68例大疱性类天疱疮回顾性临床分析

回顾分析68例大疱性类天疱疮患者的临床资料.探讨大疱性类天疱疮的临床特征和合理的治疗方案.结果轻、中症大疱性类天疱疮患者糖皮质激素控制量(按泼尼松计算)为(56.96±14.89)mg/d,重症患者控制量为(86.67±15.92)mg/d.目前系统使用糖皮质激素仍是治疗大疱性类天疱疮的主要药物.     

大剂量静注丙球冲击疗法治疗皮肤病5例

采用大剂量静注丙型冲击疗法（ＨＤＩＶＩＧ）治疗２例伴发肿瘤的泛发性太疱疹患者,常规治疗效果欠佳的皮肌炎,大疱性类天疱疮和系统性红斑狼疮患者各１例,剂量为０．４／（ｋｇ．ｄ）共５天,并辅以中等剂量的皮质类固醇激素。经治疗虱的症状迅速缓解,治疗中无明显不良反应。     

天疱疮的治疗进展

天疱疮（pemphigus）是皮肤科较为常见的获得性表皮内大疱病，是一组较严重的慢性、复发性大疱性皮肤黏膜疾病。现已证实本病为一种自身免疫性疾病。应用皮质类固醇激素治疗，可使天疱疮预后大为改观。近年来皮质类固醇激素与免疫抑制剂联合应用以及在此基础上与血浆置换疗法，大剂量免疫球蛋白静脉滴注联合应用，使天疱疮得到有效控制，甚至长期缓解，使天疱疮的治愈成为可能。     

类天疱疮60例回顾性分析

目的：探讨类天疱疮的临床分型标准及比较合理的治疗方案,了解类天疱疮与其它疾病之间的关系。方法：回顾性分析了60例类天疱疮住院患者,包括患者发病年龄、病程、住院日、皮损分布、组织病理、直接和间接免疫荧光、常规实验室检查、站疗方案、合并症及并发症等。结果：泛发型类天疱疮和局限型类天疱疮在临床、病理表现、实验室检查及治疗所用皮质类固醇剂量等方面均有显著不同。泛发型组55.6％（25/45）发生粘膜损害,14．29％（5／45）合并恶性肿瘤。治疗本病以系统用皮质类固醇（泼尼松）为主,初始日剂量泛发型组为49．22±13．94mg,局限型组为27．67±16.35mg（P＝0.000001）;激素最大量分别为65．82±36.13mg和34±20.28mg（P=0.001）。使用最大剂量的维持时间两组之间无显著性差异。但是两组所用维持量不同（分别为23．45mg日和13．5mg／日,P＝0．0292）。结论：类天疱疮病情严重程度差异较大,局限型和泛发型类无疱疮的临床和试验室特点以及治疗方案等方面均不相同。     

结节性类天疱疮

结节性类天疱疮是大疱性类天疱疮的罕见型,多见于老年患者,主要临床表现是结节、水疱,病理、直接免疫荧光、间接免疫荧光显示大疱性类天疱疮特征。治疗上需系统应用皮质类固醇激素,有时需合并使用免疫抑制剂。免疫印迹试验发现多数PN患者血清与230kD的表皮抗原相结合。     

大疱性类天疱疮89例临床分析

目的 探讨大疱性类天疱疮患者的临床表现、实验室检查和治疗的特点。方法 回顾性分析89例大疱性类天疱疮患者的临床资料。结果 89例患者,男女之比1.07:1,平均发病年龄58岁。皮损除典型的水疱、红斑外,还有多形红斑、疱疹样皮炎样损害。33.7%的患者有口腔粘膜损害,6.7%的患者以口腔水疱、糜烂为首发症状。18%的患者尼氏征阳性。间接免疫荧光法检测阳性率74.4%,直接免疫荧光法检测阳性率94.9%.皮质类固醇以及皮质类固醇联合免疫抑制剂是治疗大疱性类天疱疮的主要手段,除接受冲击治疗的患者外,控制皮损所需的皮质类固醇剂量平均值为65.5mg(相当于泼尼松).结论 组织病理和免疫荧光检测是确诊的主要依据,控制皮损的皮质类固醇最大用量存在个体差异。     

