自身免疫性疱病与银屑病的相关性

自身免疫性疱病（AIBD）和银屑病的发病机制有部分相似性。近年来,多项研究报道银屑病与AIBD之间具有相关性,以大疱性类天疱疮多见,还包括寻常型天疱疮、红斑型天疱疮、线状IgA大疱性皮病等,多数AIBD在银屑病发病后发生,部分患者两病同时出现或AIBD先出现。本文综述AIBD与银屑病发病的相关性及可能存在的机制。     

10例寻常型银屑病合并大疱性类天疱疮临床分析

目的：总结寻常型银屑病合并大疱性类天疱疮患者的临床特征及治疗方法。方法：回顾性分析2016年1月至2018年12月我院住院患者中诊断为寻常型银屑病合并大疱性类天疱疮的临床资料。结果：共发现10例寻常型银屑病合并大疱性类天疱疮患者,其中男9例,女1例,平均年龄为（59.90±10.18）岁,银屑病均先于大疱性类天疱疮发病。8例患者水疱发生于银屑病斑块上,2例水疱发生于外观正常的皮肤上。9例患者采用糖皮质激素和/或免疫抑制剂治疗,1例患者应用口服四环素、烟酰胺配合光疗治疗。出院后随访150~1065天,7例患者无新发水疱,银屑病皮损病情稳定;2例失访,1例死亡。结论：寻常型银屑病合并大疱性类天疱疮临床相对少见,中老年患者居多,糖皮质激素联合免疫抑制剂治疗有效。     

13例线状IgA大疱性皮病临床病理特征分析

收集2012年7月至2019年6月在本科室诊治的13例LABD,其中男11例,女2例.儿童型6例,成人7例.临床表现:红斑基础上或外观正常的皮肤上出现水疱和大疱,疱壁紧张,疱液清亮,尼氏征(-).皮损组织病理均见表皮下疱,直接免疫荧光见IgA线状沉积于基底膜带.1例患者合并类风湿关节炎,1例患者合并器质性焦虑障碍,1例...     

丙种球蛋白联合糖皮质激素治疗自身免疫性大疱病26例观察疗效

目的评价大剂量丙种球蛋白在治疗自身免疫性大疱病方面的疗效。方法对应用较大剂量糖皮质激素1周以上疗效不佳的自身免疫性大疱病患者加用大剂量丙种球蛋白静脉注射治疗。结果所有患者应用丙种球蛋白后均取得明显效果,92.3%的患者2～5天后水疱停止发生,尼氏征转阴,黏膜损害消退。结论大剂量静脉注射丙种球蛋白对大剂量糖皮质激素疗效不佳的重症患者是一种很好的辅助治疗方法。     

利妥昔单抗治疗自身免疫性大疱病

自身免疫性大疱性皮肤病是一类少见的重症疾病,主要包括天疱疮和类天疱疮.近年来,多种生物制剂和靶向治疗陆续应用于自身免疫性大疱性皮肤病.目前在美国与欧洲,利妥昔单抗已作为中重度寻常型天疱疮的一线治疗方案.本文将对利妥昔单抗治疗自身免疫性大疱病的作用机制、治疗方案以及安全性做一概述.     

寻常性银屑病合并大疱性类天疱疮l例

患者男，50岁，躯干、肘、膝红斑鳞悄10余年，3月前在红斑基础上出现水疱，不易破溃，轻度瘙痒，而就诊于我院皮肤科。患者10年前无明显诱因躯干四肢起散在红斑、丘疹、     

