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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
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
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...
February 28, 2017: American Journal of Clinical Dermatology
Elisabeth de Graauw, Cassian Sitaru, Michael Horn, Luca Borradori, Shida Yousefi, Hans-Uwe Simon, Dagmar 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)...
January 30, 2017: Allergy
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...
January 19, 2017: HLA
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
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 pathogenesis of bullous pemphigoid (BP), particularly inflammatory and erythematous phenotypes. OBJECTIVE: To develop reliable ELISAs for 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. To determine the influence of excess amount of IgG autoantibodies, all normal and BP sera were tested before and after IgG adsorption...
October 7, 2016: British Journal of Dermatology
Kahori Nasu, Norio Hanafusa, Masaomi Nangaku
Bullous pemphigoid (BP) is an autoimmune blistering skin disorder characterized by circulating serum IgG antibodies against two hemidesmosomal proteins: BP180 and BP230. Fundamentally, immunosuppressive therapies are administered to treat this disease, but plasmapheresis can be added for refractory patients. We experienced the case of a 63-year-old patient with refractory BP for which we administered double filtration plasmapheresis (DFPP). His skin lesions improved along with decreased IgG BP180 antibodies, but factor XIII (FXIII) and fibrinogen were also reduced by DFPP repetition...
October 6, 2016: Journal of Clinical Apheresis
Susanna Esposito, Sophie Guez, Annalisa Orenti, Gianluca Tadini, Giulietta Scuvera, Laura Corti, Alessia Scala, Elia Biganzoli, Emilio Berti, Nicola Principi
In order to evaluate the serum anti-skin autoantibodies and cytokine concentrations in patients with different epidermolysis bullosa (EB) types and severity, 42 EB patients and 38 controls were enrolled. Serum anti-skin antibodies were significantly higher in the patients than in the controls (p = 0.008, p < 0.001, p < 0.001, p < 0.001 and p < 0.001 for desmoglein 1 (DSG1) desmoglein 3 (DSG3), bullous pemphigoid 180 (BP180), BP230 and type VII collagen (COL7), respectively). The same trend was observed for interleukin (IL)-1β, IL-2, IL-6, IL-10, tumor necrosis factor-β, and interferon-γ (p < 0...
September 24, 2016: International Journal of Molecular Sciences
Nina Kokkonen, Sanna-Kaisa Herukka, Laura Huilaja, Merja Kokki, Anne M Koivisto, Päivi Hartikainen, Anne M Remes, Kaisa Tasanen
Bullous pemphigoid (BP) is a subepidermal blistering skin disease, which has shown a strong association with neurological diseases in epidemiological studies. The BP autoantigens BP180 and BP230 are expressed in the cutaneous basement membrane and the central nervous system. Using BP180 and BP230 ELISA assays and immunoblotting against BP180, we analyzed the IgG reactivity in the sera of 115 patients with Alzheimer's disease (AD) and 40 neurologically healthy controls. BP180 autoantibodies were found in 18% of patients with AD, whereas only 3% of controls had positive results (P = 0...
January 2017: Journal of Investigative Dermatology
Roland Schneiderbauer, Stefanie Martinache, Monika Engstner, Alexander H Enk, Eva N Hadaschik
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)...
November 2016: Dermatologic Therapy
N Yoshimoto, H Ujiie, Y Hirata, K Izumi, W Nishie, H Shimizu
No abstract text is available yet for this article.
April 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
G Balakirski, A Alkhateeb, H F Merk, M Leverkus, M Megahed
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering disease that is characterized by formation of subepidermal bullae due to functional disturbance of the hemidesmosomal proteins on the keratinocytes at the basal membrane zone. In recent years, several studies have emphasized the important role of IgE autoantibodies in the pathogenesis of BP. Consequently, a therapeutic approach using IgE depleting antibodies, such as a humanized monoclonal anti-IgE antibody (e.g. omalizumab) may represent a new option for treatment of this autoimmune disease...
October 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Björn Dangel, Lukas Kofler, Gisela Metzler
INTRODUCTION: Itching nodules and papules are common findings. A rare but important differential diagnosis is the nodular subtype of bullous pemphigoid. METHODS AND RESULTS: The investigators report a female patient presenting with strongly itching papules disseminated over her extremities and trunk. Physical examination revealed multiple erythematous, mostly excoriated papules and nodules on her back, abdomen, and extremities. Histology showed changes compatible with prurigo lesion, and immunofluorescence showed positive results for BP180 and BP230...
