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Blistering disease

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https://www.readbyqxmd.com/read/28811514/differential-epitope-recognition-in-the-immunodominant-staphylococcal-antigen-a-of-staphylococcus-aureus-by-mouse-versus-human-igg-antibodies
#1
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
#2
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
https://www.readbyqxmd.com/read/28808661/the-role-of-intereukin-31-in-pathogenesis-of-itch-and-its-intensity-in-a-course-of-bullous-pemphigoid-and-dermatitis-herpetiformis
#3
Lilianna Kulczycka-Siennicka, Anna Cynkier, Elżbieta Waszczykowska, Anna Woźniacka, Agnieszka Żebrowska
Itch which is one of the major, subjective symptoms in a course of bullous pemphigoid and dermatitis herpetiformis makes those two diseases totally different than other autoimmune blistering diseases. Its pathogenesis is still not fully known. The aim of this research was to assess the role of IL-31 in development of itch as well as to measure its intensity. Obtained results, as well as literature data, show that lower concentration of IL-31 in patients' serum may be correlated with its role in JAK/STAT signaling pathway which is involved in development of autoimmune blistering disease...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28801011/paraneoplastic-pemphigus-a-life-threatening-autoimmune-blistering-disease
#4
A Tirado-Sánchez, A Bonifaz
Paraneoplastic pemphigus (PNP), a subset of pemphigus, is a unique autoimmune blistering condition that can affect multiple organs other than the skin. It is a life-threatening disease associated with an underlying malignancy, most commonly of lymphoproliferative origin. The clinical picture may resemble pemphigus, pemphigoid, erythema multiforme, graft-versus-host disease, or lichen planus. The earliest and most consistent finding is a painful, severe, chronic and often recalcitrant stomatitis. Treatment of PNP is difficult...
August 8, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28800953/col7a1-editing-via-crispr-cas9-in-recessive-dystrophic-epidermolysis-bullosa
#5
Stefan Hainzl, Patricia Peking, Thomas Kocher, Eva M Murauer, Fernando Larcher, Marcela Del Rio, Blanca Duarte, Markus Steiner, Alfred Klausegger, Johann W Bauer, Julia Reichelt, Ulrich Koller
Designer nucleases allow specific and precise genomic modifications and represent versatile molecular tools for the correction of disease-associated mutations. In this study, we have exploited an ex vivo CRISPR/Cas9-mediated homology-directed repair approach for the correction of a frequent inherited mutation in exon 80 of COL7A1, which impairs type VII collagen expression, causing the severe blistering skin disease recessive dystrophic epidermolysis bullosa. Upon CRISPR/Cas9 treatment of patient-derived keratinocytes, using either the wild-type Cas9 or D10A nickase, corrected single-cell clones expressed and secreted similar levels of type VII collagen as control keratinocytes...
July 13, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28793012/refractory-pemphigus-foliaceus-associated-with-herpesvirus-infection-case-report
#6
Nurimar C Fernandes, Heloisa Rampinelli, Leandro Magalhães de Souza, Maria Angélica A M Guimarães
Pemphigus foliaceus (PF) is an autoimmune disease characterized by blistering of the skin. Infections caused by members of the herpesviridae family have been suggested as a possible triggering factor for pemphigus vulgaris (PV), but not for PF. The present study aimed to investigate the presence of Human herpesvirus (types 1, 2, 3) in corticosteroid refractory skin lesions from a patient with PF, by a Polymerase chain reaction (PCR) assay. The sample collected from cutaneous blisters has tested positive for herpes simplex virus type 1 (HSV1) after sequence analysis of the amplified viral genomic segment...
2017: Revista do Instituto de Medicina Tropical de São Paulo
https://www.readbyqxmd.com/read/28791608/achievement-of-the-longest-survival-of-paraneoplastic-pemphigus-with-bronchiolitis-obliterans-associated-with-follicular-lymphoma-using-r-chop-chemotherapy
#7
Shin Lee, Takahiro Yamauchi, Norito Ishii, Takashi Hashimoto, Keiichi Kinoshita, Shin Imamura, Kenichi Kamiya
Paraneoplastic pemphigus (PNP) is a rare, fatal, paraneoplastic autoimmune mucocutaneous blistering disease, commonly associated with lymphoproliferative disorders, including malignant lymphomas. Lymphoproliferative disorders associated with PNP are sometimes associated with a serious lung complication, bronchiolitis obliterans (BO). Due to its rarity, guidelines for the management of PNP have not been established. Furthermore, most patients die within 1 year. Here we report the successful treatment of lymphoma-associated PNP and BO using R-CHOP chemotherapy...
