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Pemphigus

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https://www.readbyqxmd.com/read/28093721/application-of-autologous-hematopoietic-stem-cell-transplantation-for-pemphigus
#1
Menglei Wang, Can Cao, Jing Sun, Xuebiao Peng, Qifa Liu, Liang Huang, Yanyan Chai, Kuan Lai, Pingjiao Chen, Qingxiu Liu, Qian Li, Yusheng Peng, Hao Xiong, Jing Zhang, Minghua Chen, Kang Zeng
BACKGROUND: Pemphigus is a rare and fatal autoimmune disease for which the treatment options are limited. This study aimed to evaluate the efficacy of autologous peripheral hematopoietic stem cell transplantation (APHSCT) for pemphigus. METHODS: We conducted APHSCT for 12 pemphigus patients (seven males and five females, mean age 23.8 years) with life-threatening complications or who responded poorly to conventional therapy. Peripheral blood stem cells were mobilized with cyclophosphamide, granulocyte colony-stimulating factor, and rituximab, and purified autologous CD34(+) stem cells were infused...
January 17, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28093595/mortality-and-cause-of-death-in-israeli-patients-with-pemphigus
#2
Khalaf Kridin, Shira Zelber Sagi, Reuven Bergman
All-cause and cause-specific mortality among patients with pemphigus compared with the general population is yet to be established. This study investigated overall mortality and cause-specific mortality in a large immunopathologically validated cohort of patients with pemphigus. Mortality of patients with pemphigus was compared with age- and gender-matched control subjects in the general population. All-cause and cause-specific standardized mortality ratios (SMRs) were estimated. The study cohort included 245 patients newly-diagnosed with pemphigus between January 1990 and June 2016...
January 17, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28089115/correlation-of-antimuscarinic-acetylcholine-receptor-antibody-titers-and-antidesmoglein-antibody-titers-with-the-severity-of-disease-in-patients-with-pemphigus
#3
Manimegalai Jeyasekaran Dhanabhakya Lakshmi, Telanseri Jaykar Jaisankar, Medha Rajappa, Devinder Mohan Thappa, Laxmisha Chandrashekar, Dakshinamurthy Divyapriya, Malathi Munisamy, Gunaseelan Revathy
BACKGROUND: Acetylcholine receptor (AchR) antibody levels significantly correlate with disease severity at initial pemphigus diagnosis and during follow-up. However, it is not clear if they are just an epiphenomenon or a potential trigger of the known pathogenic process in pemphigus vulgaris. OBJECTIVE: We sought to assess the changes in anti-muscarinic (M3) AchR and anti-desmoglein (Dsg) antibody titers with therapy. METHODS: This was a hospital-based cohort study involving 45 patients with active pemphigus...
January 11, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28079925/pregnancy-outcome-after-rituximab-treatment-before-conception-in-patients-affected-by-severe-pemphigus-vulgaris-superficialis
#4
C Vassallo, S Grassi, E Tagliabue, A Piccolo, V Brazzelli
Pregnancy is rare among patients affected by pemphigus, especially in European countries, mainly because of older patients, lower fertility rates and possible teratogenic risk.(1) Avoidance of immunosuppressive medications during pregnancy has been recommended, but inadequate treatment of pemphigus may increase morbidity and mortality.(2) Corticosteroids remain the first-line agent for treatment of pemphigus in pregnancy when low dosages are sufficient for disease control; however, prednisone and its metabolites crosses the placenta barrier, and high levels in the serum have been associated with adverse fetal outcomes...
January 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28064226/salivary-samples-for-the-diagnosis-of-pemphigus-vulgaris-using-the-biochip-approach-a-pilot-study
#5
Irene Russo, Andrea Saponeri, Anna Michelotto, Mauro Alaibac
Pemphigus vulgaris (PV) is a rare autoimmune intraepithelial blistering skin disease characterized by the presence of circulating autoantibodies against desmoglein 3 (DSG3) and desmoglein 1 (DSG1), resulting in loss of the normal epithelial cell-to-cell adhesion, through a process called acantholysis. In recent years, a BIOCHIP-based indirect immunofluorescence technique for the determination of anti-DSG3 and anti-DSG1 autoantibodies has been described. Even though, the use of saliva anti-DSG3 and anti-DSG1 ELISA for the diagnosis of PV has been already reported, there are no studies concerning the utilization of saliva by the BIOCHIP approach...
January 2017: In Vivo
https://www.readbyqxmd.com/read/28052410/can-immunohistochemistry-replace-immunofluorescence-in-diagnosis-of-skin-bullous-diseases
#6
Hanan Al-Saeid Al-Shenawy
Autoimmune bullous diseases are distressing and sometimes risky bullous dermatoses characterized by the presence of antibodies focused against disease-specific target antigens. Recognition of these antibodies using immunofluorescence is used to be the only sure diagnostic method after reviewing the routine histopathological section. Because of many causes that make the using of immunofluorescence difficult, we tried to evaluate the role of immunohistochemistry in diagnosis of these bullous skin diseases; 40 pemphigus cases (30 pemphigus vulgaris and 10 pemphigus foliaceus) and 37 non-pemphigus cases (35 vesiculobullous skin diseases and 2 normal skin)...
