大疱性类天疱疮抗原1和2在大疱性类天疱疮并发神经系统损害中的作用

大疱性类天疱疮可见于多种神经系统疾病患者,二者并发的机制尚不清楚.动物研究证实鼠大疱性类天疱疮抗原(BPAG)1至少存在3种亚型:皮肤亚型、神经业型和肌肉亚型.BPAG2除在皮肤有表达外,在人脑神经元也有广泛表达.有可能神经系统病变导致BPAG1和BPAG2神经亚型暴露于免疫系统,产生拮抗神经亚型的抗体,与皮肤亚型发生免疫反应及交叉反应,导致大疱性类天疱疮发生.     

大疱性类天疱疮合并神经系统损害研究进展

大疱性类天疱疮可发生于多种神经系统疾病患者，包括痴呆、脑血管病、癫痫、多发性硬化、帕金森病、周围神经病等。二者合并发生的机制尚不清楚。BPAgl至少存在3种亚型：皮肤亚型、神经亚型和肌肉亚型。各亚型之间有某些相似表位。有可能神经系统病变导致神经亚型暴露于免疫系统，拮抗神经亚型的抗体，通过免疫交叉反应与皮肤亚型发生反应，导致大疱性类天疱疮出现。     

大疱性类天疱疮1例

患者女,54岁。颜面,躯干及四肢紫红色斑丘疹伴瘙痒半年余,双下肢水疱半个月,于2006年1月收入院治疗。半年多前无明显诱因患者颜面部出现散在黄豆至钱币大圆形、[第一段]     

大疱性类天疱疮1例

患者男,64岁.双下肢出现丘疹伴瘙痒3个月,全身起水疱1个月.患者3个月前无明显诱因双下肢出现红色丘疹,渐增多并融合成片,瘙痒剧烈,曾就诊于多家医院,多按"湿疹"给予治疗,效果欠佳,皮损渐加重.     

大疱性类天疱疮1例

大疱性类天疱疮是一种获得性自身免疫性大疱性皮肤病,好发于老年人,多表现为累及身体屈侧的张力性水疱、大疱,伴不同程度的瘙痒.发病的早期和恢复期,大疱性类天疱疮的皮损常可呈多形性,临床易误诊.现报告1例大疱性类天疱疮误诊为多形性日光疹.     

大疱性类天疱疮抗原的研究进展

大疱性天类天疱疹的两个自身抗原存在着不同的分子生物学特性，大疱性类天疱疮抗原２２０～２４０ｋＤ是人表皮基底细胞内半桥粒斑的成分，含２６０６氨基酸，空间结构可能是双螺旋。大疱性类天疱疮抗原１８０ｋＤ为跨膜蛋白，由１５７２氨基酸组成，其中有很多胶原域，推测为一种新的胶原蛋白成分，在大疱性类天疱疮的发病机制中，大疱性类天疱疮抗原１８０ｋＤ可能具有更重要的意义。     

大疱性类天疱疮抗原分子的研究进展

大疱性类天疱疮存在BPAg1和BPAg2两个抗原分子,前者分子量约220～240kD,后者分子量约160～180kD。目前用分子生物学的方法已经克隆出BPAg1和BPAg2的cDNA,发现二者的碱基序列和长度截然不同。编码BPAg1的基因位于染色体6pter-q15位点,编码BPAg2的基因位于染色体10q24.3位点。BPAg的分子生物学研究进展有利于进一步探讨BP的发病机制。     

大疱性类天疱疮常合并的疾病

大疱性类天疱疮(BP)是皮肤科最常见的大疱性疾病之一,是一种自身免疫性疾病,越来越多的文献报道,BP可以合并多种疾病,如神经系统疾病,恶性肿瘤、其他皮肤病、移植排斥反应、溃疡性结肠炎等,本文综述了国内外报道的BP常合并的疾病.     

大疱性类天疱疮230000和160000抗原的区域性分布

利用免疫印迹技术筛选出2份大疱性类天疱疮(BP)血清,一份只和230 000分子结合,一份只和160 000分子结合,分别和人体22处正常皮肤作间接免疫荧光(ⅡF),发现230 000抗原和160 000抗原的表达存在明显的区域性差异.230 000抗原在胸腹和大腿屈侧含量最高,头皮和足跖最低.160 000抗原在胸腹含量最高,头皮和颈部含量最低.230 000抗原和160 000抗原在同一部位的表达基本一致,但在腋窝、大腿伸侧和大腿屈侧三个部位明显高于后者.上述差异与临床上BP的好发部位有一定关联,靶抗原表达的区域性差异是造成自身免疫性皮肤病皮疹特异分布形式的一个重要原因.     

