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https://www.readbyqxmd.com/read/28932036/current-concepts-of-pemphigus-with-a-deep-insight-into-its-molecular-aspects
#1
REVIEW
Jayakiran Madala, Rithika Bashamalla, M Praveen Kumar
Pemphigus vulgaris is an autoimmune bullous disease involving both the skin and mucosal areas, which is characterized by intraepithelial flaccid blisters and erosions. The pathogenesis of this disease is not yet completely established, but novel intuitions into its pathogenesis have recently been published. An unanswered question in its pathophysiology is the mechanism of acantholysis or loss of keratinocyte cell adhesion. Acantholysis seems to result from a communal action of autoantibodies against numerous keratinocyte self-antigens, of which desmogleins 1 and 3, desmocollins and nondesmosome components, such as the mitochondrion, might take part in the disease initiation...
May 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
https://www.readbyqxmd.com/read/28928733/crosstalk-between-signaling-pathways-in-pemphigus-a-role-for-endoplasmic-reticulum-stress-in-p38-mitogen-activated-protein-kinase-activation
#2
Gabriel A Cipolla, Jong Kook Park, Robert M Lavker, Maria Luiza Petzl-Erler
Pemphigus consists of a group of chronic blistering skin diseases mediated by autoantibodies (autoAbs). The dogma that pemphigus is caused by keratinocyte dissociation (acantholysis) as a distinctive and direct consequence of the presence of autoAb targeting two main proteins of the desmosome-desmoglein (DSG) 1 and/or DSG3-has been put to the test. Several outside-in signaling events elicited by pemphigus autoAb in keratinocytes have been described, among which stands out p38 mitogen-activated protein kinase (p38 MAPK) engagement and its apoptotic effect on keratinocytes...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28916029/oral-mucosa-biology-and-salivary-biomarkers
#3
Rosie Qin, Andrea Steel, Nasim Fazel
Although the surfaces of both the skin and oral mucosa are protected by squamous epithelial cells and fall within the scope of dermatologic practice, the oral cavity contains highly specialized structures and functions distinct from other skin biology and pathologic conditions and are also the purview of clinicians who care for patients with skin and mucosal diseases. We describe the distinct features of the tongue, mucosa, and salivary glands. In particular, we examine the composition and function of the saliva, with special focus on salivary biomarkers...
September 2017: Clinics in Dermatology
https://www.readbyqxmd.com/read/28906088/bullous-cutaneous-larva-migrans-case-series-and-review-of-atypical-clinical-presentations
#4
Stefano Veraldi, Ermira Çuka, Paolo Pontini, Fabrizio Vaira
Hookworm-related cutaneous larva migrans (HRCLM) is caused by the penetration and migration in the epidermis of larvae of Ancylostoma braziliense and Ancylostoma caninum. It is characterized by slightly raised and erythematous tracks, located especially on the feet. These tracks may be single or multiple, serpiginous or linear, ramified and intertwined, accompanied by pruritus. Atypical clinical presentations of HRCLM are currently more frequent than in the past. We present six patients with bullous HRCLM and discuss the possible pathogenetic factors...
October 2017: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/28905096/comparison-of-suction-blistering-and-tape-stripping-for-analysis-of-epidermal-genes-proteins-and-lipids
#5
Marek Svoboda, Marta Hlobilová, Markéta Marešová, Michaela Sochorová, Andrej Kováčik, Kateřina Vávrová, Iva Dolečková
Analysis of epidermal genes, proteins and lipids is important in the research and diagnosis of skin diseases. Although punch biopsy is the first-choice technique for the skin sampling, it is unnecessarily invasive for obtaining a sample just for the epidermal analysis. Here we compare two less invasive methods, suction blistering (SB) and tape stripping (TS), for the analysis of selected epidermal genes (quantitative real-time reverse transcription PCR, qRT-PCR), proteins (western blotting, WB), and lipids in ten healthy volunteers...
September 13, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28892093/rapid-generation-of-col7a1-mouse-model-of-recessive-dystrophic-epidermolysis-bullosa-and-partial-rescue-via-immunosuppressive-dermal-mesenchymal-stem-cells
#6
Beau R Webber, Kyle T O'Connor, Ron T McElmurry, Elise N Durgin, Cindy R Eide, Christopher J Lees, Megan J Riddle, Wendy E Mathews, Natasha Y Frank, Mark A Kluth, Christoph Ganss, Branden S Moriarity, Markus H Frank, Mark J Osborn, Jakub Tolar
Recessive dystrophic epidermolysis bullosa (RDEB) is a debilitating and ultimately lethal blistering disease caused by mutations to the Col7a1 gene. Development of novel cell therapies for the treatment of RDEB would be fostered by having immunodeficient mouse models able to accept human cell grafts; however, immunodeficient models of many genodermatoses such as RDEB are lacking. To overcome this limitation, we combined the clustered regularly interspaced short palindromic repeats and associated nuclease (CRISPR/Cas9) system with microinjection into NOD/SCID IL2rγc(null) (NSG) embryos to rapidly develop an immunodeficient Col7a1(-/-) mouse model of RDEB...
