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https://www.readbyqxmd.com/read/29159879/new-insights-into-pemphigoid-diseases
#1
Frank Antonicelli, Ralf J Ludwig
Pemphigus and pemphigoid diseases are organ-specific autoimmune blistering diseases (AIBD), characterized and caused by autoantibodies to structural components of the skin (1). The autoantigens targeted in pemphigus are desmoglein 1 and 3, two proteins of the desmosomal structure, while the autoantigens in pemphigoid diseases (PD) are components of the basal membrane. For example, bullous pemphigoid (BP), the most frequent PD is characterized by autoantibodies against type XVII collagen (COL17, BP180) and BP230, and epidermolysis bullosa acquisita (EBA) is caused by autoantibodies against type VII collagen (COL7)...
November 21, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/29105148/biomarkers-related-to-bullous-pemphigoid-activity-and-outcome
#2
REVIEW
Delphine Giusti, Sébastien Le Jan, Gregory Gatouillat, Philippe Bernard, Bach Nga Pham, Frank Antonicelli
Bullous pemphigoid (BP) is the most common autoimmune sub-epidermal blistering disease of the skin. Investigation of the BP-associated pathophysiological processes during the last decades showed that the generation of auto-antibodies directed against the hemidesmosome proteins BP180 and BP230, a hallmark of the BP-associated autoimmune response, leads to the recruitment of inflammatory immune cells at the dermal-epidermal junction, and subsequently to the release of a large amount of inflammatory molecules involved in blister formation...
November 3, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/29054606/semaphorin-4d-from-cd15-granulocytes-via-adam10-induced-cleavage-contributes-to-antibody-production-in-bullous-pemphigoid
#3
Shengxian Shen, Yao Ke, Erle Dang, Hui Fang, Yuqian Chang, Jieyu Zhang, Zhenlai Zhu, Shuai Shao, Pei Qiao, Tongmei Zhang, Hongjiang Qiao, Gang Wang
Autoreactive B cells activation and antibody production are critical events for the development of bullous pemphigoid (BP). However, the mechanism that is involved in the modulation of B cell activation and autoantibody generation has not been fully understood. Semaphorin 4D (Sema4D/CD100) plays important roles in immune regulation related to B cell, but its implication in BP remains obscure. The aim of our study was to characterize Sema4D and the underlying mechanism contributing to the autoimmune features of BP...
October 17, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/29025244/a-comparative-study-of-expression-of-fc-receptors-in-relation-to-the-autoantibody-mediated-immune-response-and-neutrophil-elastase-expression-in-autoimmune-blistering-dermatoses
#4
Justyna Gornowicz-Porowska, Agnieszka Seraszek-Jaros, Monika Bowszyc-Dmochowska, Elżbieta Kaczmarek, Paweł Pietkiewicz, Paweł Bartkiewicz, Marian Dmochowski
Here we investigated the cutaneous CD32A and CD89 expression in relation to the neutrophil elastase (NE) expression and serum level of anti-desmoglein 1 and 3 (DSG1/DSG3) IgG in pemphigus, anti-BP180/BP230 IgG in bullous pemphigoid (BP), anti-gliadin nonapeptides (npG), tissue (tTG), and epidermal transglutaminases (eTG) IgA in dermatitis herpetiformis (DH). The examined material consisted of skin/mucosal tissues and sera. In total, 87 patients were studied. Immunohistochemistry on paraffin-embedded sections with quantitative digital morphometry was used to measure the intensity of CD32A/CD89/NE expressions...
2017: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
https://www.readbyqxmd.com/read/28948640/clinical-evaluation-of-a-multiparametric-elisa-as-a-rapid-tool-for-routinely-diagnosing-igg-mediated-autoimmune-blistering-dermatoses-in-ethnic-slavs
#5
Justyna Gornowicz-Porowska, Agnieszka Seraszek-Jaros, Monika Bowszyc-Dmochowska, Paweł Bartkiewicz, Elżbieta Kaczmarek, Marian Dmochowski
BACKGROUND: Technical innovation of autoimmune blistering dermatoses (ABDs) diagnosis aimed at multiplex approach. Two multiparametric ELISA tests are commercially available for ABDs serology. The aim was to compare diagnostic accuracy of multiparametric and monospecific ELISAs and to examine the diagnostic value/agreement of multivariant ELISA in compliance with traditional diagnostic setup for ABDs. METHODS: In total, 128 sera from suspected ABDs patients were studied (27 sera in order to compare ELISAs)...
