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Anh Tran Thi Van, Thuong Van Nguyen, Sau Nguyen Huu, Lan Pham Thi, Phuong Pham Thi Minh, NghiDinh Huu, Van Tran Cam, My Le Huyen, Minh Vu Nguyet, Khang Tran Hau, Marco Gandolfi, Francesca Satolli, Claudio Feliciani, Michael Tirant, Aleksandra Vojvodic, Torello Lotti
BACKGROUND: Pemphigus Vulgaris (PV) is a chronic disease, is characterized by the presence of flacid bullous in skin and mucosa. There are 2 main autoantibodies against desmoglein3 (Dsg3) and desmoglein1 (Dsg1). AIM: The aims of this study were to evaluate the before and after treatment outcome with corticosteroid, using Desmoglein ELISA test. METHOD: Forty patients with Pemphigus include 36 PV and 4 PF (28 women, 12 women) were enrolled. The titers of Dsg in pemphigus patients by using ELISA test were done before and 1-month treatment...
January 30, 2019: Open Access Macedonian Journal of Medical Sciences
Sacarin Bunbanjerdsuk, Nutchavadee Vorasan, Thammakorn Saethang, Tanjitti Pongrujikorn, Duangjai Pangpunyakulchai, Narongsak Mongkonsiri, Lalida Arsa, Nintita Thokanit, Warut Pongsapich, Tauangtham Anekpuritanang, Nuttapong Ngamphaiboon, Artit Jinawath, Somkiat Sunpaweravong, Trairak Pisitkun, Bhoom Suktitipat, Natini Jinawath
Patients with head and neck squamous cell carcinoma are at increased risk of developing a second primary malignancy, which is associated with poor prognosis and early death. To help improve clinical outcome, we aimed to identify biomarkers for second primary malignancy risk prediction using the routinely obtained formalin-fixed paraffin-embedded tissues of the index head and neck cancer. Liquid chromatography-tandem mass spectrometry was initially performed for candidate biomarker discovery in 16 pairs of primary cancer tissues and their matched normal mucosal epithelia from head and neck squamous cell carcinoma patients with or without second primary malignancy...
February 8, 2019: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Toshiya Handa, Takafumi Kamiya, Yasuyuki Sumikawa, Tomoyuki Minowa, Ayako Kumagai, Masahide Sawada, Hisashi Uhara
No abstract text is available yet for this article.
January 30, 2019: JAMA Dermatology
Elena Svirshchevskaya, Ekaterina Doronina, Maria Grechikhina, Elena Matushevskaya, Olga Kotsareva, Gulnar Fattakhova, Alexander Sapozhnikov, Klaus Felix
AIMS: Multicellular tumor spheroids (MCTS) produced by different methods vary in forms, sizes, and properties. The aim of this work was to characterize MCTS formed by six pancreatic cell lines on a non-adherent surface. MATERIALS AND METHODS: Human pancreatic cells were grown in 2D and 3D conditions and compared for the expression of E- and desmosomal cadherins (PCR, confocal microscopy), growth, cell cycling, apoptosis (flow cytometry), and a response to antitumor drugs doxorubicin and gemcitabine (MTT-assay)...
January 23, 2019: Life Sciences
Khalaf Kridin, Rimma Laufer-Britva, Mouhammad Kridin, Doron Comaneshter, Erez Batat, Arnon D Cohen
The coexistence of pemphigus and systemic lupus erythematosus (SLE) had been reported anecdotally. Anti-desmoglein (Dsg)1 and anti-Dsg3 antibodies were detected concomitantly with antinuclear autoantibodies among blood donors. The aim of the current study was to study the association between pemphigus and SLE in Israeli patients and to synthesize existing data on this association in the current literature. The current study included two sections. Initially, a cross-sectional study was performed to compare pemphigus patients with age-, sex-, and ethnicity-matched control subjects regarding the prevalence of SLE using a real-life large-scale computerized database...
