天疱疮121例临床分析

目的:加强对天疱疮的认识,提高治疗水平。方法:回顾性分析121例天疱疮患者的临床资料。结果:大部分天疱疮患者通过激素及免疫抑制剂的治疗均能获得临床缓解,免疫抑制剂、血浆置换、大剂量丙种免疫球蛋白冲击治疗等"辅助疗法"能减少激素用量及其所产生的远期并发症,自体外周血造血干细胞移植有望根治难治性天疱疮。结论:目前天疱疮的治疗药物仍以激素为主,但由于其副作用,一些新型的治疗方案的研究正积极展开。     

天疱疮43例临床资料分析

目的 探讨天疱疮各型的临床特征和治疗方法 .方法 对43例天疱疮住院患者的临床资料进行回顾性分析.结果 35.71%的寻常型天疱疮患者出现口腔、外阴黏膜损害,14.29%红斑型天疱疮患者出现口腔溃疡,1例增殖性天疱疮患者表现为近3年口腔黏膜破溃.直接免疫荧光确诊阳性72.72%.糖皮质激素治疗为首选,糖皮质激素联合免疫抑制剂治疗疗效好.结论 直接免疫荧光为诊断天疱疮的常用方法 ,天疱疮的分型是影响激素用量的重要因素,糖皮质激素联合免疫抑制剂是目前治疗此病的主要方法 .     

寻常型天疱疮53例临床分析

通过对1996～2002年本院53例寻常型天疱疮住院患者的临床资料回顾性分析,以探讨寻常型天疱疮临床、组织病理、免疫病理和治疗方案及预后。结果28例单用糖皮质激素(简称激素)治疗,按轻、中、重三组激素控制剂量分别相当于泼尼松(60.0±19.6)mgd、(85.6±19.8)mgd、(112.5±39.4)mgd,平均(98.2±48.8)mgd。三组之间有统计学差异。18例患者激素联合免疫抑制剂治疗,激素控制剂量相当于泼尼松(102.1±41.4)mgd。与单用激素组无统计学差异。5例采用激素冲击疗法。2例患者死于激素副作用,死亡率为3.9%。寻常型天疱疮治疗首选激素,若控制不满意可用激素冲击疗法或联合免疫抑制剂。寻常型天疱疮预后较好,死亡率较低。     

23例中重度大疱性类天疱疮皮质类固醇激素用量分析

回顾分析23例中重度大疱性类天疱疮患者的临床资料,探讨中重度大疱性类天疱疮合理的治疗方案。结果：中度大疱性类天疱疮患者皮质类固醇激素控制量（相当于泼尼松剂量）为（57．43±11．58）mg／d,重度患者控制量为（87．26±14．52）mg／d。联合免疫抑制剂治疗,可减少皮质类固醇激素的用量,利于其较快减量,减少激素使用副作用的发生。     

重症寻常型天疱疮24例分析

目的探讨重症寻常型天疱疮的的临床特点、实验室特点以及合理的治疗方案。方法回顾分析24例重症寻常型天疱疮的临床资料。结果24例重症寻常型天疱疮患者中男女之比2.43:1,平均44.63岁,职业以农民和工人为主,患者均有躯干部受累,黏膜受累75%,尼氏征阳性19例,入院时并发症特别是感染发生率高,创面细菌培养阳性率41.7%,血培养阳性率25%,激素早期联合免疫抑制剂治疗取得满意疗效,总有效率为87.5%。结论本病多合并感染,激素联合免疫抑制剂是治疗重症寻常型天疱疮的首选治疗方案。     

天疱疮149例临床分析

目的 总结天疱疮发病的临床规律和特点,探寻最佳治疗方案,为科学治疗天疱疮和改善预后提供依据。方法 回顾性分析1994年4月-2011年1月昆明医学院附属二院皮肤科收治的149例天疱疮住院患者的临床资料。结果 男71例,女78例;平均年龄(46.24±13.10)岁。轻症26例,中症58例,重症65例。轻、中和重症天疱疮患者激素治疗初始用量分别为(42.15±11.60)mg,(76.94±25.80mg)和(104.02±14.31)mg(P<0.05);重症患者中激素联合免疫抑制剂组病情缓解率(82.05%,32/39)高于激素组(57.69%,15/26,P<0.05)。结论 天疱疮好发于中年人,无性别差异。口腔/头皮受累常提示病情严重;激素用量根据病情严重程度而定,激素联合免疫抑制剂治疗优于单用激素;感染是其主要并发症;疾病类型、病情严重程度及是否合并低蛋白血症是影响预后的重要因素。     

