Wakamatsu Ryo, Watanabe Hitoshi, Suzuki Keisuke, Suga Norihiro, Kitagawa Wataru, Miura Naoto, Nishikawa Kazuhiro, Yokoi Toyoharu, Banno Syogo, Imai Hirokazu
Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan.
Intern Med. 2011;50(10):1109-12. doi: 10.2169/internalmedicine.50.4515. Epub 2011 May 1.
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.
一名58岁的日本女性出现反复腹痛、慢性荨麻疹、四肢瘀点以及补体低下，这些表现符合低补体血症性荨麻疹性血管炎综合征（HUVS）。实验室检查显示她的IgG水平显著升高（4448mg/dL；正常范围870 - 1700mg/dL），尤其是IgG4水平很高（1050mg/dL；正常范围48 - 105mg/dL），且IgG4/总IgG比值较高（0.22；正常范围0.02 - 0.05）。皮肤活检显示白细胞破碎性血管炎，IgG4沉积于血管腔和血管壁。淋巴结活检显示反应性淋巴样增生，滤泡周围区域有大量IgG4阳性细胞，但无硬化表现。对一个未经植物血凝素（PHA）刺激的肿大淋巴结进行染色体分析，结果显示每三个分析细胞中就有一个存在异常，如44, XX, - 13, add(15)(p11), - 17, - 17, 和mar。