January 3, 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28052253/proteomic-analysis-of-pemphigus-autoantibodies-indicates-a-larger-more-diverse-and-more-dynamic-repertoire-than-determined-by-b-cell-genetics
#7
Jing Chen, Qi Zheng, Christoph M Hammers, Christoph T Ellebrecht, Eric M Mukherjee, Hsin-Yao Tang, Chenyan Lin, Huijie Yuan, Meng Pan, Jana Langenhan, Lars Komorowski, Don L Siegel, Aimee S Payne, John R Stanley
In autoantibody-mediated diseases such as pemphigus, serum antibodies lead to disease. Genetic analysis of B cells has allowed characterization of antibody repertoires in such diseases but would be complemented by proteomic analysis of serum autoantibodies. Here, we show using proteomic analysis that the serum autoantibody repertoire in pemphigus is much more polyclonal than that found by genetic studies of B cells. In addition, many B cells encode pemphigus autoantibodies that are not secreted into the serum...
January 3, 2017: Cell Reports
https://www.readbyqxmd.com/read/28038889/periodontitis-in-oral-pemphigus-and-pemphigoid-a-systematic-review-of-published-studies
#8
REVIEW
Isabella Jascholt, Olivia Lai, Detlef Zillikens, Michael Kasperkiewicz
Periodontitis and autoimmune bullous diseases, including pemphigus vulgaris and mucous membrane pemphigoid, are immunoinflammatory disorders leading to microbial plaque- and autoantibody-elicited tissue injury of the oral cavity, respectively. Evidence indicates that these autoimmune conditions may represent a risk factor for periodontitis, but no systematic evaluation exists to corroborate this assumption. A systematic literature review of periodontal status in pemphigus and pemphigoid was conducted. Electronic searches using PubMed from inception to July 2016 identified 10 studies meeting predetermined inclusion and exclusion criteria...
December 28, 2016: Journal of the American Academy of Dermatology
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
#9
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...
December 28, 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28035228/discrepancies-among-clinical-histological-and-immunological-findings-in-iga-pemphigus-a-case-report-and-literature-survey
#10
Kaja Męcińska-Jundziłł, Takashi Hashimoto, Cezary Kowalewski, Katarzyna Woźniak, Norito Ishii, Rafał Czajkowski
No abstract text is available yet for this article.
December 2016: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28025226/response-of-pemphigus-betae-hemiptera-aphididae-and-beneficial-epigeal-arthropod-communities-to-sugarbeet-plant-density-and-seed-applied-insecticide-in-western-nebraska
#11
R J Pretorius, G L Hein, E E Blankenship, F F Purrington, J D Bradshaw
This study investigated the impact of a neonicotinoid seed-applied insecticide (Poncho Beta) and two plant densities (86,487 and 61,776 plants per hectare) on the sugarbeet root aphid (Pemphigus betae Doane), beneficial epigeal arthropods, and selected crop yield parameters in sugarbeet (Beta vulgaris L. var. vulgaris). Ground beetles and centipedes were the most commonly collected taxa during 2012 and 2013, respectively. Centipede, spider, and rove beetle activity densities were not affected by the seed-applied insecticide, whereas plant density had a marginal effect on centipede activity density during 2012...
December 26, 2016: Environmental Entomology
https://www.readbyqxmd.com/read/28024684/non-pathogenic-pemphigus-foliaceus-pf-igg-acts-synergistically-with-a-directly-pathogenic-pf-igg-to-increase-blistering-by-p38mapk-dependent-desmoglein-1-clustering
#12
Kenji Yoshida, Ken Ishii, Atsushi Shimizu, Mariko Yokouchi, Masayuki Amagai, Ken Shiraishi, Yuji Shirakata, John R Stanley, Akira Ishiko
BACKGROUND: Pemphigus foliaceus (PF) is an autoimmune blistering disease caused by autoantibodies (Abs) against desmoglein 1 (Dsg1). PF sera contain polyclonal Abs which are heterogeneous mixture of both pathogenic and non-pathogenic Abs, as shown by isolation of monoclonal Abs (mAbs). OBJECTIVE: To investigate how pathogenic and non-pathogenic anti-Dsg1 Abs contribute to blister formation in PF. METHODS: Using organ-cultured human skin, we compared the effect of a single pathogenic anti-Dsg1 IgG mAb, a single non-pathogenic anti-Dsg1 IgG mAb, and their mixture on blister formation as analyzed by histology, subcellular localization of IgG deposits and desmosomal proteins by confocal microscopy, and desmosomal structure by electron microscopy...
