大疱性类天疱疮227例回顾性临床分析

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。     

大疱性类天疱疮122例临床分析

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。     

大疱性系统性红斑狼疮文献回顾性分析

目的探讨大疱性系统性红斑狼疮临床、诊断及治疗特征,提高临床诊治水平。方法采用回顾性研究方法,对2001年至2019年中国期刊全文数据库报道的44例大疱性系统性红斑狼疮的临床特点、实验室检查、皮肤病理特征、治疗及预后进行分析。结果 44例大疱性系统性红斑狼疮患者平均年龄(27±12)岁,男女比例为1∶5.2。其中27.3%患者既往有SLE病史,15.9%被误诊为其他大疱性疾病,43.2%全身可见红斑、水疱,20.5%累及口腔黏膜。44例患者ANA均为阳性,病检均表现为表皮下水疱,72.7%出现真皮中性粒细胞浸润;75%患者肾脏受累,79.5%血液系统受累,95.5%使用糖皮质激素治疗。结论大疱性系统性红斑狼疮病情重、误诊率高。临床医生应加强与其他大疱性疾病的鉴别,积极评估患者系统受累情况,及早选择适宜的治疗方案以控制病情进展。     

45例大疱性类天疱疮初次住院患者临床回顾性分析

目的总结45例初次住院的大疱性类天疱疮患者的临床特点及治疗经验。方法分析2009年8月—2014年12月45例住院治疗的大疱性类天疱疮患者临床表现、组织病理、免疫病理、治疗方案及并发症等方面的情况。结果 45例住院患者86.67%为老年人,均表现为瘙痒性紧张性大疱,普通组织病理表现为表皮下水疱,直接免疫荧光检查见IgG和(或)C3在基膜带呈线状沉积。6例患者转科或自动出院,入院时均存在低白蛋白血症。余39例患者好转或临床治愈出院,总有效率为86.67%。39例患者中9例单纯使用糖皮质激素治疗,30例糖皮质激素联合免疫抑制剂治疗(主要为甲氨蝶呤)。单纯糖皮质激素治疗组的住院天数显著少于糖皮质激素联合免疫抑制剂治疗组(P0.05)。糖皮质激素联合使用免疫抑制剂治疗感染发生率更高(P0.05)。结论系统使用糖皮质激素是大疱性类天疱疮患者住院治疗的首选,视病情联合免疫抑制剂治疗可以增加疗效,但感染发生率会增加,要警惕低白蛋白血症的危险性。     

大疱性皮肤病53例免疫荧光和组织病理检查结果分析

目的探讨直接免疫荧光(DIF)和盐裂皮肤DIF技术在诊断大疱性皮肤病中的价值。方法采用DIF和盐裂皮肤DIF技术检测53例大疱性皮肤病标本,并与其组织病理检查结果进行比较。结果 DIF和盐裂皮肤DIF技术诊断该类疾病的阳性率(90.57%)显著高于对其进行组织病理检查的阳性率(73.58%),差异有统计学意义(P<0.05)。结论 DIF和盐裂皮肤DIF技术仍是目前诊断和鉴别诊断大疱性皮肤病的可靠方法。     

大剂量糖皮质激素联合静脉注射用人免疫球蛋白治愈中毒性表皮坏死松解症2例

<正>中毒性表皮坏死松解症(toxic epidermal necrolysis, TEN)又称大疱性表皮坏死松解型药疹、大疱性表皮松解萎缩型药疹,是一种爆发性表皮及黏膜剥脱、死亡率高达30%～35%的疾病。其中的74%～94%患者多由药物或上呼吸道感染诱发。本病常伴有系统和内脏损害,若抢救不及时,患者可能死于感染、败血症、肾衰竭、肺炎或出血,甚至危及患者生命。我科于2016年11月—2017年2月收治2例肌内注射复方氨林巴比     