January 14, 2019: Immunologic Research
I Ujiie, H Ujiie, H Iwata, H Shimizu
BACKGROUND: More than half of pemphigus patients experience relapse during the disease course. However, the risk factors and clinical and immunological characteristics of relapse remain largely unclear. OBJECTIVE: To elucidate risk factors and clinical features of pemphigus relapse. METHODS: Retrospective review of the clinical records of 42 pemphigus cases in a single center. RESULTS: 61·9% of cases experienced relapse, usually when oral prednisolone was tapered to around 0·1mg/kg...
December 26, 2018: British Journal of Dermatology
Jens Waschke
Cell-Cell contacts are crucial for intercellular cohesion and formation of endothelial and epithelial barriers. Desmosomes are the adhesive contacts providing mechanical strength to epithelial intercellular adhesion and therefore are most abundant in tissues subjected to high mechanical stress such as the epidermis and heart muscle. Desmogleins (Dsg) besides intercellular adhesion serve as signalling hubs regulating cell behaviour. In desmosomal diseases such as the autoimmune blistering skin disease pemphigus or arrhythmic cardiomyopathy (AC), which is caused by mutations of desmosomal components of cardiomyocyte intercalated discs, the adhesive and signalling functions of desmosomes are impaired...
December 7, 2018: Annals of Anatomy, Anatomischer Anzeiger: Official Organ of the Anatomische Gesellschaft
Jong Hoon Kim, Song-Ee Kim, Hae Seok Park, Si-Hyung Lee, Sang Eun Lee, Soo-Chan Kim
No abstract text is available yet for this article.
December 5, 2018: Journal of Investigative Dermatology
Chandar Kumar, Shen Song, Lin Jiang, Xiaohong He, Qianjun Zhao, Yabin Pu, Kanwar Kumar Malhi, Asghar Ali Kamboh, Yuehui Ma
The Tibetan cashmere goat is one of the main goat breeds used by people living in the plateau. It exhibits the distinct phenotypic characteristics observed in lowland goats, allowing them to adapt to the challenging conditions at high altitudes. It provides an ideal model for understanding the genetic mechanisms underlying high-altitude adaptation and hypoxia-related diseases. Our previous exome sequencing of five Chinese cashmere breeds revealed a candidate gene, DSG3 (Desmoglein 3 ), responsible for the high-altitude adaptation of the Tibetan goat...
2018: Frontiers in Genetics
Islam Saad, Suzan Salem
AIM: The current study will attempt to throw light on the role of desmoglein 1 and desmoglein 3 in the pathogenesis of erosive lichen planus and their response to topical application of tacrolimus. MATERIALS AND METHODS: Twenty patients with erosive oral lichen planus received tacrolimus ointment three times daily for eight weeks. Assessments using the clinical score and a visual analog scale were recorded at each visit. Serum concentrations of circulating autoantibodies to desmoglein 1 and desmoglein 3 will be determined by enzyme-linked immunosorbent assay (ELISA) at baseline, four weeks and eight weeks after treatment...