天疱疮治疗的研究进展

天疱疮是一种罕见且可危及生命的自身免疫性大疱性皮肤病。依据临床及病理特征,天疱疮分为寻常型、增殖型、落叶型及红斑型四种类型。天疱疮治疗目的是尽快控制病情、修复松解的表皮,减少或延缓复发、预防感染、减少药物副作用、提高患者生活质量。对于病情轻中度的患者,外用或系统应用中强效糖皮质激素仍是首选,联合应用免疫抑制剂或生物靶向制剂治疗难治性天疱疮有望取得满意疗效,对于不能耐受激素和免疫抑制剂等治疗抵抗的患者,造血干细胞移植带来了新的希望。本文旨在归纳总结出针对不同病情、患者情况的个性化方案。     

2%磺胺嘧啶银外用治疗天疱疮创面3例

２％磺胺嘧啶银外用治疗天疱疮创面３例江萍①蒋健②覃江炜①近年来我们对３例合并感染的重度寻常型天疱疮（ＶＰ）住院患者，在给予抗生素、皮质类固醇激素及免疫抑制剂治疗的同时，辅以２％磺胺嘧啶银、烧伤罩治疗，获得良效，现报告如下。１材料和方法１．１临床资料：...     

天疱疮31例护理体会

目的　探讨护理在天疱疮治疗中的重要性。方法　通过严密观察病情变化,及时发现激素及免疫抑制剂的副作用,并保护创面,严格消毒隔离,进行心理护理和健康教育。结果　促进了创面愈合,减少了继发感染,增强了患者战胜疾病的信心,很好地配合了临床治疗,使31例天疱疮患者顺利出院。结论　精心护理是天疱疮患者康复的重要保证。     

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。     

自体外周血干细胞移植治疗难治性天疱疮一例

目的 探讨自体外周血干细胞移植治疗天疱疮的可能性.病历资料男性患者,35岁,全身反复大疱4年余,根据临床表现、组织病理和免疫荧光检查确诊为天疱疮.经糖皮质激素和免疫抑制剂治疗效果不佳,2月前出现髓关节疼痛,经X线和CT检查诊断为股骨头无菌性坏死.采取环磷酞胺及粒细胞集落刺激因子作为动员剂,利妥昔单抗联合净化.用环磷酞胺联合抗胸腺细胞球蛋白、利妥昔单抗进行预处理,同时碱化和水化尿液,保护心、肝和肾功能.观察移植后临床表现、造血和免疫指标等的变化.结果 患者移植结束即停用糖皮质激素,3周后自觉双侧靛关节疼痛减轻,复查CT示股骨头缺血性改变减轻.移植6周后直接免疫荧光和间接免疫荧光检查转阴并持续阴性.经一年复诊,临床及免疫学检查均无复发迹象.结论 本研究结果提示自体外周血干细胞移植治疗天疙疮近期疗效显著,可能是治疗存在治疗抵抗的重症天疤疮潜在的“根治”方法,但其风险收益比尚需进一步评价.     

天疱疮临床治疗进展

传统上治疗天疱疮首选使用糖皮质激素或(和)免疫抑制剂,但部分患者存在药物不敏感、减药困难以及治疗中出现严重的药物副作用情况,而近年来新的药物研发及治疗方法的应用,成为治疗天疱疮的新选择,本文就此方面的进展做一综述。     