December 12, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28012821/validation-of-chemiluminescent-enzyme-immunoassay-in-detection-of-autoantibodies-in-pemphigus-and-pemphigoid
#13
Yumi Fujio, Kazuo Kojima, Masahiro Hashiguchi, Masatoshi Wakui, Mitsuru Murata, Masayuki Amagai, Jun Yamagami
BACKGROUND: A novel chemiluminescent enzyme immunoassay (CLEIA) was recently developed to quantify autoantibodies specific for desmogleins (Dsgs) and BP180, the target antigens of pemphigus and pemphigoid. This assay is automated and highly accurate and efficient. OBJECTIVE: To validate the use of the CLEIA for detection of autoantibodies during the clinical courses of patients with pemphigus and pemphigoid. METHODS: To define cut-off values for Dsg1, Dsg3, and BP180, we evaluated 47 serum samples from patients with pemphigus foliaceus (PF), 59 from those with pemphigus vulgaris (PV), 52 from those with bullous pemphigoid (BP), and 995 from healthy individuals...
December 6, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28010761/research-techniques-made-simple-mouse-models-of%C3%A2-autoimmune-blistering-diseases
#14
Robert Pollmann, Rüdiger Eming
Autoimmune blistering diseases are examples of autoantibody-mediated, organ-specific autoimmune disorders. Based on a genetic susceptibility, such as a strong HLA-class II association, as yet unknown triggering factors induce the formation of circulating and tissue-bound autoantibodies that are mainly directed against adhesion structures of the skin and mucous membranes. Compared with other autoimmune diseases, especially systemic disorders, the pathogenicity of autoimmune blistering diseases is relatively well described...
January 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28005817/pemphigus-vulgaris-how-to-perform-an-oral-biopsy-properly
#15
Aneliza de Fatima Moraes da Silva, Daniel Galera Bernabé, Glauco Issamu Miyahara, Eder Ricardo Biasoli, Renata Callestini, Kellen Cristine Tjioe
Oral biopsy of vesiculobullous diseases such as pemphigus vulgaris often raises questions due to some particularities involving this procedure. The adequate selection of the area to be biopsied defines if the final diagnosis will be reached, being the Achiles heel of the proper management of the patient. Here, the authors report a case of a woman who sought for treatment of generalized oral blisters and ulcers that caused severe pain. She had undergone a previous biopsy by other professionals that was inconclusive...
November 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27981628/concurrent-pemphigus-foliaceus-and-graves-disease
#16
Rachel Laarman, Kimberly A Horii
Pemphigus foliaceus (PF) is an uncommon immunobullous disease in children. Other autoimmune diseases have rarely been reported in children with PF. We report the case of an adolescent girl who presented with concurrent PF and Graves' disease.
December 16, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27981211/concomitant-pemphigus-herpetiformis-and-sarcoidosis
#17
Aaron R Mangold, Collin M Costello, Mark R Pittelkow, David J DiCaudo
No abstract text is available yet for this article.
November 2016: JAAD Case Reports
https://www.readbyqxmd.com/read/27931211/gonadectomy-effects-on-the-risk-of-immune-disorders-in-the-dog-a-retrospective-study
#18
Crystal R Sundburg, Janelle M Belanger, Danika L Bannasch, Thomas R Famula, Anita M Oberbauer
BACKGROUND: Gonadectomy is one of the most common procedures performed on dogs in the United States. Neutering has been shown to reduce the risk for some diseases although recent reports suggest increased prevalence for structural disorders and some neoplasias. The relation between neuter status and autoimmune diseases has not been explored. This study evaluated the prevalence and risk of atopic dermatitis (ATOP), autoimmune hemolytic anemia (AIHA), canine myasthenia gravis (CMG), colitis (COL), hypoadrenocorticism (ADD), hypothyroidism (HYPO), immune-mediated polyarthritis (IMPA), immune-mediated thrombocytopenia (ITP), inflammatory bowel disease (IBD), lupus erythematosus (LUP), and pemphigus complex (PEMC), for intact females, intact males, neutered females, and neutered males...
December 8, 2016: BMC Veterinary Research
https://www.readbyqxmd.com/read/27914107/non-paraneoplastic-autoimmune-subepidermal-bullous-disease-associated-with-fatal-bronchiolitis-obliterans
#19
Mari Orime, Katsuhiro Tomiyama, Hideki Hashidate, Satoru Yoshida, Satoshi Hokari, Akiko Tsuda, Hisashi Yokoyama, Jun-Ichi Narita, Youhei Uchida, Takuro Kanekura, Riichiro Abe, Norito Ishii, Takashi Hashimoto, Kazuhiro Kawai
Bronchiolitis obliterans is a small-airway obstructive lung disease for which immunologically mediated pathogenesis is supposed. Frequent association of bronchiolitis obliterans with paraneoplastic pemphigus is well known, but its association with other autoimmune bullous diseases has not been reported except for a case of anti-laminin-332-type mucous membrane pemphigoid in a patient with chronic graft-versus-host disease. We report a case of non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans in a patient without transplantation...
December 3, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27902996/pemphigus-pathogenesis-to-treatment
#20
Christopher DiMarco
Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. These lesions correlate histologically with splits forming in the epidermis, leaving a blister roof composed of a few cell layers...
December 1, 2016: Rhode Island Medical Journal
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