October 1, 2018: Journal of Contemporary Dental Practice
Vivien Hebert, Claire Boulard, Estelle Houivet, Sophie Duvert Lehembre, Luca Borradori, Rocco Della Torre, Claudio Feliciani, Luca Fania, Giovanna Zambruno, Diana B Camaioni, Biago Didona, Branka Marinovic, Enno Schmidt, Nina Schumacher, Christian Hünefeld, Stefan Schanz, Johannes Steffen Kern, Silke Hofmann, Anne Charlotte Bouyeure, Catherine Picard-Dahan, Catherine Prost-Squarcioni, Frederic Caux, Marina Alexandre, Saskia Ingen-Housz-Oro, Martine Bagot, Emmanuelle Tancrede-Bohin, Jean David Bouaziz, Nathalie Franck, Pierre Vabres, Bruno Labeille, Marie Aleth Richard, Emmanuel Delaporte, Alain Dupuy, Michel D'Incan, Gaelle Quereux, François Skowro, Carle Paul, Cristina Bulai Livideanu, Marie Beylot-Barry, Marie Sylvie Doutre, Martine Avenel-Audran, Christophe Bedane, Philippe Bernard, Laurent Machet, Hervé Maillard, Denis Jullien, Sebastien Debarbieux, Bruno Sassolas, Laurent Misery, Claire Abasq, Olivier Dereure, Philippe Lagoutte, Vincent Ferranti, Victoria P Werth, Dedee F Murrell, Michael Hertl, Jacques Benichou, Pascal Joly
The Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity-Score (ABSIS) scores have been proposed to provide an objective measure of pemphigus activity. These scores have been evaluated only on already treated patients mainly with mild to moderate activity. The objective was to assess the interrater reliability of ABSIS and PDAI scores and their correlation with other severity markers in a large international study. Consecutive patients with newly diagnosed pemphigus were enrolled in 31 centers...
October 6, 2018: Journal of Investigative Dermatology
Emmanuelle Jeannot, Alexandre Harlé, Allyson Holmes, Xavier Sastre-Garau
Anal carcinomas (AC) are associated with human papillomavirus (HPV) DNA sequences, but little is known about the physical state of the viral genome in carcinoma cells. To define the integration status and gene(s) targeted by viral insertions in AC, tumor DNAs extracted from 35 tumor specimen samples in patients with HPV16-associated invasive carcinoma were analyzed using the detection of integrated papillomavirus sequences-PCR approach. The genomic status at integration sites was assessed using comparative genomic hybridization-array assay and gene expression using reverse transcription quantitative PCR (RT-qPCR)...
December 2018: Genes, Chromosomes & Cancer
M Sar-Pomian, J Czuwara, L Rudnicka, M Olszewska
BACKGROUND: Pemphigus-associated alopecia is considered rare, and has not been studied in detail. AIM: To evaluate the clinical and immunological characteristics of patients with pemphigus-associated alopecia. METHODS: This prospective observational study included 80 consecutive patients with histopathologically and immunopathologically confirmed pemphigus, of whom 11 (13.8%) were found to have pemphigus-associated alopecia. Alopecia was observed in 11/52 patients with pemphigus and scalp involvement: [0/28 (35...
September 24, 2018: Clinical and Experimental Dermatology
Shin-Ichi Funahashi, Shigeto Kawai, Etsuko Fujii, Kenji Taniguchi, Kiyotaka Nakano, Shumpei Ishikawa, Hiroyuki Aburatani, Masami Suzuki
It is ideal for the target antigen of a cytotoxic therapeutic antibody against cancer to be cancer-specific, but such antigens are rare. Thus an alternative strategy for target selection is necessary. DSG3 is highly expressed in lung squamous cell carcinoma, while it is well known that anti-DSG3 antibodies cause pemphigus vulgaris, an autoimmune disease. We evaluated DSG3 as a novel target by selecting an epitope that exerts efficacy against cancer with no pathogenic effects in normal tissues. Pathogenic anti-DSG3 antibodies induce skin blisters by inihibiting the cell-cell interaction in a Ca2+-dependent manner...
September 18, 2018: Journal of Biochemistry
Maxi Hofrichter, Jenny Dworschak, Shirin Emtenani, Jana Langenhan, Fanny Weiß, Lars Komorowski, Detlef Zillikens, Winfried Stöcker, Christian Probst, Enno Schmidt, Stephanie Goletz
Pemphigus vulgaris (PV) is a potentially life-threatening autoimmune blistering disease which is associated with autoantibodies directed against two desmosomal proteins, desmoglein (Dsg) 3 and 1. Treatment of PV is rather challenging and relies on the long-term use of systemic corticosteroids and additional immunosuppressants. More recently, autoantibody-depleting therapies such as rituximab, high-dose intravenous immunoglobulins, and immunoadsorption were shown to be valuable treatment options in PV. Specific removal of pathogenic autoantibodies would further increase efficacy and usability of immunoadsorption...