107例天疱疮回顾性研究

通过回顾性分析107例天疱疮住院病例临床资料，研究其病情受控前的各项指标，发现病情的轻重是影响皮质类固醇（下称激素）初始量、激素减量前时间、减量前激素总量、最大控制量和激素总量等指标的重要因素；加用免疫抑制可减少激素用量；红斑型大疱疮和疱疹样天疱疮病情轻，容易控制。对其中的55例进行了随访，发现患者的死亡率与年龄、天疱疮类型和有无应用免疫抑制剂有关，缓解率与病情轻重、住院间最大控制量和天疱类型相关。     

环孢菌素治疗大疱性类天疱疮和寻常天疱疮的效果

类固醇药治疗自身免疫性大疱性疾患(大疱性类天疱疮和寻常天疱疮)有效,但由于其副作用严重,病人难以耐受。环孢菌素主要作用于辅助淋巴细胞,而且副作用少。本文报告以环孢菌索(6mg/kg/d),血浆浓度为80～180μg/l)治疗2例大疱性类天疱疮和2例寻常天疱疮的情况。寻常天疱疮2例,曾用泼尼松治疗好转,但减量治疗后复发,泼尼松量增至1mg/kg/d不能控制复发,故决定在不增加泼尼松剂量的情况下给予环孢菌素口服试验治疗(2例剂量相同)。结果分别于10天和15天治愈。3     

天疱疮与类天疱疮的治疗

天疱疮与大疱性类天疱疮是最为常见的获得性大疱病。 50年代，皮质类固醇的问世并应用于大疱病的治疗，使这 2个重症皮肤病的预后大大得到改善。早期制定的大剂量服用皮质类固醇方案虽然能控制皮疹，但长期服药的结果也带来一系列的不良反应，如糖尿病、高血压、骨质疏松、股骨头无菌坏死等，甚至因不良反应导致死亡，如严重的感染、消化道溃疡穿孔造成大出血等。目前，对大疱病的治疗可选择不同的药物，然而皮质类固醇仍是治疗天疱疮、类天疱疮的首选药物，但如何合理使用，如何减少和预防不良反应的发生则是临床医生共同探讨的问题。本文…     

局限型大疱性类天疱疮1例

患者女,75岁,手、足起红斑、大疱伴瘙痒8月。皮损局限于手足,结合组织病理、免疫病理确诊为局限型大疱性类天疱疮。予口服氨苯砜、四环素、烟酸片及外用强效皮质类固醇激素等治疗后症状明显缓解。     

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。     

盐酸非索非那定片递减疗法治疗慢性自发性荨麻疹的疗效观察

目的：探讨盐酸非索非那定片递减疗法治疗慢性自发性荨麻疹的有效性。方法在临床病史评估和自体血清皮肤试验（ASST）的基础上,将80例慢性自发性荨麻疹患者随机分配到常规剂量组和递减剂量组。常规剂量组给予口服盐酸非索非那定片120 mg/d,连续12周;递减剂量组前4周每日给予口服盐酸非索非那定片120 mg/d,第5周和第9周时尝试逐渐减量至可控制症状的最小服药量。在治疗前（基线）和治疗后第4、8、12周时评价所有患者荨麻疹疾病活动程度（UAS）、皮肤病生活质量指数（DLQI）和抗组胺药物总服用量。结果共76例患者完成12周的治疗,包括常规剂量组37例和递减剂量组39例。治疗4、8、12周后,常规剂量组（UAS 0.64±0.82、0.37±0.68、0.27±0.56;DLQI：3.62±1.82、2.81±1.65、1.37±1.14）和递减剂量组（UAS：0.61±0.87、0.48±0.72、0.28±0.61;DLQI：3.79±2.57、2.74±2.11、1.15±1.47）UAS、DLQI评分均分别显著低于各组治疗前评分（UAS：4.08±0.79、4.07±0.81;DLQI：16.19±3.79、15.92±4.2）,差异有统计学意义（均P<0.001）,但两组在治疗后同一时间UAS和DLQI评分差异均无统计学意义（P>0.05）。递减剂量组在治疗8、12周后分别有71.79%（28/39）和82.05%（32/39）患者在原有剂量基础上减少剂量仍能控制症状,且服药总量显著低于常规剂量组对应时间段的药量（均P<0.001）。结论慢性荨麻疹患者使用盐酸非索非那定治疗4~8周后,逐步减少药物剂量可取得与常规剂量类似的临床疗效。     