656例自身免疫性大疱病住院患者皮肤感染的危险因素及病原学分析

目的: 总结自身免疫性大疱病住院患者皮肤感染的相关因素、病原体以及药物敏感特征.方法: 收集2013-2019年自身免疫性大疱病住院患者的临床资料,并对其感染相关因素以及皮肤感染病原体、药物敏感试验进行回顾性分析.结果: 共656例自身免疫性大疱病住院患者纳入分析,皮肤感染率为74.29％,住院时长≥14天与<14天患...     

大疱性类天疱疮白细胞介素8的测定

目的:探讨IL-8在大疱性类天疱疮发病机制的作用及临床意义.方法:采用ELISA方法检测大疱性类天疱疮患者活动期血清、疱液和缓解期血清中IL-8的水平.结果:大疱性类天疱疮患者活动期血清中IL-8水平明显高于健康对照者血清中的水平,病情缓解后血清中IL-8的水平明显下降,活动期疱液IL-8水平明显高于活动期血清中的水平.结论:IL-8可能参与了大疱性类天疱疮的发病,可作为观察病情活动的指标.     

盐裂间接免疫荧光技术在大疱性类天疱疮鉴别诊断中的应用研究

目的:探讨盐裂皮肤间接免疫荧光(IIF)技术在大疱性类天疱疮(BP)鉴别诊断中的作用.方法:应用盐裂IIF技术检测78例常规方法诊断为BP的患者血清.结果:43例血清IgG沉积于表皮侧,7例IgG沉积于双侧,11例IgG沉积于真皮侧,另有17例双侧均未见抗体沉积.结论:盐裂IIF仅能用于BP的初步鉴别诊断.     

Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study

Background Bullous pemphigoid (BP), pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune bullous diseases characterized by the presence of tissue‐bound and circulating autoantibodies directed against disease‐specific target antigens of the skin. Although rare, these diseases run a chronic course and are associated with significant morbidity and mortality. There are few prospective data on gender‐ and age‐specific incidence of these disorders. Objectives Our aims were: (i) to evaluate the incidence of BP and PV/PF in Swiss patients, as the primary endpoint; and (ii) to assess the profile of the patients, particularly for comorbidities and medications, as the secondary endpoint. Methods The protocol of the study was distributed to all dermatology clinics, immunopathology laboratories and practising dermatologists in Switzerland. All newly diagnosed cases of BP and pemphigus occurring between 1 January 2001 and 31 December 2002 were collected. In total, 168 patients (73 men and 95 women) with these autoimmune bullous diseases, with a diagnosis based on clinical, histological and immunopathological criteria, were finally included. Results BP showed a mean incidence of 12·1 new cases per million people per year. Its incidence increased significantly after the age of 70 years, with a maximal value after the age of 90 years. The female/male ratio was 1·3. The age‐standardized incidence of BP using the European population as reference was, however, lower, with 6·8 new cases per million people per year, reflecting the ageing of the Swiss population. In contrast, both PV and PF were less frequent. Their combined mean incidence was 0·6 new cases per million people per year. Conclusions This is the first comprehensive prospective study analysing the incidence of autoimmune bullous diseases in an entire country. Our patient cohort is large enough to establish BP as the most frequent autoimmune bullous disease. Its incidence rate appears higher compared with other previous studies, most likely because of the demographic characteristics of the Swiss population. Nevertheless, based on its potentially misleading presentations, it is possible that the real incidence rate of BP is still underestimated. Based on its significant incidence in the elderly population, BP should deserve more public health concern.     

