...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Frontiers in Medicine >Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab
【24h】

Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab

机译：严重的耐火榫膜塑料粘膜膜分发膜用rituximab成功处理

获取原文
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Mucous membrane pemphigoid (MMP) is a rare autoimmune bullous disease of the mucous membranes, which can cause irreversible scarring and is discussed to be associated with cancer, if laminin-332-autoantibodies are present. MMP with severe ocular and laryngeal involvement is difficult to treat and can be treatment-refractory to conventional immunosuppressant therapy. A 67-year-old man with a history of prostate cancer presented to our clinic with sore throat, intraoral bullae, odynophagia, dysphonia, exertional dyspnea, and erosions of the glans penis. Clinical examination confirmed a laryngo-pharyngitis with involvement of the epiglottis and bilateral symblepharon. Diagnostics comprising multiple biopsies, direct and indirect immunofluorescence, serology analysis, and immunoblotting confirmed the diagnosis of a paraneoplastic MMP by showing a subepithelial split in histology and the presence of anti-laminin-332-antibodies. Despite combined systemic treatment with prednisolone and either dapsone or azathioprine, a progress of the disease occurred leading to severe ocular and laryngeal complications. Two month after rituximab treatment, complete disease control was achieved. This case report shows a severe ocular and life threatening laryngeal involvement of therapy-refractory paraneoplastic MMP highlighting the importance of interdisciplinary management and difficulty of diagnosing MMP despite repeated diagnostic testing.
机译：粘膜Pemphigoid（MMP）是一种罕见的自身免疫性大疱性粘膜，这可能导致不可逆的瘢痕，如果存在层粘连蛋白-332-自身抗体，则讨论与癌症相关。具有严重眼和喉部受累的MMP难以治疗，并且可以对常规免疫抑制治疗进行治疗难治。一名67岁的男子，患有前列腺癌的历史，呈现给我们的诊所，喉咙痛，口腔疱疹，繁体食管，呼吸困难，令人讨厌的呼吸困难和龟头阴茎的腐蚀。临床检查证实了喉咽炎，患有外膜淋巴和双侧搅拌的咽炎。包括多种活组织检查，直接和间接免疫荧光，血清学分析和免疫印迹的诊断证实了通过显示组织学中的亚本皮细胞分裂和抗层粘连蛋白-332-抗体的映上的诊断。尽管使用泼尼松龙和抗酮或偶氮唑来说，尽管具有泼尼松龙和双酮，但疾病的进展导致严重的眼镜和喉部并发症。利妥昔单抗治疗后两个月，实现完全疾病控制。本病例报告显示了治疗 - 难治性坐标性MMP的严重眼部和危及喉头参与，突出跨学科管理的重要性，尽管诊断检测，但仍然存在诊断MMP的难度。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号