扁平苔藓178例临床分析

目的 :分析总结扁平苔藓(LP)的发病年龄、临床表现、分型及实验室检查结果。方法 :回顾性分析该院178例LP患者的临床资料。结果:178例LP患者平均发病年龄(46.7±17.1)岁,男女比例1.0∶1.3,50~59岁发病率最高(37.1%)。其中慢性局限性LP(48.3%)最常见,其次为线状LP(16.3%)、急性泛发性LP(5.6%)、光线性LP(5.1%)、大疱性LP(5.1%)、环状LP(4.5%)、色素性LP(3.4%)、口腔LP(2.8%)、毛囊性LP(2.8%)、肥厚性LP(2.2%)、LP-红斑狼疮重叠综合征(2.2%)、类天疱疮样LP(1.1%)及掌跖LP(0.6%)。38例(21.3%)伴口腔受累。所有患者抗核抗体(ANA)阳性率为32.6%,其中LP-红斑狼疮重叠综合征阳性率最高。雷公藤多苷治疗对皮肤型LP有效。结论:LP临床表现差异较大,慢性局限性LP是最常见亚型。LP-红斑狼疮重叠综合征患者ANA阳性率最高,其他亚型与ANA关系有待进一步探讨。     

C3d在大疱性类天疱疮患者皮损组织中的表达

目的 探讨C3d在石蜡包埋的大疱性类天疱疮患者皮损组织中的表达及临床意义。方法 免疫组化SP法在25例大疱性类天疱疮、10例大疱性表皮松解症及10例正常成人皮肤组织标本中进行C3d、IgG、IgA进行检测,并对其在大疱性类天疱疮皮损中阳性率进行比较。结果 大疱性类天疱疮皮损中C3d、IgG、IgA的阳性率分别为96%、72%、0%。C3d、IgG在BP组织中表达阳性率的差异有统计学意义（χ2 = 4.17,P < 0.05）,C3d、IgA在BP组织中表达阳性率的差异有统计学意义（χ2 = 22.04,P < 0.01）。C3d、IgG、IgA在10例EB及正常成人皮肤组织标本中表达均为阴性。结论 免疫组化方法检测C3d的表达可以协助在石蜡组织中进行大疱性类天疱疮的诊断。     

自身免疫性大疱病合并银屑病20例临床分析

收集2011年1月1日至2021年9月30日就诊于四川大学华西医院的自身免疫性大疱病合并银屑病患者的资料,对其临床表现、组织病理、免疫病理、血清学特异性抗体、治疗和转归等进行回顾性分析。共分析20例自身免疫性大疱病合并银屑病患者,男14例,女6例,平均年龄(64.75±13.13)岁,其中17例患者表现为银屑病先于自身免疫性大疱性疾病发生,平均时间(18.03±14.69)年。治疗上采用糖皮质激素和(或)免疫抑制剂治疗,15例患者皮损控制良好,病情较稳定,4例患者失访,1例死亡。自身免疫性大疱病合并银屑病临床少见,临床工作中应警惕两种疾病并发的情况。     

表现为环状红斑水疱的自身免疫性表皮下水疱病25例回顾性分析

目的总结表现为环状红斑水疱的自身免疫性表皮下水疱病的临床、组织病理、免疫血清学及治疗特点。方法回顾性分析2015—2022年就诊于中国医学科学院皮肤病医院表现为环状红斑水疱的自身免疫性表皮下水疱病患者的资料。结果共纳入患者25例, 男10例、女15例, 年龄(39.21 ± 24.65)岁, 包括线状IgA大疱性皮病9例, 大疱性类天疱疮7例, 抗P200类天疱疮5例, 获得性大疱性表皮松解症4例, 20例(80%)有不同程度瘙痒。15例(60%)出现真皮组织嗜酸性粒细胞浸润, 11例(44%)外周血嗜酸性粒细胞计数增加, 7例(28%)同时有嗜酸性粒细胞组织浸润和外周血嗜酸性粒细胞升高。盐裂皮肤-间接免疫荧光及免疫印迹实验显示, 9例同时存在抗基底膜带IgG及IgA抗体, 包括4例大疱性类天疱疮、1例线状IgA大疱性皮病、2例抗P200类天疱疮、2例获得性大疱性表皮松解症;5例同时存在多种抗基底膜带靶抗原的抗体。7例大疱性类天疱疮均予系统糖皮质激素治疗, 其中5例联合免疫抑制剂, 2例联合米诺环素;线状IgA大疱性皮病、抗P200类天疱疮、获得性大疱性表皮松解症患者对抗炎药物及氨苯砜...     

