中华皮肤科杂志 ›› 2009, Vol. 42 ›› Issue (6): 384-386.

• 论著 • 上一篇    下一篇

天疱疮282例和类天疱疮366例回顾性研究

朱玉洁1,骆肖群1,罗燕1,罗燕1,翁孟武2   

  1. 1. 上海复旦大学附属华山医院皮肤科
    2. 上海复旦大学医学院附属华山医院皮肤科
  • 收稿日期:2008-07-03 修回日期:2008-12-22 出版日期:2009-06-15 发布日期:2009-06-03
  • 通讯作者: 朱玉洁
  • 基金资助:

    无

A retrospective study of clinical diagnosis and therapy in 282 cases with pemphigus and 366 cases with pemphigoid

ZHU Yu-jie1,2,LUO Xiao-qun1,2,LUO Yan1,2,LUO Yan1,2,WENG Meng-wu1,2   

  1. 1. Department of Dermatology,Hua Shan Hospital,Shanghai 200040,China
    2. Department of Dermatology,Hua Shan Hospital,Shanghai 200040,China
  • Received:2008-07-03 Revised:2008-12-22 Online:2009-06-15 Published:2009-06-03
  • Contact: ZHU Yu-jie

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摘要：

目的 分析天疱疮和类天疱疮诊断与治疗特点。方法 对2003年至2007年天疱疮和类天疱疮648例患者的临床资料进行回顾性分析。结果 天疱疮平均发病年龄低于类天疱疮（p<0.001）。直接免疫荧光(DIF)确诊的175例天疱疮患者中IgG100%阳性，C392.0％阳性；类天疱疮223例患者中C3 99.1％阳性，IgG51.12%阳性。两种大疱性疾病的诊断中①天疱疮患者：临床诊断、病理检查与DIF三者一致率为68.8%。病理检查、DIF两者一致率为80.7%。②类天疱疮患者：临床诊断、病理检查与DIF三者一致率为62.8%。病理检查、DIF两者一致率为78.1%。皮质类固醇是治疗两种疾病的主要手段，泼尼松(0.5～1.5) mg?kg-1?d-1可控制大部分患者病情。结论 临床和病理无法确诊的天疱疮和类天疱疮病例,DIF可以作为诊断该病的重要方法。在基层医院，临床怀疑天疱疮，DIF可主要检查IgG、C3，怀疑类天疱疮，可主要检查C3。皮质类固醇是控制两种疾病的主要手段，必要时可加用静脉丙球(IVIG)冲击治疗。

关键词: 天疱疮;类天疱疮;DIF;皮质类固醇

Abstract:

Objective To analyze the diagnosis and therapy of pemphigus and pemphigoid.Methods Clinical data of 648 patients of pemphigus or pemphigoid were retrospectively analyzed.Results The mean first-onset-age of pemphigus was smaller than pemphigoid(p<0.001).In 175 patients of pemphigus confirmed by direct immunoflorescence(DIF), IgG deposition were 100% positive,while C3 92.0％.In 223 patients of pemphigoid,the proportion of C3 positive was 99.1％. ①To patients of pemphigus,coincidence proportion of clinical diagnosis, pathology and DIF was 68.8%,while the proportion of the later two was 80.7%. ②To patients of pemphigoid, consistent rate of clinical diagnosis, pathology and DIF was 62.8%,while the proportion of the later two was 78.1%.The patients were mainly treated with corticosteroids in a dosage of 0.5～1.5 mg?kg-1?d-1(prednisone). Conclusion For cases of pemphigus and pemphigoid which can’t be confirmed by clinical features and pathology, DIF is an important diagnostic method.Doctors in community hospital with limited funds could mainly test IgG and C3 when the diagosis of pemphigus was doubtable.Similarly,they could mainly test C3 if they consider pemphigoid as first diagnosis. Corticosteroid is the chief way for controlling the two dieases,while intra venousimmunoglobulin(IVIG) could be added when necessary.

Key words: Pemphigus;Pemphigoid;DIF;Corticosteroid