Hypocomplementemic Urticarial Vasculitis Syndrome is Associated with High Levels of Serum IgG4: A Clinical Manifestation that Mimics IgG4-related Disease

DOI Web Site 被引用文献4件 参考文献16件
  • Wakamatsu Ryo
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Watanabe Hitoshi
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Suzuki Keisuke
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Suga Norihiro
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Kitagawa Wataru
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Miura Naoto
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Nishikawa Kazuhiro
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Yokoi Toyoharu
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Banno Syogo
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan
  • Imai Hirokazu
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan

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A 58-year-old Japanese woman presented with recurrent abdominal pain, chronic urticaria, and petechiae on her extremities, and hypocomplementemia, findings that were consistent with hypocomplementemic urticarial vasculitis syndrome (HUVS). A laboratory examination revealed that she had markedly elevated IgG levels (4,448 mg/dL; normal range, 870-1,700 mg/dL) with particularly high IgG4 levels (1,050 mg/dL; normal range, 48-105 mg/dL) and a high IgG4/total IgG ratio (0.22; normal range, 0.02-0.05). A skin biopsy demonstrated leukocytoclastic vasculitis with IgG4 deposition in the vascular lumen and vascular walls. A lymph node biopsy revealed reactive lymphoid hyperplasia with numerous IgG4-positive cells in the perifollicular area, but no sclerotic findings. A chromosomal analysis of an enlarged lymph node, without phytohemagglutinin (PHA) stimulation, demonstrated that one in every three analyzed cells had abnormalities, such as 44, XX, -13, add(15)(p11), -17, -17, and mar.<br>

収録刊行物

  • Internal Medicine

    Internal Medicine 50 (10), 1109-1112, 2011

    一般社団法人 日本内科学会

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