95例住院天疱疮患者回顾性治疗分析

目的:对95例住院天疱疮患者的治疗情况进行回顾性分析。方法:95例患者分为3组,第1组采用糖皮质激素联合免疫抑制剂治疗;第2组在第1组基础上加用糖皮质激素冲击疗法;第3组在第1组基础上加用静脉滴注大剂量免疫球蛋白,分别评价近期疗效。结果:出院时第1组77例患者中68例(88.31%)皮损完全消退;第2组13例患者中10例(76.92%)皮损完全消退;第3组5例患者中4例(80.00%)皮损完全消退。结论:糖皮质激素联合免疫抑制剂是目前治疗天疱疮的主要方法,对部分常规剂量不能控制病情者加用糖皮质激素冲击疗法或静脉滴注大剂量免疫球蛋白可取得较好疗效。     

Narrowband ultraviolet B phototherapy for alopecia areata

Although narrowband ultraviolet B (NB UVB) phototherapy is a well-established treatment in many dermatosis, there is little evidence of efficacy of this method for alopecia areata (AA) treatment in the literature. We undertook a retrospective review of the 25 AA patients treated with NB UVB. Intramuscular triamcinolone acetonide injections per month were used as concomitant treatment in some patients who did not have any contraindication. Eight patients (32%) received monthly intramuscular corticosteroid injections. Four (22.2%) and two (20%) patients achieved excellent response in extensive patchy hair loss patients and entire scalp hair loss patients, respectively. Four of six patients who achieved excellent response also received monthly intramuscular corticosteroid injections. When patients receiving systemic corticosteroid injections were compared with patients given only NB UVB with respect to the treatment responses, a statistically significant difference was seen in patients who achieved excellent response. NB UVB is not an effective treatment with only 20% excellent treatment responses in patients with severe AA, most of whom were also treated with systemic corticosteroids.     

Comparison of High-Dose Corticosteroid Pulse Therapy and Combination Therapy Using Oral Cyclosporine with Low-Dose Corticosteroid in Severe Alopecia Areata

BackgroundSevere alopecia areata (AA) is resistant to conventional treatment. Although systemic oral corticosteroids are an effective treatment for patients with severe AA, those drugs have many adverse effects. Corticosteroid pulse therapy has been introduced to increase therapeutic effects and reduce adverse effects. However, the treatment modality in severe AA is still controversial.ObjectiveTo evaluate the effectiveness of corticosteroid pulse therapy in patients with severe AA compared with treatment with oral cyclosporine with corticosteroid.MethodsA total of 82 patients with severe AA were treated with corticosteroid pulse therapy, and 60 patients were treated with oral cyclosporine with corticosteroid. Both groups were retrospectively evaluated for therapeutic efficacy according to AA type and disease duration.ResultsIn 82 patients treated with corticosteroid pulse therapy, 53 (64.6%) were good responders (>50% hair regrowth). Patients with the plurifocal (PF) type of AA and those with a short disease duration (≤3 months) showed better responses. In 60 patients treated with oral cyclosporine with corticosteroid, 30 (50.0%) patients showed a good response. The AA type or disease duration, however, did not significantly affect the response to treatment.ConclusionCorticosteroid pulse therapy may be a better treatment option than combination therapy in severe AA patients with the PF type.     

