大疱性类天疱疮227例回顾性临床分析
|
摘要
目的:总结大疱性类天疱疮(BP)的临床特点及治疗经验,为本病的诊疗提供借鉴。方法:回顾227例BP住院患者临床资料,分析其一般情况、临床表现、辅助检查、治疗方法、疗效、误诊与复发情况及合并症。结果:(1)男女发病比例为1.2∶1,平均发病年龄为(72.6±11.0)岁;(2)8.4%的患者伴有口腔黏膜受累,45.9%患者发病1个月以上才出现典型的水疱或大疱;(3)直接免疫荧光检查阳性率高于间接免疫病理检查阳性率,分别为94.8%和75.9%;(4)皮损程度越重,控制症状需要的糖皮质激素量越大;(5)发病早期误诊率>50%,非大疱期最易误诊为湿疹,大疱期最易误诊为单纯疱疹或带状疱疹;(6)24.3%的BP合并神经系统疾病,与对照组比较差异有统计学意义。结论:(1)对老年初发湿疹样皮疹患者应尽早行皮损组织病理检查及免疫病理检查,排除BP;(2)糖皮质激素治疗BP时起始需足量,缓慢减量;(3)避免神经系统疾病的危险诱发因素可能会降低BP的发病率。
Objective: To summarize the clinical characteristics and treatments of bullous pemphigoid(BP) in order to provide reference for the diagnosis and treatment of this disease. Methods: The clinical data, including the general information,clinical manifestations, auxiliary examination, therapeutic methods and efficacy, misdiagnosis, recurrence and complication were analyzed in 227 cases of BP inpatients. Results:(1)The ratio of male to female was 1.2∶1; The average age of onset was72.6±11.0 years old.(2) 8.4% of patients were accompanied by oral mucosal involvement, while 45.9% of patients had typical skin blisters or bullae for over a month.(3)The positive rate of direct immunofluorescence was higher than that of indirect immunofluorescence(94.8% versus 75.9%).(4)The severer the skin lesion, the higher dose of glucocorticoids was required to control the symptoms.(5)The misdiagnosis rate was >50% in earlier stage of BP. Disease in non-bullous phase was the easiest to be misdiagnosed as eczema, while in bullous stage, was the most likely to be misdiagnosed as herpes simplex/herpes zoster.(6)BP with nervous system diseases accounted for 24.3%, which was significantly higher than controls. Conclusions:(1)Pathological examination should be given to the elderly patients with primary eczematous lesions in order to exclude BP.(2)When glucocorticoids is used, it is ideally to start with sufficient dose and to lower dose slowly.(3)Avoidance of risk factors associated with the nervous system diseases may lower the incidence of BP.
Objective: To summarize the clinical characteristics and treatments of bullous pemphigoid(BP) in order to provide reference for the diagnosis and treatment of this disease. Methods: The clinical data, including the general information,clinical manifestations, auxiliary examination, therapeutic methods and efficacy, misdiagnosis, recurrence and complication were analyzed in 227 cases of BP inpatients. Results:(1)The ratio of male to female was 1.2∶1; The average age of onset was72.6±11.0 years old.(2) 8.4% of patients were accompanied by oral mucosal involvement, while 45.9% of patients had typical skin blisters or bullae for over a month.(3)The positive rate of direct immunofluorescence was higher than that of indirect immunofluorescence(94.8% versus 75.9%).(4)The severer the skin lesion, the higher dose of glucocorticoids was required to control the symptoms.(5)The misdiagnosis rate was >50% in earlier stage of BP. Disease in non-bullous phase was the easiest to be misdiagnosed as eczema, while in bullous stage, was the most likely to be misdiagnosed as herpes simplex/herpes zoster.(6)BP with nervous system diseases accounted for 24.3%, which was significantly higher than controls. Conclusions:(1)Pathological examination should be given to the elderly patients with primary eczematous lesions in order to exclude BP.(2)When glucocorticoids is used, it is ideally to start with sufficient dose and to lower dose slowly.(3)Avoidance of risk factors associated with the nervous system diseases may lower the incidence of BP.
引文
[1]王娣,陈喜雪,朱学骏. 124例大疱性类天疱疮治疗回顾[J].中华皮肤科杂志,2007,40(1):7-9.
[2]周小勇,李慧珠.大疱性类天疱疮89例临床分析[J].中华皮肤科杂志,2011,34(6):425-427.
[3] de Graauw E, Sitaru C, Horn M, et al. Evidence for a role of eosinophils in blister formation in bullous pemphigoid[J]. Allergy, 2017, 72(7):1105-1113.
[4]刘景业,尹莉,尹志强,等. 45例大疱性类天疱疮初次住院患者临床回顾性分析[J].实用皮肤病学杂志,2015,8(4):253-256.
[5] Amber KT, Zikry J, Hertl M. A multi-hit hypothesis of bullous pemphigoid and associated neurological disease:Is HLADQB1*03:01, a potential link between immune privileged antigen exposure and epitope spreading?[J]. HLA, 2017, 89(3):127-134.
[6] Daneshpazhooh M, Khorassani J, Balighi K, et al. Neurological diseases and bullous pemphigoid:A case-control study in Iranian patients[J]. Indian J Dermatol Venereol Leprol, 2017, 83(2):195-199.
[7] Brick KE, Weaver CH, Savica R, et al. A population-based study of the association between bullous pemphigoid and neurologic disorders[J]. J Am Acad Dermatol, 2014, 71(6):1191-1197.
[2]周小勇,李慧珠.大疱性类天疱疮89例临床分析[J].中华皮肤科杂志,2011,34(6):425-427.
[3] de Graauw E, Sitaru C, Horn M, et al. Evidence for a role of eosinophils in blister formation in bullous pemphigoid[J]. Allergy, 2017, 72(7):1105-1113.
[4]刘景业,尹莉,尹志强,等. 45例大疱性类天疱疮初次住院患者临床回顾性分析[J].实用皮肤病学杂志,2015,8(4):253-256.
[5] Amber KT, Zikry J, Hertl M. A multi-hit hypothesis of bullous pemphigoid and associated neurological disease:Is HLADQB1*03:01, a potential link between immune privileged antigen exposure and epitope spreading?[J]. HLA, 2017, 89(3):127-134.
[6] Daneshpazhooh M, Khorassani J, Balighi K, et al. Neurological diseases and bullous pemphigoid:A case-control study in Iranian patients[J]. Indian J Dermatol Venereol Leprol, 2017, 83(2):195-199.
[7] Brick KE, Weaver CH, Savica R, et al. A population-based study of the association between bullous pemphigoid and neurologic disorders[J]. J Am Acad Dermatol, 2014, 71(6):1191-1197.
© 2004-2018 中国地质图书馆版权所有 京ICP备05064691号 京公网安备11010802017129号 地址：北京市海淀区学院路29号 邮编：100083 电话：办公室：(+86 10)66554848；文献借阅、咨询服务、科技查新：66554700 |