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.     

Proteinase inhibitors and pemphigus vulgaris. An in vitro and in vivo study

The aim of this study was to determine the inhibitory effect of clinically usable proteinase inhibitors p -aminomethylbenzoic acid (PAMBA), and aprotinin on acantholysis in skin organ culture and in clinical trials with pemphigus patients. PAMBA added to the culture medium at a concentration of 1 mg/ml fully prevented the acantholysis, while Contrykal at 10 ATrE/ ml reduced acantholysis. Subsequently, we treated 12 patients (group 1) with PAMBA 100–200 mg daily for 7 to 26 days in combination with a moderate dose of corticosteroid (mean dose 36.1 mg prednisolone equivalent) or immunosuppressive drugs. A second group of 12 patients (group 2) were treated with a high dose of corticosteroid (mean 94.2 mg prednisolone equivalent) and immunosuppressive drugs. Evaluation was performed before treatment, after 3 weeks and on discharge using a clinical scoring system. The inclusion of PAMBA in the treatment protocol of group 1 resulted in active disease being brought under control with lower corticosteroid doses. As a result, fewer side effects were observed in group 1 than in group 2. In our opinion, protease inhibitors may be useful as adjuvant drugs in the combination therapy of pemphigus. Received: 2 November 1995     

不同剂量甲基强的松龙联合神经节苷脂治疗大鼠急性脊髓损伤

目的比较两种不同剂量的甲基强的松龙（MP）与单唾液酸神经节苷脂（GM—1）联合应用治疗大鼠实验性脊髓损伤的效果。方法36只大鼠随机分为3组，复制挤压性脊髓损伤动物模型，分别应用大剂量MP和两种不同剂量MP与GM—1联合治疗，术后24h、7d分别采血测定各组血清丙二醛（MDA）含量和后肢运动功能改良Tarlov评分。结果术后24h各组血清MDA含量显著升高，改良Tarlov评分显著降低，术后7d以上指标均有不同程度恢复，联合用药组较单独应用大剂量MP组恢复明显。其中，以小剂量MP＋GM-1组恢复效果最明显。结论小剂量MP和GM-1联合应用治疗大鼠实验性脊髓损伤的效果优于另外两种药物疗法。     

Studies of blood histamine levels during topical corticosteroid therapy compared with those during systemically administered corticosteroids

Blood histamine levels during topical corticosteroid therapy have not been reported, although decreased blood histamine levels are known to occur after the systemic administration of corticosteroid. Blood histamine levels and serum 11-hydroxycorticosteroids (11-OHCS) after systemic corticosteroid were compared with those after topical corticosteroid. Both blood histamine and serum 11-OHCS levels decreased in a parallel and dose-dependent manner during systemic administration of 0.5-1.0 mg/day of betamethasone. Blood histamine levels increased and serum 11-OHCS levels decreased during systemic administration of 0.25 mg/day to the betamethasone and topical corticosteroid group. Changes in blood histamine levels in the topical corticosteroid group appeared between 0.25 and 0.5 mg/day of systemically administered betamethasone. Fifteen cases of dermatological inpatients suffering from wide-spread eczema or exfoliative dermatitis were treated with the topical corticosteroids betamethasone 17-valerate, budesonide and diflorasone diacetate. In each case, blood histamine levels were examined and an equipotent dose of oral administeration of betamethasone was calculated. Thirteen cases in whom blood histamine levels were measured fell into the dosage range from 0.1 to 0.5 mg/day. In each case, serum 11-OHCS levels were also examined and an equipotent dose of oral administration of betamethasone was calculated by the same method. Fourteen cases in whom serum 11-OHCS levels were measured compared to a dose of less than 1.0 mg/day of betamethasone. Blood histamine levels in rats treated with 20 or 50 mg/kg/day of prednisolone were decreased significantly, and peripheral total leukocytes, lymphocytes, neutrophils and basophils were also decreased significantly.     