September 26, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28887824/structural-proteins-of-the-dermal-epidermal-junction-targeted-by-autoantibodies-in-pemphigoid-diseases
#6
REVIEW
Stephanie Goletz, Detlef Zillikens, Enno Schmidt
The dermal-epidermal junction consists of a network of several interacting structural proteins which strengthen adhesion and mediate signaling events. This structural network consists of hemidesmosomal-anchoring filament complexes connecting the basal keratinocytes to the basement membrane. The anchoring filaments in turn interact with the anchoring fibrils to attach the basement membrane to the underlying dermis. Several of these structural proteins are recognized by autoantibodies in pemphigoid diseases, a heterogeneous group of clinically and immunopathologically diverse entities...
September 8, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28868796/ige-auto-reactivity-in-bullous-pemphigoid-eosinophils-and-mast-cells-as-major-targets-of-pathogenic-immune-reactants
#7
P C Freire, C H Muñoz, G Stingl
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune disease characterised by tense blisters that are usually preceded by urticarial eruptions. Affected patients exhibit IgG and/or IgE auto-antibodies against BP180 and/or BP230. Their relative importance in disease pathogenesis has not been fully elucidated. OBJECTIVES: The aim of this study was to better characterise circulating and tissue-resident IgE in BP patients at the serological, structural and functional level...
September 4, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28833620/eosinophils-as-putative-therapeutic-targets-in-bullous-pemphigoid
#8
REVIEW
Dagmar Simon, Luca Borradori, Hans-Uwe Simon
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering skin disease and is characterized by the presence of autoantibodies directed against the hemidesmosomal proteins BP180 and BP230 that can be detected in the skin and serum of BP patients. Histologically, the dermal infiltration of eosinophils is obvious. The objective of this review was to present evidence that eosinophils play a key role in the pathogenesis of BP. Eosinophils, together with cytokines and chemokines regulating their production, recruitment and activation, are abundantly present in lesional skin, in blisters and in peripheral blood of patients with BP...
August 20, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28736206/bpag1-in-muscles-structure-and-function-in-skeletal-cardiac-and-smooth-muscle
#9
REVIEW
Masao Horie, Nozomu Yoshioka, Hirohide Takebayashi
BPAG1, also known as Dystonin or BP230, belongs to the plakin family of proteins, which has multiple cytoskeleton-binding domains. Several BPAG1 isoforms are produced by a single BPAG1 genomic locus using different promoters and exons. For example, BPAG1a, BPAG1b, and BPAG1e are predominantly expressed in the nervous system, muscle, and skin, respectively. Among BPAG1 isoforms, BPAG1e is well studied because it was first identified as an autoantigen in patients with bullous pemphigoid, an autoimmune skin disease...
July 20, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28680335/analysis-of-the-autoimmune-response-against-bp180-and-bp230-in-ethnic-poles-with-neurodegenerative-disorders-and-bullous-pemphigoid
#10
Justyna Gornowicz-Porowska, Agnieszka Seraszek-Jaros, Monika Bowszyc-Dmochowska, Elżbieta Kaczmarek, Paweł Pietkiewicz, Paweł Bartkiewicz, Marian Dmochowski
Recent studies postulated the association between bullous pemphigoid (BP) and neurodegenerative disorders (ND). The autoantibodies to BP180 and/or BP230 may be present not only in BP, but also in ND as neuronal isoforms of these proteins are identified in the central nervous system. However, there are only scant data about the precise pathogenetic mechanisms interlinking ND and BP as well as the immunologic profile in these patients. The aim is to analyze the serological immunopathological profiles (anti-BP180 IgG, anti-BP230 IgG) in BP patients with and without ND in order to identify the specific autoantibody(ies) and corresponding antigens responsible for ND development in BP patients...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28677172/neurological-and-psychiatric-associations-in-bullous-pemphigoid-more-than-skin-deep
#11
REVIEW
Anna-Kaisa Försti, Laura Huilaja, Enno Schmidt, Kaisa Tasanen
In elderly patients, bullous pemphigoid (BP) is associated with several comorbidities; the strongest association occurs between BP and neurological diseases. Different types of dementia, Parkinson's disease, cerebrovascular disorders and epilepsy all have a significant association with BP, but patients with multiple sclerosis have the highest risk of BP. An existing neurological disorder appears to increase the risk for subsequent BP, but an increased risk for developing some neurological diseases has also been reported following BP diagnosis...