2018: Frontiers in Immunology
Nina van Beek, Detlef Zillikens, Enno Schmidt
Blasenbildende Autoimmundermatosen (BAIDs) sind eine heterogene Gruppe seltener Erkrankungen, die klinisch durch Erosionen und/oder Blasen an Haut und Schleimhäuten charakterisiert sind. BAIDs können in zwei Gruppen eingeteilt werden: Pemphigus-Erkrankungen, die durch intraepidermale Blasenbildung und Autoantikörper gegen desmosomale Proteine wie Desmoglein (Dsg) 1, Dsg3 und Mitglieder der Plakin-Familie charakterisiert sind, sowie subepidermale BAIDs, die Pemphigoid-Erkrankungen und die Dermatitis herpetiformis umfassen...
September 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Nina van Beek, Detlef Zillikens, Enno Schmidt
Autoimmune bullous disorders (AIBDs) are a heterogeneous group of rare diseases clinically characterized by erosions and/or blisters on the skin and mucous membranes. AIBDs can be categorized into two groups: pemphigus diseases, characterized by intraepidermal blistering and autoantibodies against desmosomal proteins such as desmoglein (Dsg) 1, Dsg3, members of the plakin family, and subepidermal AIBDs, comprised of pemphigoid diseases and dermatitis herpetiformis. Autoantibodies in dermatitis herpetiformis target transglutaminases 2 and 3, while in pemphigoid disease, autoantibodies are directed against structural proteins of the dermal-epidermal junction...
September 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Justin M Hintze, Yourka D Tchoukalova, Ramachandra Sista, Manisha K Shah, Nan Zhang, David G Lott
INTRODUCTION: Reconstruction of respiratory epithelium is critical for the fabrication of bioengineered airway implants. Epithelial differentiation is typically achieved using bovine pituitary extract (BPE). Due to the xenogenic nature and undefined composition of BPE, an alternative for human clinical applications, devoid of BPE, must be developed. The goal of this study was to develop two different BPE-free media, with and without select pituitary hormone (PH), which could initiate epithelial differentiation for use in human implantation...
September 18, 2018: Biochemical and Biophysical Research Communications
Michael McFarlane, Ayesha Azam, David Snead, Ben Disney
Pemphigus vulgaris (PV) is a rare autoimmune bullous disease which affects the skin and mucous membranes. Oesophageal involvement is rare and has previously been limited to case reports and case series. A recent large case series of 477 PV patients showed that 26/477 (5.4%) had symptomatic oesophageal involvement. We present the case of a 54-year-old Somalian lady with a 10-year history of cutaneous PV, currently in remission, who developed dysphagia and odynophagia and was subsequently found to have oesophageal PV involvement with multiple flaccid bullae which were positive for anti-DSG3 antibodies on in-direct immunofluorescence...
August 23, 2018: Clinical Journal of Gastroenterology
Kyle T Amber, Manuel Valdebran, Sergei A Grando
Paraneoplastic autoimmune multiorgan syndrome (PAMS) is characterized by a heterogenous group of signs and symptoms including severe desquamative stomatitis, a polymorphous cutaneous eruption, humoral immunity against plakin proteins, contribution of cell-mediated autoimmunity and commonly a progressive respiratory failure. Autoantibodies in PAMS target a wide array of antigens including plakins, cadherins, alpha-2-macroglobulin like 1 (A2ML1), BP180, plakophilin-3, and several neuromuscular antigens. Originally described as paraneoplastic pemphigus in 1990 due to some of its clinical and immunologic similarities to classic pemphigus (pemphigus vulgaris and pemphigus foliaceus), PAMS is a multiorganopathy with several distinct features from these classic forms of pemphigus...
October 2018: Autoimmunity Reviews
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