IgA autoantibodies in the pemphigoids and linear IgA bullous dermatosis

Please cite this paper as: IgA autoantibodies in the pemphigoids and linear IgA bullous dermatosis. Experimental Dermatology 2010; 19: 648–653. Background: Patients with bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and pemphigoid gestationis (PG) have IgG antibodies against BP180 and BP230, components of the hemidesmosomes. Patients with linear IgA bullous dermatosis (LABD) have IgA autoantibodies against a 97/120‐kDa protein which is highly homologous to a shedded fragment of the BP180‐ectodomain. Objectives: The aim of our study was to determine the incidence of IgA autoantibodies directed against BP180/BP230 in the pemphigoids and LABD and to determine the antigenic regions that are targeted by IgA autoantibodies. Methods: Utilizing baculovirus‐expressed recombinant BP180 and BP230 proteins, we performed immunoblot analyses for IgA reactivity of sera from patients with BP (n = 30), MMP (n = 10), PG (n = 6), LABD (n = 6) and from control patients with non‐related pruritic dermatoses (n = 8). Results: IgA reactivity against BP180 and/or BP230 was detected in 19/30 of the BP, in 7/10 of the MMP, in 6/6 of the LABD and in 3/6 of the PG sera, respectively, but not in the control group. In all subgroups, the major antigenic site recognized by IgA antibodies was located within the NH₂‐terminus of the BP180‐ectodomain, but only a minority of the sera showed also IgA reactivity against the BP180‐NC16a‐domain. IgA reactivity against the central domain of BP180 was more frequently seen than against its COOH‐terminus. IgA against the COOH‐ and NH₂‐terminus of BP230, respectively, was detected in 6/30 of the BP, 1/10 of the MMP, 1/6 of the LABD and 0/8 control sera. Conclusion: IgA reactivity against BP180 and/or BP230 is a common finding in the pemphigoids.     

Case of localized bullous pemphigoid with unique clinical manifestations in the lower legs

We report the case of a 71-year-old Japanese woman who presented with persistent band-like erosions in the lower legs. Histological examination suggested subepidermal blister formation. Direct and indirect immunofluorescence studies revealed tissue-deposited and circulating immunoglobulin G autoantibodies against the basement membrane. Western blotting revealed autoantibodies to BP230, but not to BP180. Based on these findings, the patient was diagnosed as having a localized type of bullous pemphigoid. We suggest that the unique clinical manifestations in this patient could be attributable to bacterial or fungal infection, and/or mechanical trauma, such as the pressure of her socks.     

High-dose intravenous immune globulin for the treatment of autoimmune blistering diseases: an evaluation of its use in 14 cases

High-dose intravenous immune globulin (IVIG) is used to treat a wide variety of autoimmune diseases. We report our experiences of its use in a retrospective study of 14 patients with autoimmune blistering diseases, namely epidermolysis bullosa acquisita (EBA), two; bullous pemphigoid (BP), two; pemphigoid gestationis (PG), one; nodular pemphigoid, two; and pemphigus vulgaris (PV), seven. Two patients with refractory EBA improved following regular courses of IVIG given as monotherapy. IVIG had a steroid-sparing effect in 10 patients with PV, BP and PG. However, the clinical effects were transient and of variable intervals, and repeated courses of IVIG were required. The rapid actions of IVIG were of particular benefit in two patients with extensive, rapidly progressive PV and in one patient with BP in whom swift disease control was required. In such cases, when rapid disease control is paramount, we recommend IVIG used in conjunction with conventional treatments as a safer and less invasive alternative to plasmapheresis. IVIG was ineffective in two patients with nodular pemphigoid. Analysis of indirect immunofluorescence (IIF) titres before and after IVIG showed that a fall in titre occurred after 78% of treatments and was observed in all disease groups. However, like the clinical improvements, the falls in IIF titres were transient and of variable interval, and titres rose back to pretreatment levels in all but one patient. IVIG appears to be beneficial under certain circumstances for the treatment of autoimmune blistering diseases but controlled trials are required to define its therapeutic role further.     

1mol／L NaCl分离表皮DIF染色法的临床实用价值

对１９例大疱性类天疱疮（ＢＰ）和５例获得性大疱性表皮松解症（ＥＢＡ）病人进行了常规ＤＩＦ、１ｍｏｌ／Ｌ ＮａＣｌ分离表皮ＤＩＦ和１ｍｏｌ／Ｌ ＮａＣｌ分离正常人皮肤ⅡＦ的对比研究。结果显示１ｍｏｌ／Ｌ ＮａＣｌ分离皮肤ＤＩＦ染色法是诊断和鉴别诊断ＢＰ和ＥＢＡ的一种简单、可靠、敏感的方法。