大疱性类天疱疮122例临床分析
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摘要
目的总结122例住院的大疱性类天疱疮(bullous pemphigoid,BP)患者的临床特点及治疗经验。方法选择并分析2009年1月-2016年12月住院的122例大疱性类天疱疮患者,并根据年龄、黏膜累及情况、治疗方法进行分组比较。结果122例住院患者年龄在60岁以上占79. 51%,首发皮损表现为:水疱和/或/大疱者占57. 38%;红斑和/或/丘疹者占42. 62%;黏膜受累占26. 23%,合并一种或多种系统疾病占80. 33%,抗BP180抗体阳性率为84. 62%。治疗:122例患者中46例为单纯糖皮质激素治疗组,66例为联合治疗组,联合治疗组的住院天数、激素初始剂量和最大控制量均分别高于激素治疗组(P均<0. 05)。黏膜累及组的住院天数、激素初始剂量均高于无黏膜累及组(P均<0. 05),中青年组的激素初始剂量高于老年组(P <0. 05)。转归:临床治愈出院59例,好转51例,总有效率为90. 16%。结论 BP集中发生于老年人,以心血管系统及神经系统疾病居多。抗BP180抗体检测在BP的诊断上有很高的敏感性,并且在一定程度上与病情变化相关。系统使用糖皮质激素以及糖皮质激素联合免疫抑制剂是治疗大疱性类天疱疮的主要手段,血浆置换对于重症的患者具有可行性。
Objective To analysis the clinical features and therapeutic experience of bullous pemphigoid. Methods A total 122 hospitalized patients with bullous pemphigoid(BP) from January 2009 to December 2016 were collected and analyzed. Results The patients(79. 51%) was mainly elderly whose age were above 60 years old. The initial lesions were blisters or bullae(57. 38%) and erythema or papule(42. 62%),meanwhile mucosal lesions occurred in 32 patients(26. 23%) and80. 33% BP patients were complicated by one or more system diseases. The positivity rate of anti-BP180 was 84. 62%. A total 46 patients were treated with systemic glucocorticoids only,66 patients were treated with glucocorticoids combined with immunosuppressant. The hospital stays,initial steroid dose and the disease-controlled doseof glucocorticoids-only group were less than combined therapy group(P < 0. 05).The hospital stays and initial steroid dose inmucosal involvement group were higher than non-inovlement group(P < 0. 05),and the initial steroid dose in the young and middle-aged group was higher than the elderly group(P < 0. 05). A total of 59 patients were discharged from hospilal after recovery,51 cases were improved,and the total effective rate was 90. 16%. Conclusion BP primarily affects elder people,which often accompanies with cardiovascular disease and nervous system disorder. Serum titer of anti-BP180 autoantibody is correlated with disease severity in BP patients. Systemic glucocorticoids therapy and combination therapy of glucocorticoids with immunosuppressant are the main treatment for BP. Plasmapheresis is feasible for severe patients.
Objective To analysis the clinical features and therapeutic experience of bullous pemphigoid. Methods A total 122 hospitalized patients with bullous pemphigoid(BP) from January 2009 to December 2016 were collected and analyzed. Results The patients(79. 51%) was mainly elderly whose age were above 60 years old. The initial lesions were blisters or bullae(57. 38%) and erythema or papule(42. 62%),meanwhile mucosal lesions occurred in 32 patients(26. 23%) and80. 33% BP patients were complicated by one or more system diseases. The positivity rate of anti-BP180 was 84. 62%. A total 46 patients were treated with systemic glucocorticoids only,66 patients were treated with glucocorticoids combined with immunosuppressant. The hospital stays,initial steroid dose and the disease-controlled doseof glucocorticoids-only group were less than combined therapy group(P < 0. 05).The hospital stays and initial steroid dose inmucosal involvement group were higher than non-inovlement group(P < 0. 05),and the initial steroid dose in the young and middle-aged group was higher than the elderly group(P < 0. 05). A total of 59 patients were discharged from hospilal after recovery,51 cases were improved,and the total effective rate was 90. 16%. Conclusion BP primarily affects elder people,which often accompanies with cardiovascular disease and nervous system disorder. Serum titer of anti-BP180 autoantibody is correlated with disease severity in BP patients. Systemic glucocorticoids therapy and combination therapy of glucocorticoids with immunosuppressant are the main treatment for BP. Plasmapheresis is feasible for severe patients.
引文
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[2]Jeon HW,Yun SJ,Lee SC,et al.Mortality and comorbidity profiles of patients with bullous pemphigoid in Korea[J].Ann Dermatol,2018,30(1):13-19.