July 4, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28623863/linear-immunoglobulin-a-g-bullous-dermatosis-associated-with-ulcerative-colitis
#12
Asuka Onoe, Daisuke Matsuura, Tadashi Terui, Norito Ishii, Takashi Hashimoto, Toyoko Ochiai
Linear immunoglobulin (Ig)A/G bullous dermatosis (LAGBD) is an autoimmune bullous disease characterized by formation of subepidermal blisters and linear deposition of IgA and IgG antibodies along the basement membrane zone (BMZ). The association between linear IgA bullous dermatosis and ulcerative colitis (UC) is well recognized, but reports of UC-associated LAGBD are lacking. We have reported a 24-year-old man suffering from LAGBD associated with UC, which occurred before exacerbations of skin rash. A skin biopsy indicated a subepidermal blister with an infiltration of primarily neutrophils and eosinophils in the dermis...
June 17, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28605575/bullous-pemphigoid-and-chronic-kidney-graft-rejection
#13
LETTER
F Rosique López, A Martínez Losa, P Sánchez-Pedreño Guillén, N Ishii, T Hashimoto, P Martínez García
No abstract text is available yet for this article.
June 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28261030/neurodegenerative-disorders-bullous-pemphigoid-and-psoriasis-a-comparative-study-in-ethnic-poles-indicates-that-parkinson-s-disease-is-more-relevant-to-bullous-pemphigoid
#14
Paweł Bartkiewicz, Justyna Gornowicz-Porowska, Paweł P Pietkiewicz, Anna Świrkowicz, Monika Bowszyc-Dmochowska, Marian Dmochowski
INTRODUCTION: Bullous pemphigoid (BP) is an autoimmune blistering dermatosis of the elderly with autoimmunity to hemidesmosomal proteins, BP180 and BP230, which are expressed also in neuronal tissue. AIM: The aim here was to retrospectively compare the prevalence of neurodegenerative disorders (ND), particularly Parkinson's disease (PD), unspecified conditions manifesting as dementia and stroke, in two groups of ethnic Poles, with BP and with psoriasis (Ps), in order to obtain data whether BP is more prone to coexist with ND than Ps in the elderly...
February 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28261028/accuracy-of-molecular-diagnostics-in-pemphigus-and-bullous-pemphigoid-comparison-of-commercial-and-modified-mosaic-indirect-immunofluorescence-tests-as-well-as-enzyme-linked-immunosorbent-assays
#15
Justyna Gornowicz-Porowska, Agnieszka Seraszek-Jaros, Monika Bowszyc-Dmochowska, Elżbieta Kaczmarek, Paweł Pietkiewicz, Paweł Bartkiewicz, Marian Dmochowski
INTRODUCTION: Pemphigus and bullous pemphigoid (BP) are identified by autoantibodies (abs) against desmoglein 1, 3 (DSG1/3) and BP180/BP230, respectively. A novel mosaic to indirect immunofluorescence (IIF) using purified BP180 recombinant proteins spotted on slide and transfected cells expressing BP230, DSG1, DSG3 is available. The commercial (IgG detection) and modified (IgG4 detection) mosaic for indirect immunofluorescence (IIFc - IIF commercial, IIFm - IIF modified) and IgG ELISAs were evaluated in pemphigus and bullous pemphigoid (BP) molecular diagnostics...
February 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28247089/bullous-pemphigoid-a-review-of-its-diagnosis-associations-and-treatment
#16
REVIEW
Philippe Bernard, Frank Antonicelli
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries, and typically affects the elderly. BP is immunologically characterized by tissue-bound and circulating autoantibodies directed against either the BP antigen 180 (BP180, or BPAG2) or the BP antigen 230 (BP230, or BPAG1e), or even both, which are components of hemidesmosomes involved in the dermal-epidermal cohesion. Risk factors for BP include old age, neurologic diseases (dementia, Parkinson's disease, cerebrovascular disease), and some particular drugs, including loop diuretics, spironolactone and neuroleptics...