Stevens-Johnson综合征及中毒性表皮坏死松解症61例回顾性分析

目的 探讨Stevens-Johnson综合征（SJS） 及中毒性表皮坏死松解症（TEN）患者的病因及治疗经验。方法 回顾性分析1994年7月至2007年5月确诊为SJS和TEN的61例患者的临床资料,根据治疗分为静脉滴注免疫球蛋白联合糖皮质激素组（简称IVIG组）16例及糖皮质激素组（简称激素组）45例,采用SCORTEN评分评价病情严重程度及预后。比较激素初始量、最大控制量、减量前激素总量、IVIG总量、激素减量前时间、住院时间等指标。结果 引起SJS和TEN常见致敏药物依次为非甾体抗炎药类（26例）、抗癫痫药（15例）、抗生素类（10例）,其中最多的为卡马西平（13例）。IVIG组SCORTEN评分（1.44 ± 1.21）显著高于激素组（0.80 ± 1.10）,两组差异有统计学意义（P < 0.05）。IVIG组减量前激素总量、减量前时间及住院治疗时间（分别为12.06 ± 4.32 mg/kg,7.81 ± 2.29 d,18.00 ± 5.92 d）与激素组（分别为12.52 ± 8.29 mg/kg,8.29 ± 4.18 d,21.07 ± 13.36 d）比较,差异均无统计学意义。11例SCORTEN评分等于2的患者联用IVIG及激素能使住院治疗时间从（27.57 ± 9.90） d缩短至（14.50 ± 2.38） d（P < 0.05）。IVIG组并发症发生率（43.8％）高于激素组（24.4％）（P < 0.05）。IVIG组与激素组实际死亡率分别为12.5％及4.4％,均低于预期死亡率（分别为12.9％及7.9％）。结论 激素及IVIG治疗SJS和TEN有效。     

Combination therapy of intravenous immunoglobulin and corticosteroid in the treatment of toxic epidermal necrolysis and Stevens–Johnson syndrome: a retrospective comparative study in China

Background Toxic epidermal necrolysis (TEN) and Stevens–Johnson Syndrome (SJS) are drug‐induced diseases with no well‐established treatments. The application of corticosteroid therapy is controversial. Intravenous immunoglobulin (IVIG) therapy is emerging as a promising new method for the treatment of these two diseases. The efficacy of combination therapy of IVIG and corticosteroid in the treatment of TEN/SJS has seldom been reported. Methods Sixty‐five consecutive patients with either TEN or SJS, admitted over a 14‐year period from January 1993 to October 2007, were treated with corticosteroid and analyzed retrospectively using SCORTEN, a severity‐of‐illness scoring system for TEN/SJS prognosis, to evaluate efficacy. For patients admitted after January 2001, additional therapy with a dose of 0.4 g/kg/day of IVIG for 5 days was applied. Results In the 45 patients with TEN treated without IVIG, 8.63 patients were expected to die based on the SCORTEN system, but 10 deaths were observed. Standardized mortality ratio (SMR) analysis [(Σobserved deaths/Σexpected deaths) × 100] suggested that patients with TEN treated with systemic corticosteroid were 16% more likely to die than those treated with routine therapy (SMR = 1.16; 95% confidence interval, 0.56–2.13). In the further study of combination therapy, 12 patients with TEN and eight patients with SJS were admitted. There were two deaths in the TEN group and one death in the SJS group, with 3.51 deaths expected on the basis of the SCORTEN system. SMR analysis showed that combination therapy had a tendency to reduce the mortality rate of TEN (SMR = 0.85; 95% confidence interval, 0.18–2.50). Nevertheless, in both the TEN and SJS groups, the difference in mortality rate between the two therapies was not statistically significant (P = 0.651 and P = 1, respectively). In patients with TEN, combination therapy also reduced significantly the time of arrested progression (P = 0.019) and the total hospitalization time (P = 0.043), but could not reduce the time to the tapering of corticosteroid (P = 0.96). In SJS patients, the times of arrested progression and hospitalization were also reduced significantly (P = 0.019 and P = 0.0475, respectively). Likewise, the time to the tapering of corticosteroid was not reduced (P = 0.122). Conclusion Combination therapy with corticosteroid and IVIG exhibited a tendency to reduce the mortality rate in comparison with the solo administration of corticosteroid. The decrease in the mortality rate, however, was not statistically significant. Combination therapy also arrested progression earlier and decreased the hospitalization time, meaning that the total dose of corticosteroid may be reduced. Combination therapy, however, did not lead to earlier tapering of corticosteroid. No severe adverse effects of IVIG were found during treatment.     

