graduate School of Medical Science
  • Morita Reiji
    Department of Dermatology, Kanazawa University Graduate School of Medical Science
  • Hasegawa Minoru
    Department of Dermatology, Kanazawa University Graduate School of Medical Science
  • Kawano Mitsuhiro
    Second Department of Internal Medicine, Kanazawa University Graduate School of Medical Science
  • Yamazaki Masahide
    Third Department of Internal Medicine, Kanazawa University Graduate School of Medical Science
  • Sato Shinichi
    Department of Dermatology, Kanazawa University Graduate School of Medical Science
  • Takehara Kazuhiko
    Department of Dermatology, Kanazawa University Graduate School of Medical Science

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Other Title
  • クリオグロブリン血症と皮膚に血管炎がみられたシェーグレン症候群の1例
  • クリオグロブリン ケツショウ ト ヒフ ニ ケッカンエン ガ ミラレタ シェーグレン ショウコウグン ノ 1レイ

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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.

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