自身免疫性大疱性皮肤病的临床研究

目的:探讨各种自身免疫性大疱病的临床特征和合适的治疗方法。方法:对135例自身免疫性大疱病进行回顾性分析。结果:天疱疮、类天疱疮和线状IgA大疱性皮病是自身免疫性大疱病中常见的3种疾病,分别易累及中年人、老年人和儿童。寻常型天疱疮伴发口腔黏膜受累多见(73.1%)。在病情得到控制的患者中,重症患者糖皮质激素用量泼尼松(78.7±23.9)mg/d显著高于轻、中症患者泼尼松(45.8±19.8)mg/d和(59.4±20.8)mg/d,P<0.001;寻常型、落叶型天疱疮糖皮质激素用量(66.2±24.3)mg/d、(73.0±14.9)mg/d显著高于红斑型(49.1±21.8)mg/d,P<0.05;寻常型天疱疮痊愈率(24.2%)显著低于红斑型(63.6%),P<0.05。接受相近剂量的糖皮质激素治疗,类天疱疮痊愈率(56.1%)明显高于天疱疮(35.5%),P<0.05。结论:应根据不同情况确定糖皮质激素合适的初始剂量。对于线状IgA大疱性皮病和IgA型大疱性系统性红斑狼疮,氨苯砜是首选治疗药物。     

银屑病并发免疫性大疱性皮肤病11例临床分析

目的:对银屑病并发免疫性大疱性皮肤病患者的临床相关因素及其临床特征进行回顾性分析.方法:收集11例银屑病并发免疫性大疱性皮肤病患者资料,对其临床特征、大疱病的诱发因素、免疫病理以及治疗等方面进行回顾性分析;并对部分患者的特异性致病性抗体进行检测.结果:11例患者中1例为女性,10例为男性,平均年龄(70.55±11.08)岁.患者在银屑病发生后平均(15.36±9.19)年出现大疱性皮肤病;4例患者在出现大疱病前有明确的窄谱中波紫外线(NB-UVB)照射史.7例患者并发天疱疮,4例并发类天疱疮.7例天疱疮患者中4例免疫荧光检查阳性,而阴性的2例患者中外周血检出高滴度抗Dsg1抗体,另有1例结果不详;4例类天疱疮患者中3例患者免疫荧光检查阳性,2例患者抗BP180抗体阳性.这些患者应用小剂量糖皮质激素联合免疫抑制剂,对并发的免疫性大疱病均有较好疗效,对原有的银屑病亦有一定的疗效.结论:银屑病并发免疫性大疱性皮肤病临床上较为罕见,对该病的正确认识,有助于早期合理的临床治疗,尽早控制病情.     

儿童自身免疫性大疱病15例分析

自身免疫性大疱病是以自身抗体在皮肤内沉积、水疱形成为特征的一组皮肤病,包括寻常型天疱疮(PV)、类天疱疮(BP)、获得性大疱性表皮松解症(EBA)、线状IgA大疱病(LAD)及大疱性系统性红斑狼疮(BSLE)等,常见于中老年人,偶尔也可发生于儿童。为了解此类疾病在儿童中的发病情况、临床表现及治疗特点,我们对1991～2000年诊治的15例儿童自身免疫性大疱病进行总结分析。     

寻常性银屑病合并大疱性类天疱疮1例

1临床资料 患者男,50岁,躯干、肘、膝红斑鳞屑10余年,3月前在红斑基础上出现水疱,不易破溃,轻度瘙痒,而就诊于我院皮肤科.患者10年前无明显诱因躯干四肢起散在红斑、丘疹、鳞屑,伴瘙痒.     

Prevalence of infectious diseases in patients with autoimmune blistering diseases

A long-term immunosuppressive treatment can provoke latent infections. Autoimmune blistering diseases (AIBD) are mostly treated with systemic immunosuppressive agents. To prevent the reactivation or exacerbation of existing latent infections, patients must be screened for infectious diseases before immunosuppressive treatments are initiated. However, the prevalence of infectious diseases in AIBD remains to be elucidated. To evaluate the necessity of screening infectious diseases in AIBD, we retrospectively reviewed the clinical records of 215 patients at a single center with AIBD for hepatitis B virus (HBV), hepatitis C virus (HCV), Mycobacterium tuberculosis, Treponema pallidum, human T-cell leukemia virus type 1 (HTLV-1) and HIV infections. Approximately 40% of patients were infected with HBV. During systemic corticosteroid treatment, HBV DNA became positive in 3.4% of cases. Antibodies to HCV, interferon-γ release assays for M. tuberculosis and the T. pallidum latex agglutination test were positive in 0.6%, 6.6% and 1.2% cases, respectively. Neither HTLV-1 nor HIV infections were detected. In conclusion, checks for HBV and M. tuberculosis infections should be made before immunosuppressive treatments are started, because of the high prevalence of these potentially life-threatening infections. Other infections should be tested for depending on the patient’s risk factors.     