免疫抑制剂联合糖皮质激素治疗寻常型天疱疮疗效观察

目的:探讨环磷酰胺联合糖皮质激素治疗寻常型天疱疮的近、远期疗效。方法:收集近4年42例在我科住院经临床、病理确诊为寻常型天疱疮患者的详细资料,比较评估糖皮质激素联合硫唑嘌呤(AZP组)和糖皮质激素联合环磷酰胺(CTX组)治疗后的近、远期疗效。结果:近期疗效,AZP组痊愈率80.0%(16/20),未愈20.0%(4/20);CTX组痊愈率86.4%(19/22),未愈14.6%(3/22),两组相近,差异无统计学意义(P>0.05)。远期疗效,AZP组痊愈40.0%(8/20),控制35.0%(7/20),未控制25.0%(5/20);CTX组痊愈率81.8%(18/22),控制9.0%(2/22),未控制9.0%(2/22),两组差异有统计学意义(P<0.05)。结论:环磷酰胺静脉冲击(五日疗法)联合糖皮质激素疗法是治疗寻常型天疱疮的较好方法。     

糖皮质激素联合环磷酰胺治疗寻常型天疱疮疗效观察

目的 :　探讨糖皮质激素联合环磷酰胺 (CTX)治疗寻常型天疱疮近、远期疗效 ;方法 :　收集近 8年 5 9例在我科住院经临床、病理和直接免疫荧光确诊为寻常型天疱疮患者的详细资料 ,对糖皮质激素(对照 )和糖皮质激素联合环磷酰胺 (试验 )两组治疗后近、远期疗效进行评估 ;结果 :　近期疗效 ,对照组痊愈 78% (2 1 2 7) ,未愈 2 2 .2 % (6 2 7)。试验组痊愈 87.5 % (2 8 32 ) ,未愈 14 .8% (4 32 )。两组相近 ,差异无显著性 (P >0 .0 5 )。远期疗效 ,对照组痊愈 14 .8% (4 2 7) ,控制 37.0 % (10 2 7) ,未控制 4 8.1% (13 2 7)。试验组痊愈 31.2 5 % (10 32 ) ,控制 5 6 .2 5 % (18 32 ) ,未控制 12 .5 % (4 32 ) ,两组差异有显著性意义 (P <0 .0 5 )。结论 :　糖皮质激素联合CTX治疗寻常型天疱疮近期疗效与单用糖皮质激素相近 ,远期疗效明显优于单用糖皮质激素。     

Contact allergy to corticosteroids and Malassezia furfur in seborrhoeic dermatitis patients

Background Seborrhoeic dermatitis (SD) is a chronic skin disease, requiring long‐term treatment, which might promote sensitization. Malassezia furfur (Mf) plays an important role in seborrhoeic dermatitis. Objectives The aim of this study was to determine the frequency of contact sensitivity in SD patients. Patients and methods A total of 100 patients and 20 healthy controls (HC) were investigated: 50 suffering from SD with no previous local corticosteroid treatment (SDN), 50 SD patients treated with local corticosteroids (SDC). Mycological examination for Mf was performed. All patients were patch tested with the baseline standard, corticosteroid series, with 12 commercial corticosteroid preparations frequently used in Croatia; and also with Mf. Results Malassezia furfur was found in 44 (88%) SDN, 37 (74%) SDC, and in 4 (20%) HC; patch test reaction to Mf was positive in one SDN and in three SDC. Positive patch tests to standard allergens were observed in 17 (34%) SDN, 33 (66%) SDC and 2 (10%) HC. Patch tests to the corticosteroid series revealed positive reactions in 4 SDC and to commercial corticosteroids in seven patients, i.e. 2 SD and 5 SDC. Conclusions Patch tests to the baseline series and to both individual corticosteroid and commercial corticosteroid preparations should be performed in SD patients with persistent dermatitis, as contact‐allergic reactions may complicate their dermatitis. Sensitization to Mf was found to be infrequent.     