[3]Bastuji-Garin S,Joly P,Lemordant P,et al.Risk factors for bullous pemphigoid in the elderly:a prospective case-control study[J].J Invest Dermatol,2011,131(3):637-643.
[4]Feliciani C,Joly P,Jonkman MF,et al.Management of bullous pemphigoid:the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology[J].Br J Dermatol,2015,172(4):867-877.
[5]Kalabalikis D,Patsatsi A,Trakatelli MG,et al.Hemorrhagic bullae and skin fragility-a clinicopathologicchallenge[J].Int J Dermatol,2010,49(1):21-23.
[6]林景荣,韩世新,刘晓明,等.初诊为泛发性湿疹的大疱性类天疱疮2例[J].中国皮肤性病学杂志,2013,27(7):704-705,710.
[7]Jedlickova H,Hlubinka M,Pavlik T,et al.Bullous pemphigoid and internal diseases-A case-control study[J].Eur J Dermatol,2010,20(1):96-101.
[8]Lai YC,Yew YW,Lambert WC.Bullous pemphigoid and its association with neurological diseases:a systematic review and meta-analysis[J].J Eur Acad Dermatol Venereol,2016,30(12):2007-2015.
[9]Saniklidou AH,Tighe PJ,Fairclough LC.Ig E autoantibodies and their association with the disease activity and phenotype in bullous pemphigoid:a systematic review[J].Arch Dermatol Res,2018,310(1):11-28.
[10]Feng S,Wu Q,Jin P,et al.Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid[J].Int J Dermatol,2000,47(3):225-228.
[11]Tsuji AY,Akiyama MY,Kikuchi T,et al.Correlation of clinical severity and ELISA indices for the NC16A domain of BP180 measured using BP180 ELISA kit in bullous pemphigoid[J].J Dermatol Sci,2005,37(3):145-149.
[12]中国医师协会皮肤科医师分会自身免疫性疾病亚专业委员会.大疱性类天疱疮诊断和治疗的专家建议[J].中华皮肤科杂志,2016,49(6):384-387.
[13]Venning VA,Taghipour K,MohdMustapa MF,et al.British Association of Dermatologists'guidelines for the management of bullouspemphigoid[J].Br J Dermatol,2012,167(6):1200-1214.
[2]Jeon HW,Yun SJ,Lee SC,et al.Mortality and comorbidity profiles of patients with bullous pemphigoid in Korea[J].Ann Dermatol,2018,30(1):13-19.
[3]Bastuji-Garin S,Joly P,Lemordant P,et al.Risk factors for bullous pemphigoid in the elderly:a prospective case-control study[J].J Invest Dermatol,2011,131(3):637-643.
[4]Feliciani C,Joly P,Jonkman MF,et al.Management of bullous pemphigoid:the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology[J].Br J Dermatol,2015,172(4):867-877.
[5]Kalabalikis D,Patsatsi A,Trakatelli MG,et al.Hemorrhagic bullae and skin fragility-a clinicopathologicchallenge[J].Int J Dermatol,2010,49(1):21-23.
[6]林景荣,韩世新,刘晓明,等.初诊为泛发性湿疹的大疱性类天疱疮2例[J].中国皮肤性病学杂志,2013,27(7):704-705,710.
[7]Jedlickova H,Hlubinka M,Pavlik T,et al.Bullous pemphigoid and internal diseases-A case-control study[J].Eur J Dermatol,2010,20(1):96-101.
[8]Lai YC,Yew YW,Lambert WC.Bullous pemphigoid and its association with neurological diseases:a systematic review and meta-analysis[J].J Eur Acad Dermatol Venereol,2016,30(12):2007-2015.
[9]Saniklidou AH,Tighe PJ,Fairclough LC.Ig E autoantibodies and their association with the disease activity and phenotype in bullous pemphigoid:a systematic review[J].Arch Dermatol Res,2018,310(1):11-28.
[10]Feng S,Wu Q,Jin P,et al.Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid[J].Int J Dermatol,2000,47(3):225-228.
[11]Tsuji AY,Akiyama MY,Kikuchi T,et al.Correlation of clinical severity and ELISA indices for the NC16A domain of BP180 measured using BP180 ELISA kit in bullous pemphigoid[J].J Dermatol Sci,2005,37(3):145-149.
[12]中国医师协会皮肤科医师分会自身免疫性疾病亚专业委员会.大疱性类天疱疮诊断和治疗的专家建议[J].中华皮肤科杂志,2016,49(6):384-387.
[13]Venning VA,Taghipour K,MohdMustapa MF,et al.British Association of Dermatologists'guidelines for the management of bullouspemphigoid[J].Br J Dermatol,2012,167(6):1200-1214.