Necrobiotic xanthogranuloma-rapid progression under treatment with melphalan.
Necrobiotic xanthogranuloma-rapid progression under treatment with melphalan.
复制标题
坏死性黄色肉芽肿 - 在马法兰治疗下快速进展。
DOI：
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发表时间：
2005
期刊：
影响因子：
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通讯作者：
P. Elsner
中科院分区：
文献类型：
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作者：
M. Ziemer;U. Wedding;C. Sander;P. Elsner
坏死性黄色肉芽肿是一种全身性疾病，在大多数情况下与单克隆副蛋白血症。虽然推测副蛋白血症的致病作用，但这种疾病的发病机制仍不清楚。我们报告一位65岁女性，有28年的病史，她的脸部、躯干和四肢都有播散性硬化斑块。自1994年以来，存在IgG κ副蛋白血症。目前的活组织检查显示典型的坏死性黄色肉芽肿的组织学特征。我们用美法仑和泼尼松龙治疗这种非常普遍的疾病。虽然IgG κ轻链和γ球蛋白的血清水平下降，皮肤斑块的大小和数量迅速扩大，这使人怀疑的致病意义的副蛋白血症的坏死性黄色肉芽肿的发病机制。坏死性黄色肉芽肿患者的副蛋白血症可能反映了继发现象而不是原发原因。到目前为止已经提出的基于此的致病和治疗概念应该被批判性地重新考虑。
Necrobiotic xanthogranuloma is a systemic disease associated in most cases with monoclonal paraproteinaemia. Although the causative role of the paraproteinaemia is supposed, the pathogenesis of this disease remains unknown. We report a 65-year-old woman with a 28-year history of disseminated indurated plaques involving her face, trunk and extremities. Since 1994 an IgG kappa paraproteinaemia was present. Current biopsies showed typical histological features of necrobiotic xanthogranuloma. We treated this extraordinary widespread condition with melphalan and prednisolone. Although the serum levels of IgG kappa light chains and gamma globulins decreased, the cutaneous plaques extended rapidly in size and number, which casts doubt on the causative significance of the paraproteinaemia in the pathogenesis of necrobiotic xanthogranuloma. The paraproteinaemia in patients with necrobiotic xanthogranuloma may rather reflect a secondary phenomenon than the originating cause. Pathogenetic and therapeutical concepts based thereon that have been proposed so far should be critically reconsidered.