September 11, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28890623/childhood-occurrence-of-pemphigus
#7
Raju U Patil, Rajesh T Anegundi, Kumar R Gujjar, K R Indushekar
Pemphigus is a chronic mucocutaneous disease that initially manifests in the form of intraoral blisters which spread to other mucous membrane and skin. This study describes an unusual case of chronic generalized childhood pemphigus disease in an 11-year-old girl, who presented with multiple vesicles all over her body. Such a condition is seen more often in older people rather than children. It is crucial for dental professionals to be familiar with the diagnosis of bullous skin diseases in children and adolescents, especially in its initial stages in order to prevent the serious consequences and morbidity...
April 2017: International Journal of Clinical Pediatric Dentistry
https://www.readbyqxmd.com/read/28887841/evidence-for-a-contributory-role-of-a-xenogeneic-immune-response-in-experimental-epidermolysis-bullosa-acquisita
#8
Markus Niebuhr, Michael Kasperkiewicz, Sebastian Maass, Eva Hauenschild, Katja Bieber, Ralf J Ludwig, Jürgen Westermann, Kathrin Kalies
Autoimmune diseases affect a large fraction of the population in Western countries. To elucidate the underlying causes, autoantibody transfer-induced mouse models have been established that greatly contributed to the understanding of the pathophysiology of these diseases. However, the role of a potentially co-occurring murine xenogeneic immune response to commonly utilized rabbit anti-mouse IgG remains poorly understood. Using the established rabbit anti-mouse type VII collagen (COL7) IgG-induced mouse model of the mucocutaneous blistering disorder epidermolysis bullosa acquisita (EBA), we found in this study a profound T and B cell response along with an altered cytokine expression profile in draining lymph nodes of mice injected with the xenogeneic IgG...
September 8, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28887823/variation-of-the-epidermal-expression-of-glucocorticoid-receptor-beta-as-potential-predictive-marker-of-bullous-pemphigoid-outcome
#9
Adrien Brulefert, Sébastien Le Jan, Julie Plée, Anne Durlach, Philippe Bernard, Frank Antonicelli, Aurélie Trussardi-Régnier
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries. Although topical and/or systemic glucocorticoids treatment efficacy is widely recognized, up to 30% of patients with BP may undergo a relapse during the first year of treatment. We investigated the protein expression of the total glucocorticoid receptor and GRβ isoform in the skin biopsy specimens from patients with BP, and wondered whether such investigation at baseline provided a tool to predict disease outcome...
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
#10
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/28856675/failure-of-initial-disease-control-in-bullous-pemphigoid-a-retrospective-study-of-hospitalized-patients-in-a-single-tertiary-center
#11
Noa Kremer, Tal Zeeli, Eli Sprecher, Shamir Geller
BACKGROUND: Bullous pemphigoid (BP) is the most prevalent autoimmune blistering skin disease in Western countries and in Israel. Initial disease control is achieved in 60-90% of BP patients within 1-4 weeks of corticosteroid therapy. In the remainder of patients, recalcitrant disease is controlled with additional immunosuppressive treatment. OBJECTIVES: We aimed to evaluate the rate of BP patients who needed adjuvant therapy to achieve initial disease control and to identify potential predictors for recalcitrant disease...
October 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28854309/determining-the-incidence-of-pneumocystis-pneumonia-in-patients-with-autoimmune-blistering-diseases-not-receiving-routine-prophylaxis
#12
Kyle T Amber, Aniek Lamberts, Farzan Solimani, Arianna F Agnoletti, Dario Didona, Ilona Euverman, Emanuele Cozzani, Lee Haur Yueh, Giovanni Di Zenzo, Yael Anne Leshem, Daniel Mimouni, Michael Hertl, Barbara Horvath
Importance: Pneumocystis pneumonia (PCP) is a potentially lethal opportunistic infection that primary prophylaxis can help prevent. The risk of prophylactic therapy must be weighed against the incidence of PCP in the patient population. Prophylaxis most frequently involves trimethoprim-sulfamethoxazole, with second-line therapies, including atovaquone, dapsone, and pentamide. The indication for prophylaxis in immunocompromised patients without HIV is less well defined. Previously, an incidence of at least 3...