泛发性脓疱型银屑病12例临床分析

对12例泛发性脓疱型银屑病作临床分析，发现其中多数患者因药物诱发，皮质类固醇激素、全反式维甲酸及免疫抑制剂联合治疗取得满意疗效。     

脓疱型银屑病64例临床分析

报告了64例脓疱型银屑病,按照国际上通行的分类方法进行分类、诊断,并对病史资料、临床特征和治疗经过进行分析,提示脓疱型银屑病的临床表现和治疗反应在不同患者之间差异较大。新的分类方法对指导临床具有积极意义。     

Approach to the patient with autoimmune mucocutaneous blistering diseases

Autoimmune mucocutaneous blistering diseases (AMBD) are a rare group of dermatoses that can be potentially fatal. There are many subtypes and their clinical presentation can vary from being localized to general involvement. It is crucial that a diagnosis be made as early as possible and appropriate treatments are implemented. This article will discuss the diagnosis and available treatments of the major AMBDs. There are very few case-controlled studies regarding the treatments of these diseases. Most of the treatments used for these diseases are based on anecdotal reports. Hence, a synopsis of the conventional treatments and some brief recommendations will also be discussed. A brief discussion regarding "rescue" therapies that have been used for those patients with more recalcitrant cases of AMBD will also be presented.     

抗肿瘤药物治疗银屑病诱发白血病146例临床分析

以抗肿瘤药物治疗银屑病可诱发白血病，本文详述其致白血病的药物种类，不同型的白血病，症状及预后。     

Clinical analysis of 21 patients with psoriasis arthropathy

Psoriasis arthropathy (PsA) is a chronic inflammatory arthropathy characterized by the association of arthritis with psoriasis. In this study, clinical, laboratory and radiographic signs of 21 patients (12 males and 9 females), mean age of 42.2 years old, with PsA were assessed. The clinical forms defined by Moll and Wright revealed that oligoarticular pattern was most commonly observed in ten patients (47.6%), followed by polyarticular (5 patients), distal (3 patients), spondyloarthropathy (2 patients), and mutilans (1 patient). Positive rheumatoid factor was detected in three patients and antinuclear antibodies in eight patients, suggesting the involvement of immunological disregulation in this disorder. Twelve patients were onychopathic, of whom 11 showed distal interphalangeal (DIP) joint arthritis. Based on radiologic observation, spur formation of the calcaneus (1 patient) and destructive changes of the articulatio coxa (1 patient) were seen, in addition to the findings such as joint space narrowing, erosive changes, resorptive changes and 'pencil-in-cup' appearances. Non-steroidal anti-inflammatory drugs (NSAIDs) were used in all cases for the control of joint pain, solely or in combination with immunomodulatory drugs such as bucillamine, sulfasalazine, methotrexate, cyclosporin, etretinate, and mizoribine. However, some of those drugs were often ineffective for the joint pain, while effective for cutaneous psoriasis. Immunohistological studies of the biopsied synovial tissues from two patients showed increased expressions of CD45RO and HLA-DR, suggesting that the vast majority of inflammatory cells are mature and activated T-cells. Parallel immunostaining using the involved psoriatic skin from one of the patients also showed enhanced expression of CD45RO, but less expression of HLA-DR as compared with its expression in the synovium. On the other hand, cutaneous leukocyte antigen (CLA) was abundantly detected in the inflammatory cells in the lesional skin, although less expressed in the synovium. These results are consistent with earlier observations suggesting a different subpopulation of inflammatory cells in the skin than the joint.     

Management of autoimmune blistering diseases in pregnancy

Autoimmune blistering disease (AIBD) in pregnancy raises several complex management issues associated with underlying pathogenesis and treatment options. This article considers the effects of the disease as well as its treatment for both mother and fetus. All AIBDs can occur in pregnancy but are relatively rare. Pemphigoid gestationis is a rare AIBD that is specific to pregnancy. The article considers each AIBD in turn and then looks at treatment options for the group as a whole, as there are many issues common to all.