Pemphigus

Pemphigus is an uncommon but potentially life-threatening chronic autoimmune bullous disorder. Antibodies are directed against antigens (desmoglein 1 and 3) in the desmosomes linking keratinocytes and against acetylcholine receptors. Conventional treatment with high-dose corticosteroids, sometimes with adjuvant immunosuppressive agents, may be associated with very serious adverse effects. There is an urgent need to establish the evidence for the safest and most effective form of treatment. A literature review has revealed 11 controlled (9 randomized) trials of treatment for pemphigus. The numbers of participants in the individual trials are small and the data cannot be pooled as they evaluate different forms of treatment. The results of these trials suggest that very high doses of corticosteroids, either as pulse therapy or in daily dosage, are not superior to moderate daily doses. Based on evidence from the available trials, addition of an immunosuppressive agent generally does not appear to offer substantial benefit in terms of clinical response. However, a recent study demonstrated a significant reduction in corticosteroid requirements among patients receiving immunosuppressive agents. Newer therapies, such as biologic agents (in particular rituximab), calcineurin inhibitors, or immunoadsorption appear promising but there are inadequate controlled trials to establish their role clearly. Initial open-label studies suggest that specific peptide immunotherapy may offer a safe and novel approach to the treatment of pemphigus in the future. At present, treatment of an individual patient with pemphigus requires clinical judgment and should not be based purely on guidelines or on the inadequate available evidence alone. There is an urgent need for large randomized, controlled, multicenter trials of treatment in patients with pemphigus.     

Treatment of pemphigus vulgaris with pulse intravenous cyclophosphamide.

BACKGROUND--Although corticosteroids have dramatically altered the prognosis in pemphigus vulgaris, morbidity and mortality from systemic corticosteroid side effects remains high. While immunosuppressive agents have been successfully used in pemphigus vulgaris, there is a high incidence of side effects with these agents as well. Particularly bothersome are reports of increased risk of malignancy with long-term use of immunosuppressive agents. For these reasons, we used a protocol that includes low-dose oral cyclophosphamide coupled with pulse intravenous cyclophosphamide in two patients with recalcitrant pemphigus vulgaris. OBSERVATIONS--Both patients responded well to monthly doses of intravenous cyclophosphamide with rapid decrease in the frequency and severity of blistering, resulting in resolution of their disease after 7 and 10 months, respectively. CONCLUSIONS--Pulse doses of immunosuppressive agents appear to be successful in the treatment of pemphigus vulgaris. High-dose steroid therapy can be tapered with the use of this treatment. Because monthly intravenous doses of cyclophosphamide lead to a substantially reduced cumulative dose, when compared with standard oral regimens, the risk of developing malignancy may also be reduced. Further studies using larger groups of patients are needed to evaluate the efficacy of pulse intravenous cyclophosphamide in pemphigus vulgaris. Long-term follow up will be necessary to compare the incidence of malignancy in patients receiving pulse doses of immunosuppressive agents with that in patients receiving continuous oral treatment.     

天疱疮或大疱性类天疱疮合并单纯疱疹病毒感染8例临床特征及治疗分析

目的回顾分析天疱疮/大疱性类天疱疮(BP)合并单纯疱疹病毒(HSV)感染的临床特征及治疗方法。方法回顾分析2016—2021年在武汉市第一医院住院治疗的天疱疮/BP合并HSV感染病例的临床特征及治疗和随访情况。结果 8例天疱疮/BP合并HSV感染患者中, 男2例, 女6例, 年龄(50.6 ± 8.3)岁, 包括5例寻常型天疱疮, 1例落叶型天疱疮, 2例BP。7例合并HSV-1感染, 1例合并HSV-2感染。8例均因天疱疮或BP接受系统糖皮质激素及免疫抑制剂治疗, 并对治疗抵抗入院, 其中7例表现为原发病灶加重或复发, 1例表现为全身皮损增加。HSV感染位于躯干4例, 口腔4例, 头皮3例, 面部2例。皮疹表现为不规则的糜烂面, 伴血痂, 部分为中央有脐凹的脓疱, 7例伴有皮疹处明显疼痛。发生HSV感染时, 6例天疱疮患者抗Dsg1抗体均下降, 5例寻常型天疱疮中4例抗Dsg3抗体下降;2例BP患者中1例抗BP180抗体降低, 1例升高。予足量足疗程抗病毒治疗(伐昔洛韦或更昔洛韦治疗7 ～ 14 d), 所有患者HSV感染均被控制, 自身免疫性大疱性皮肤病严重程度评分较抗病毒治疗前均...     

