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1.
We report a case of localized pretibial pemphigoid with concurrent trichilemmal carcinoma of the face in a 93-year-old woman. Immunoblot analysis showed circulating autoantibodies against the 230 kDa antigen. After removal of the skin tumor, the bullous skin lesions were reduced and healed with only the topical application of corticosteroid.  相似文献   

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Topical steroids are effective in treating bullous pemphigoid (BP). Autoantibodies against BP180 are related to disease activity, but correlation of these autoantibodies with response to topical steroid therapy has not yet been clearly evaluated. We investigate the usefulness of close and early monitoring of autoantibodies against BP180 and BP230 for assessment of response to therapy and early detection of therapeutic failure in BP patients treated topically. In eight BP patients under treatment with topical or systemic steroid therapy we retrospectively evaluated clinical course and autoantibodies against BP180 and BP230 as well as indirect immunofluorescence titres (IIF). Data were included at diagnosis, during hospitalization and follow‐ups. Autoantibodies against BP180 parallel disease activity in all topically and as well as systemically treated patients. Autoantibodies against BP230 correlated in five out of eight patients. Autoantibodies directed against BP180 and, to a lesser degree, against BP230 correlate with the clinical course of topically treated BP patients. Monitoring autoantibodies against BP180 is a useful tool to evaluate the efficacy of topical therapy in BP.  相似文献   

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Bullous pemphigoid (BP) is an autoimmune disease associated with subepidermal blistering due to autoantibodies directed against BP180 and BP230. BP180 is currently considered as the major pathogenic autoantigen. However, previous clinical findings suggested that anti-BP230 autoantibodies alone can cause skin lesions in animal models and many BP patients. The characteristics of BP230 and the pathogenic roles of anti-BP230 antibodies have been proposed. First, at the molecular level, BP230 mediates the attachment of keratin intermediate filaments to the hemidesmosomal plaque and interacts with other constituents of hemidesmosomes. Second, the presence of BP230 autoantibodies may correlate with specific clinical features of BP. The immunoglobulin (Ig)G autoantibodies from BP patients react mainly against the C-terminus of BP230, while the IgE autoantibodies are still inconclusive. Third, in vivo, autoantibodies against BP230 involved in the disease may not only induce the inflammatory response but also impair the structural stability of hemidesmosomes. This article reviews recently published work about the role of BP230 and its antibodies, including IgG and IgE, aiming to find clues of its clinical association and lay the foundation for the research on the pathogenicity of antibodies against BP230.  相似文献   

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目的:检测神经系统疾病患者血清中抗BP180、抗BP230和抗基底膜带抗体的阳性率。方法:收集神经系统疾病患者和正常对照血清,采用酶联免疫吸附试验(ELISA)和间接免疫荧光(IIF)检测两组血清中抗BPl80NC16A抗体和BP230抗体水平。结果:共收集到131例神经系统疾病患者血清(脑卒中109例,脑肿瘤17例,其他神经系统疾病19例)和131例正常对照血清。病例组中抗BP180NC16A1阳性率为1.45%,低于对照组的3.05%,差异具有统计学差异(P = 0.009),病例组中抗BP230抗体阳性率5.34%与对照组(2.29%)比较,差异无统计学意义。IIF检测抗基底膜带抗体结果均为阴性。结论:抗BP180NC16A抗体在神经系统疾病患者中有较高的阳性率,可能与神经系统疾病患者合并大疱性类天疱疮相关。  相似文献   

5.
Pemphigoid nodularis is a rare clinical variant of pemphigoid characterized by overlapping clinical features of both prurigo nodularis lesions and pemphigoid-like blisters. In pemphigoid nodularis, the immunopathological findings are identical to those of bullous pemphigoid (BP). We describe five patients who presented with the typical clinical phenotype of prurigo nodularis, who were found to have circulating and tissue-bound antibasement membrane zone autoantibodies. By immunoelectron microscopy and Western immunoblotting studies, the circulating antibodies were shown to target the hemidesmosome and specifically the BP antigens 1 and 2 (BP180 and BP230). In contrast to the majority of reported cases, none of these patients has ever developed blisters. The role of antibasement membrane zone antibodies in the development of the eruption, or the role of the eruption in the development and persistence of autoantibodies, is not clear. These cases demonstrate that the presence of these antibodies is not sufficient for the development of blisters.  相似文献   

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BACKGROUND: Bullous pemphigoid (BP) is a blistering disease associated with autoantibodies directed against two components of hemidesmosomes, BP180 and BP230. OBJECTIVES: To assess whether BP patients have autoantibodies targeting plectin, another hemidesmosomal component showing extensive homology to BP230. METHODS: Examination of sera from 16 patients with BP, using immunoprecipitation studies followed by immunoblotting. RESULTS: Serum of one of the 16 (6%) patients with BP contain autoantibodies binding to plectin, while no reactivity was found with sera from three control subjects. Sera from all 16 BP patients immunoprecipitated BP230 from extracts of biosynthetically radiolabelled human keratinocytes. CONCLUSIONS: Our results indicate that sera from BP patients might contain autoantibodies binding to plectin. Although this protein and BP230 are closely sequence-related, the occurrence of autoantibodies binding to plectin is a rare phenomenon in BP.  相似文献   

10.
结节性类天疱疮(PN)是一种罕见的大疱性类天疱疮(BP)亚型,其特征是结节性痒疹和水疱以及与类天疱疮相同的免疫学结果。本文对该疾病的流行病学、病因及发病机制、临床表现、诊断及治疗作一综述。  相似文献   

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