天疱疮282例和类天疱疮366例回顾性研究  

A retrospective study of 282 cases of pemphigus and 366 cases of pemphigoid

作　　者：朱玉洁[1] 骆肖群[1] 苏媁[1] 傅海军 罗燕[1] 翁孟武[1] 

机构地区：[1]复旦大学附属华山医院皮肤科,上海200040 [2]上海凯锐斯生物科技有限公司

出　　处：《中华皮肤科杂志》2009年第6期384-386,共3页Chinese Journal of Dermatology

摘　　要：目的探讨天疱疮和类天疱疮诊断与治疗特点。方法对2003--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，C3 92．0％阳性；类天疱疮223例患者中C3 99．1％gH性，IgG 51．12％阳性。两种大疱性疾病的诊断中，①天疱疮患者的临床诊断、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类天疱疮患者的临床诊断、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％。糖皮质激素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，司主要检查C3。Objective To analyze the diagnosis and therapy ofpemphigus and pemphigoid. Methods Clinical data were retrospectively analysed for 648 patients with pemphigus or pemphigoid admitted in Huashan Hospital, Fudan University from 2003 to 2007. Results The mean age of onset of pemphigus was younger than that of pemphigoid （47.0 ± 16.9 years vs 65.1 ± 13.9 years, P 〈 0.001）. Of 175 patients with pemphigus confirmed by direct immunoflorescence （DIF）, 100% were positive for IgG, and 92.0% for C3, while out of 223 patients with pemphigoid, 51.12% were positive for IgG, 99.1% for C3. For pemphigus, the consistency reached 68.8% among clinical diagnosis, pathological diagnosis and immunological （DIF） diagnosis, 80.7% between pathological and immunological diagnosis. In the case of pemphigoid, the consistency was 62.8% among clinical, pathological and immunological diagnosis, and 78.1% between pathological and immunological diagnosis. Corticosteroids were primary treatment strategy for both pemphigns and pemphigoid, and prednisone of 0.5 - 1.5 mg per kilogram bodyweight per day could control the condition of most patients. Conclusions DIF could be used as an important diagnostic means for patients with pemphigns or pemphigoid which can not be confirmed by clinical manifestation or pathology. In primary hospitals, IgG and C3 are recommended for DIF testing when pemphigus is suspected, and C3 is recommended for suspected pemphigoid.

关 键 词：天疱疮 类天疱疮 大疱性 回顾性研究 

分 类 号：R758.66[医药卫生—皮肤病学与性病学] R473.75[医药卫生—临床医学]