糖皮质激素治疗进展期白癜风疗效分析

目的:评价糖皮质激素治疗进展期白癜风的疗效及不良反应。方法:甲泼尼龙组进展期白癜风185例(VIDA评分4分,皮损面积1%),予甲泼尼龙片0.48 mg/kg·d口服3日,后0.24 mg/kg·d口服27日,第2个月减为0.12 mg/kg·d,第3个月减为0.12 mg/kg·d;复方倍他米松注射液组107例(VIDA评分3分或4分,皮损面积1%),成人予复方倍他米松注射液1.0 m L,儿童予0.015 m L/kg肌肉注射,1次/月,连用3次。结果:治疗3个月后,甲泼尼龙组和复方倍他米松注射液组有效率分别为93.0%和82.3%(P0.05)。甲泼尼龙组不良反应发生率为82.7%,显著高于复方倍他米松注射液组62.6%(P0.05)。结论:系统使用甲泼尼龙治疗进展期白癜风疗效优于复方倍他米松肌肉注射,但不良反应发生率相应增高。     

Successful use of acitretin in conjunction with narrowband ultraviolet B phototherapy in a child with severe pustular psoriasis, von Zumbusch type

Severe pustular psoriasis von Zumbusch type is a therapeutic challenge not only in adults, but even more in children. We report a 3(1/2)-year-old boy who developed a generalized flare of diffusely scattered pustules on erythematous skin which rapidly progressed to large exuding areas. The clinical presentation and investigations including histopathological examination of a biopsy and negative bacterial cultures were consistent with the diagnosis of pustular psoriasis von Zumbusch type. Upon initial treatment with methylprednisolone, acitretin and antibiotics the extent of the disease declined. However, several attempts to reduce the dose of the oral corticosteroid were followed by immediate severe flares. Additional treatment with narrowband ultraviolet B (NB-UVB, 311-313 nm UVB) resulted in a rapid arrest of disease activity and allowed the corticosteroid to be tapered off. After 10 irradiations the patient was both off steroid and disease free. NB-UVB therapy was subsequently reduced to twice-weekly exposures and acitretin gradually diminished to a maintenance dose of 0.3 mg kg(-1) daily. We conclude that NB-UVB in conjunction with acitretin is a potent therapeutic regimen for the treatment of severe pustular psoriasis von Zumbusch type in childhood.     

依巴斯汀三倍剂量治疗轻中度特应性皮炎的疗效和安全性

目的：评价依巴斯汀三倍剂量治疗特应性皮炎的有效性和安全性。方法：选取轻度至中度的特应性皮炎患者为研究对象,随机分为观察组和对照组。观察组口服依巴斯汀片30 mg/d联合医用凡士林外用,对照组口服依巴斯汀片10 mg/d联合医用凡士林外用,观察两组患者在28天治疗后的瘙痒评分、SCORAD总分、临床总有效率以及发生的不良反应。结果：共收集患者128例,其中观察组和对照组各64例,观察组的瘙痒评分、SCORAD总分分别为3.02±1.19和21.83±3.05均显著低于对照组的6.77±1.33和29.28±4.91（P<0.05）;观察组临床总有效率为93.33%高于对照组的68.75%,差异有统计学意义（P<0.05）;观察组不良反应发生率为6.3%,对照组为9.4%,两组不良反应比较无统计学差异（P＞0.05）。结论：与常规剂量的依巴斯汀比较,30 mg/d的依巴斯汀治疗特应性皮炎疗效优于10 mg/d,不良反应无明显差异。     

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.