天疱疮治疗的研究进展

天疱疮是一组威胁患者生命的自身免疫性大疱性疾病.传统的系统糖皮质激素和免疫抑制剂治疗虽可降低患者的死亡率,但常因其不良反应而限制了临床应用.随着对天疱疮发病机制研究的不断深入,一些新的治疗方法正逐步应用于临床或正处于试验研究阶段,如清除血清中的致病性抗体、生物制剂(CD20拮抗剂、肿瘤坏死因子-α拮抗剂)、p38促丝裂原活化蛋白激酶抑制剂、胆碱能受体激动剂、CD40/CD154阻断剂、桥粒芯蛋白3合成肽等.目前,由于上述药物价格昂贵、缺乏可靠的临床疗效数据或尚处于试验阶段等多种原因,尚未在临床治疗中广泛应用.

Abstract：

Pemphigus is a group of life-threatening autoimmune blistering disorders. Although conventional therapies with systemic corticosteroids and immunosuppressive agents can reduce the mortality from pemphigus, complications have restricted their clinical application. With new insights into the pathogenesis of pemphigus, some novel treatment modalities, such as clearance of pathogenic antibodies in sera, biologic agents (CD20 antagonist, tumor necrosis factor-α antagonist), p38 mitogen-activated protein kinase inhibitor, cholinergic receptor agonists, CD40/CD154 blockade, desmoglein 3 synthetic peptide, etc,have been gradually introduced into the treatment of pemphigus. However, high expense and lack of reliable clinical data have limited the wide use of these new modalities in pemphigus.     

Efficacy and safety of long-term mycophenolate sodium therapy in pemphigus vulgaris

Background  Pemphigus is a chronic immunobullous disease, characterized by formation of blisters and erosions in skin and/or mucous membranes. This severe disease requires systemic immunosuppressive therapy. However, some patients are refractory, and long-term use of immunosuppressive agents can cause serious side-effects. Mycophenolic acid is increasingly being used as a corticosteroid-sparing agent or as monotherapy in immunosuppressive regimens.
Objectives  We aimed to evaluate the effectiveness of mycophenolate sodium, a sodium salt of mycophenolic acid, in the treatment of pemphigus vulgaris.
Methods  Six patients who were diagnosed as pemphigus vulgaris with active, refractory disease were treated with mycophenolate sodium. Three patients received mycophenolate sodium monotherapy; three patients received mycophenolate in combination with steroid. All patients were monitored regularly.
Results  Mycophenolate sodium was well tolerated with a similar efficacy of mycophenolate mofetil, and no side-effects have been observed.
Conclusions  Mycophenolate sodium appears to be an effective and safe alternative in the treatment of pemphigus vulgaris.     

Oral pemphigus vulgaris associated with HIV infection

Vesiculobullous disease in association with HIV infection is extremely rare. This report details the presentation and management of pemphigus vulgaris in an individual with HIV infection. The clinical characteristics and response to therapy appear not to be modified by coexistent HIV infection. However, the management of pemphigus vulgaris relies on immunosuppressive therapy potentially resulting in HIV disease progression. Cyclosporine has in vitro antiretroviral activity and, currently, should be considered as a possible treatment for individuals with pemphigus vulgaris and HIV infection.     

Juvenile pemphigus

Seven cases of juvenile pemphigus vulgaris and one case of juvenile pemphigus foliaceus are presented. A detailed review of the literature is also presented. The majority of patients with juvenile pemphigus develop pemphigus vulgaris. The patients present with a wide clinical spectrum. The oral cavity is frequently involved. The mean duration is 4 years. Clinical follow-up is similar to the adult variety. Several patients warrant high doses of systemic corticosteroids and develop serious side effects, most notably growth retardation and opportunistic infections. Short-term judicious use of immunosuppressive agents is advocated. Dapsone is a helpful adjuvant to therapy. It is proposed that relevant immunofluorescent studies be done very early. Early diagnosis and therapy are associated with a better prognosis.     

天疱疮51例临床病理分析

目的:分析天疱疮的临床和病理特性。方法:分析51例天疱疮患者的临床病理资料。结果:约64.71%(33/51)患者合并系统性疾病,其中以糖尿病居多,占29.41%(15/51);皮损组织病理和免疫病理检查具有特异性。结论:目前治疗天疱疮最有效的药物为糖皮质激素,联合应用免疫抑制剂可尽快控制症状、减少糖皮质激素用量及其副作用。