静脉注射丙种球蛋白和糖皮质激素治疗重症大疱性药疹65例

目的 比较静脉注射丙种球蛋白(IVIG)联合糖皮质激素与糖皮质激素单用治疗莺症大疱性药疹的疗效.方法 从1993-2007年共收集65例重症大疱性药疹病例.使用中毒性表皮坏死松解症评分(SCORTEN)进行分析.2001年后的病例采用联合治疗,丙种球蛋白剂量0.4 g·kg-1·d-1连用5 d,此前糖皮质激素治疗的病例作为对照.结果 在45例糖皮质激素治疗的病例中10例死亡,而预期死亡数8.63.标准死亡比(SMR)分析显示接受糖皮质激素治疗患者的死亡率较常规治疗高16%(SMR=1.16;95%CI 0.56-2.13).20例接受联合治疗患者中3例死亡,而预期死亡数3.51(SMR=0.85;95%CI 0.18-2.50).在中毒性表皮坏死松解症(TEN)、Stevens-Johnson综合征(SJS)患者中两种疗法死亡率差异均无统计学意义(P>0.05).在TEN患者中,联合疗法较之糖皮质激素疗法疾病停止进展时间及总住院时间缩短(t=2.46,3.14,P值均<0.05).但糖皮质激素减量时间无差异(t=-0.045,P>0.05);SJS患者结果相同(t=2.334,t=2.275,t=1.655,P<0.05,<0.05,>0.05 o结论 IVIG和糖皮质激素联合治疗较之仅用糖皮质激素治疗显示出死亡率降低的趋势,并能早期控制病情,缩短住院时间;但联合治疗并不能使糖皮质激素早期减量.     

High-dose intravenous immunoglobulins in the treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis in Chinese patients: a retrospective study of 82 cases

Stevens-Johnson Syndrome (SJS) and Toxic epidermal necrolysis (TEN) are drug-induced diseases with a low incidence but high mortality. While there is no standard treatment, corticosteroids and intravenous immunoglobulin (IVIG) therapy have been widely used, with controversy. Our objective was to summarize the etiology and therapeutic regimen of SJS or TEN in 82 hospitalized patients in China. A retrospective study was performed on 82 patients who were diagnosed with SJS or TEN and hospitalized in Peking Union Medical College Hospital from July 1994 to August 2009. Of them, 24 were treated with IVIG plus corticosteroids (IVIG group) and the other 58 were treated with corticosteroids only (corticosteroids group). SCORTEN was used to evaluate the severity and prognosis of the patients. The efficacy of therapeutic modalities was assessed by the following parameters: starting and the maximum dose of corticosteroids, cumulative dose of corticosteroids before tapering, cumulative dose of IVIG, days of corticosteroid application before its tapering and the hospitalization days. The common agents triggering SJS/TEN in these patients were non-steroidal anti-inflammatory drugs (31 cases), anti-epileptics (18 cases), antibiotics (14 cases), antipodagrics (4 cases), sulfanilamides (4 cases) and others (11 cases), respectively. Carbamazepine was the most common drug, and induced 15 cases of SJS/TEN. The SCORTEN was significantly higher in the IVIG group than that in the corticosteroid group (2.0 ± 1.7 vs 0.8 ± 1.0, P = 0.001). Whereas no differences were observed between the two groups in the parameters including starting and maximum dose of corticosteroids, cumulative dose and the number of application days of corticosteroids before tapering and hospitalization days. However, in patients whose SCORTEN scores were 2, application of IVIG and corticosteroids shortened the duration of hospitalization from 26.4 ± 9.5 d to 18.1 ± 5.3 d (P < 0.05). No significant difference was observed in the incidence of complications between the two groups (54.2% vs 39.7%, P > 0.05). The actual mortalities were 12.5% in the IVIG group and 3.4% in corticosteroid group respectively, which were significantly lower than the predicted values (22.0% and 7.2%, respectively). Standardized mortality ratio (SMR) analysis showed a trend to a lower actual mortality (not significant) with corticosteroid treatment than the predicted mortality (SMR = 0.480; 95% CI: 0.075-1.923) and combination therapy had a tendency to reduce the mortality (not significant) rate of TEN (SMR = 0.569; 95% CI: 0.318-1.910). No significant difference in SMR was found between the two groups (P = 0.1474). Survival analysis showed that a favorable overall survival was associated with younger age (P = 0.0405). Our data indicated that early application of corticosteroids presented beneficial effects on SJS/TEN, and that combination therapy of corticosteroids and IVIG achieved a better therapeutic effect than the administration of corticosteroids alone. We recommend early treatment with IVIG at total doses of more than 2 g/kg in SJS/TEN patients whose SCORTEN are higher than 0.