August 30, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28842325/c-terminal-processing-of-collagen-xvii-induces-neoepitopes-for-linear-iga-dermatosis-autoantibodies
#13
Ellen Toyonaga, Wataru Nishie, Kentaro Izumi, Ken Natsuga, Hideyuki Ujiie, Hiroaki Iwata, Jun Yamagami, Yoshiaki Hirako, Daisuke Sawamura, Wataru Fujimoto, Hiroshi Shimizu
Transmembrane collagen XVII (COL17) is a hemidesmosomal component of basal keratinocytes that can be targeted by autoantibodies in autoimmune blistering disorders, including linear IgA dermatosis (LAD). The COL17 can be physiologically cleaved within the juxtamembranous extracellular NC16A domain, and LAD autoantibodies preferentially react with the processed ectodomains, indicating that the processing induces neoepitopes. However, the details of how neoepitopes develop have not been elucidated. In this study, we show that C-terminal processing of COL17 also plays a role in inducing neoepitopes for LAD autoantibodies...
August 22, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28839322/breast-cancer-in-a-patient-with-kindlers-syndrome
#14
Itrat Mehdi, Bassim Jaffar Al Bahrani, Taha Mohsin Al Lawati, Zahid Al Mandhari, Fatima Ramadhan Al Lawati
Breast Cancer (BC) has associated risk factors and genetic factors like BRCA1, and BRCA2. Many benign and malignant disease processes are found concurrently with BC and believed to be additional risk factors like gall bladder stones (cholelithiasis), hypertension, diabetes mellitus, cerebrovascular lesions, arthritis, spine and spinal cord degenerative lesions, infertility, depression, sleep disturbances, obesity, autoimmune diseases (SLE), and thyroid diseases. There are some malignant disease associations like synchronous or metachronous ovarian, colonic and endometrial tumours with Breast cancer...
August 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28833620/eosinophils-as-putative-therapeutic-targets-in-bullous-pemphigoid
#15
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 is 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/28833569/in-vitro-and-in-vivo-models-to-investigate-the-pathomechanisms-and-novel-treatments-for-pemphigoid-diseases
#16
Katja Bieber, Hiroshi Koga, Wataru Nishie
Pemphigoid diseases (PD) are a subgroup of rare acute or chronic autoimmune skin disorders characterized and caused by autoantibodies directed against distinct structural components of the dermal-epidermal junction. Binding of autoantibodies to their targets leads to the formation of blisters and erosions in patients. PDs comprise eight disorders for which the molecular target antigens have been identified. First, we review the available in vitro and ex vivo models for analysis of distinct aspects of the pathogenesis of PDs...
August 20, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28821219/early-congenital-syphilis-recognising-symptoms-of-an-increasingly-prevalent-disease
#17
Nupur U Patel, Elias Oussedik, Erin T Landis, Lindsay C Strowd
BACKGROUND: Congenital syphilis (CS) is an infectious disease resulting from transplacental transmission of Treponema pallidum spirochetes from an infected mother to fetus during pregnancy. While uncommon, CS has shown an increased incidence in Canada and the United States since 2001 and 2012, respectively. CASE REPORT: We present the case of a 5-week-old female infant with blistering rash on the palms and soles. The infant displayed decreased movement of the left upper extremity, clinically consistent with Parrot pseudoparalysis...
August 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28813559/coverage-of-intravenous-immunoglobulin-for-autoimmune-blistering-diseases-among-us-insurers
#18
Manuel Valdebran, Kyle T Amber
No abstract text is available yet for this article.
August 16, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28811514/differential-epitope-recognition-in-the-immunodominant-staphylococcal-antigen-a-of-staphylococcus-aureus-by-mouse-versus-human-igg-antibodies
#19
Dennis G A M Koedijk, Francisco Romero Pastrana, Hedzer Hoekstra, Sanne van den Berg, Jaap Willem Back, Carolien Kerstholt, Rianne C Prins, Irma A J M Bakker-Woudenberg, Jan Maarten van Dijl, Girbe Buist
The immunodominant staphylococcal antigen A (IsaA) is a potential target for active or passive immunization against the important human pathogen Staphylococcus aureus. Consistent with this view, monoclonal antibodies against IsaA were previously shown to be protective against S. aureus infections in mouse models. Further, patients with the genetic blistering disease epidermolysis bullosa (EB) displayed high IsaA-specific IgG levels that could potentially be protective. Yet, mice actively immunized with IsaA were not protected against S...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28809039/juvenile-melanomas-western-australian-melanoma-advisory-service-experience
#20
Jie Xin Xu, Sharnice Koek, Samantha Lee, Mark Hanikeri, Mark Lee, Trevor Beer, Christobel Saunders
BACKGROUND/OBJECTIVES: Juvenile melanoma (before 20 years of age) is a rare condition with poorly defined risk factors. We describe features of juvenile melanoma in Western Australia over the last two decades. METHOD: A retrospective review of juvenile melanomas was conducted from prospectively maintained databases, reviewed for patients' characteristics, clinical information, histology, treatment, recurrence and survival data. RESULTS: Altogether 95 cases of juvenile melanoma were reported to the Western Australian Cancer Registry between 2000 and 2013...
August 15, 2017: Australasian Journal of Dermatology
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