November 2016: Journal of Cutaneous Medicine and Surgery
Christian D Sadik, Hendri H Pas, Michael K Bohlmann, Sadegh Mousavi, Sandrine Benoit, Miklós Sárdy, Jorrit B Terra, Ana L Lima, Christoph M Hammers, Nina van Beek, Christine Bangert, Detlef Zillikens, Enno Schmidt
is missing (Short communication).
January 4, 2017: Acta Dermato-venereologica
Taihei Hayakawa, Kwesi Teye, Takahisa Hachiya, Rie Uehara, Masahiro Hashiguchi, Tamihiro Kawakami, Xiaoguang Li, Atsunari Tsuchisaka, Koji Ohara, Ryosuke Sogame, Hiroshi Koga, Takahiro Hamada, Chika Ohata, Minao Furumura, Norito Ishii, Hideo Fukano, Kazuo Shimozato, Takashi Hashimoto
OBJECTIVES: To confirm that sera from some BP patients reactive exclusively to the BP230 and to study the clinical and immunological characteristics of this condition. MATERIALS AND METHODS: BP patients were divided into three groups: BP reactive only to BP230 (BP230-BP), BP reactive to both BP180 and BP230 (BP180-BP230-BP) and BP reactive only to BP180 (BP180-BP), based on the results of standard ELISAs for BP180 and BP230. Clinical features were statistically analyzed among the three groups...
April 1, 2016: European Journal of Dermatology: EJD
Cathy Y Zhao, Dedee F Murrell
Bullous pemphigoid (BP) is the commonest subtype of autoimmune blistering disease in most countries of the world. It occurs most frequently in elderly patients and is characterised clinically by large, tense blisters in the skin preceded by urticarial plaques and pruritus. Immunopathologically, it is characterised by autoantibodies directed against the 180 kD antigen (BP180) and the 230 kD antigen (BP230). New knowledge regarding BP is being continually uncovered. This article reviews the recent advances in BP, including newer diagnostic tests, standardised outcome measures and emerging therapeutic options, as well as the evidence supporting their use...
2015: F1000Research
Jesse J Keller, Ashley L Kittridge, Sara M Debanne, Neil J Korman
Bullous pemphigoid (BP) is a common autoimmune blistering disorder of the elderly. Several diagnostic modalities are available, including clinical impression, histopathology, direct and indirect immunofluorescence, and enzyme-linked immunosorbent assay (ELISA) detection of pathogenic antibodies. In this study, we aim to examine the utility of the newest test, ELISA, in comparison to the constellation of other tests. We describe our clinical experience in which 170 patients diagnosed with bullous pemphigoid had multiple tests performed...
May 2016: Archives of Dermatological Research
Francisco Manuel Ildefonso Mendonça, Francisco José Martín-Gutierrez, Juan José Ríos-Martín, Francisco Camacho-Martinez
BACKGROUND: Bullous pemphigoid (BP) is an acquired subepidermal autoimmune blistering disease in which there are humoral and cellular responses against the BP180 and BP230 antigens. Dipeptidyl peptidase (DPP)-4 inhibitors enhance endogenous glucagon peptide-1 and glucose-dependent insulinotropic polypeptide secretion with food intake, which leads to insulin secretion, as well as to the reduction of glucagon secretion. Recently, several cases of DPP-4 inhibitor-associated BP have been reported...
2016: Dermatology: International Journal for Clinical and Investigative Dermatology
Orsolya N Horváth, Rita Varga, Makoto Kaneda, Enno Schmidt, Thomas Ruzicka, Miklós Sárdy
BACKGROUND: The "MESACUP anti-Skin profile TEST" is a new, commercially available ELISA kit to detect circulating IgG autoantibodies against desmoglein 1, desmoglein 3, BP180, BP230, and type VII collagen, both simultaneously and more rapidly than previous assays. OBJECTIVES: The aim of this study was to evaluate the diagnostic accuracy of this kit for the diagnosis of pemphigus foliaceus, pemphigus vulgaris, bullous pemphigoid and epidermolysis bullosa acquisita...
January 2016: European Journal of Dermatology: EJD
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