August 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28135772/evidence-for-a-role-of-eosinophils-in-blister-formation-in-bullous-pemphigoid
#17
E de Graauw, C Sitaru, M Horn, L Borradori, S Yousefi, H-U Simon, D Simon
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune bullous disease of the skin characterized by subepidermal blister formation due to tissue-bound and circulating autoantibodies to the hemidesmosomal antigens BP180 and BP230. Although eosinophils and their toxic mediators are found abundantly in BP lesions, their role in blister formation has remained unclear. OBJECTIVE: To investigate the role of eosinophils in the pathogenesis of BP with a specific focus on blister formation and to define conditions inducing dermal-epidermal separation (DES)...
July 2017: Allergy
https://www.readbyqxmd.com/read/28101965/a-multi-hit-hypothesis-of-bullous-pemphigoid-and-associated-neurological-disease-is-hla-dqb1-03-01-a-potential-link-between-immune-privileged-antigen-exposure-and-epitope-spreading
#18
REVIEW
K T Amber, J Zikry, M Hertl
Bullous pemphigoid (BP) is the most common autoimmune blistering disease and is linked to IgG recognition of 2 hemidesmosomal antigens, that is, BP230 (BP antigen 1) and BP180 (BP antigen 2, collagen XVII). The association of BP with other systemic diseases, particularly neurocognitive diseases, provides a potential clue in the underlying pathogenesis of BP. The role of HLA-DQB1*03:01 binding to the immunogenic portion of BP180 provides a potential mechanism by which exposure to neuronal collagen BP180 may lead to cutaneous disease...
March 2017: HLA
https://www.readbyqxmd.com/read/28038887/prospective-studies-on-the-routine-use-of-a-novel-multivariant-enzyme-linked-immunosorbent-assay-for-the-diagnosis-of-autoimmune-bullous-diseases
#19
MULTICENTER STUDY
Nina van Beek, Cornelia Dähnrich, Nora Johannsen, Susanne Lemcke, Stephanie Goletz, Franziska Hübner, Giovanni Di Zenzo, Marian Dmochowski, Kossara Drenovska, Shamir Geller, Michael Horn, Cezary Kowalewski, Ljiljana Medenica, Dedee F Murrell, Aikaterini Patsatsi, Soner Uzun, Snejina Vassileva, Detlef Zillikens, Wolfgang Schlumberger, Enno Schmidt
BACKGROUND: Serologic diagnosis of autoimmune blistering disease (AIBD) usually follows a sophisticated multistep algorithm. OBJECTIVE: We sought validation of a multivariant enzyme-linked immunosorbent assay (ELISA) in the routine diagnosis of AIBD. METHODS: The multivariant ELISA comprising 6 recombinant immunodominant forms of major AIBD target antigens, ie, desmoglein 1, desmoglein 3, envoplakin, BP180, BP230, and type VII collagen was applied in: (1) a cohort of well-characterized AIBD (n = 173) and control sera (n = 130), (2) a prospective multicenter study with 204 sera from patients with newly diagnosed AIBD with positive direct immunofluorescence microscopy, and (3) a prospective monocenter study with 292 consecutive sera from patients with clinical suspicion of AIBD in comparison with the conventional multistep diagnostic algorithm...
May 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/27716903/detection-of-ige-autoantibodies-to-bp180-and-bp230-and-their-relationship-to-clinical-features-in-bullous-pemphigoid
#20
T Hashimoto, A Ohzono, K Teye, S Numata, S Hiroyasu, D Tsuruta, T Hachiya, K Kuroda, M Hashiguchi, T Kawakami, N Ishii
BACKGROUND: IgE autoantibodies are considered to be involved in the pathogenesis of bullous pemphigoid (BP), particularly inflammatory and erythematous phenotypes. OBJECTIVES: To develop reliable enzyme-linked immunosorbent assays (ELISAs) for the detection of IgE autoantibodies to both BP180 and BP230 in BP sera, and to compare the ELISA results with clinical features. METHODS: We used commercially available IgG ELISAs to develop IgE ELISAs for both BP180 and BP230...
July 2017: British Journal of Dermatology
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