August 8, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28771827/glucocorticoids-the-mode-of-action-in-bullous-pemphigoid
#8
REVIEW
Minna E Kubin, Lars Hellberg, Riitta Palatsi
Bullous pemphigoid (BP) is the most common of pemphigoid diseases caused by autoantibodies against the structures of dermoepidermal junction followed by complement activation, innate immune cell infiltration, neutrophil proteinase secretion and subepidermal blister formation. The first line treatment of BP is topical and systemic glucocorticoids (GC). Regulation of the immune system and inflammatory cells is the main target of GC actions. GCs act through genomic and non-genomic mechanisms. The human glucocorticoid receptor (GR) mediates most of the biologic effects of glucocorticoids: cytosolic GR binds GCs and is capable to bind to glucocorticoid response elements in DNA and either transactivate or transrepress genes depending on the tissue and cell-type...
August 3, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28770240/survey-symptomatology-transmission-host-range-and-characterization-of-begomovirus-associated-with-yellow-mosaic-disease-of-ridge-gourd-in-southern-india
#9
Chandrakant V Patil, S V Ramdas, U Premchand, K S Shankarappa
Ridge gourd is an important vegetable crop and is affected by several biotic and abiotic factors. Among the different biotic factors, ridge gourd yellow mosaic disease (RgYMD) is new emerging threat for the production of ridge gourd. The incidence of the RgYMD varied from 30 to 100% in southern India with highest disease incidence of 100% observed in Belagavi district of Karnataka state. The infected plants showed chlorosis, mosaic, cupping of leaves, blistering, reduction in leaf size and stunted growth. The varieties/hybrids grown in the farmer's fields were found to be susceptible to the disease...
June 2017: Virusdisease
https://www.readbyqxmd.com/read/28770006/categorisation-of-foot-complaints-in-systemic-lupus-erythematosus-sle-from-a-new-zealand-cohort
#10
Simon J Otter, Maheswaran Rohan, Kevin A Davies, Sunil Kumar, Peter Gow, Nicola Dalbeth, Michael Corkill, Sam Panthakalam, Keith Rome
BACKGROUND: Foot complaints have been shown to be common in systemic lupus erythematosus (SLE) and heterogeneous in nature. We aimed to categorize self-reported foot complaints in people with SLE and foot symptoms. METHODS: A self-administered validated questionnaire was posted to 406 people with SLE attending adult rheumatology clinics across three health boards in Auckland, New Zealand. In addition to foot pain, vascular complaints, dermatological lesions and neurological symptoms were included in the analysis...
2017: Journal of Foot and Ankle Research
https://www.readbyqxmd.com/read/28768313/treatment-of-hailey-hailey-disease-with-low-dose-naltrexone
#11
Lauren N Albers, Jack L Arbiser, Ron J Feldman
Importance: Hailey-Hailey disease is a severe genetic blistering disease of intertriginous skin locations that can lead to poor quality of life and increased morbidities. Multiple therapies are available with inconsistent outcomes and potentially severe adverse effects. Objective: To determine whether low-dose naltrexone is an effective treatment for Hailey-Hailey disease. Design, Setting, and Participants: This study was a case series performed at a dermatology outpatient clinic of 3 patients with severe Hailey-Hailey disease recalcitrant to at least 4 therapies...
August 2, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28765827/traceless-targeting-and-isolation-of-gene-edited-immortalized-keratinocytes-from-epidermolysis-bullosa-simplex-patients
#12
Magomet Aushev, Ulrich Koller, Claudio Mussolino, Toni Cathomen, Julia Reichelt
Epidermolysis bullosa simplex (EBS) is a blistering skin disease caused by dominant-negative mutations in either KRT5 or KRT14, resulting in impairment of keratin filament structure and epidermal fragility. Currently, nearly 200 mutations distributed across the entire length of these genes are known to cause EBS. Genome editing using programmable nucleases enables the development of ex vivo gene therapies for dominant-negative genetic diseases. A clinically feasible strategy involves the disruption of the mutant allele while leaving the wild-type allele unaffected...
September 15, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28765124/demographic-and-behavioural-risk-factors-associated-with-trichomonas-vaginalis-among-south-african-hiv-positive-men-with-genital-ulcer-disease-a-cross-sectional-study
#13
Iddrisu Abdallah, Elizabeth Armstrong-Mensah, Ernest Alema-Mensah, Cheryl Jones
OBJECTIVES: Demographic and risky sexual behaviours may increase the risk for Trichomonas vaginalis (TV) infection and, thus, enhance HIV transmission to uninfected partners. We assessed the demographic and behavioural risk factors associated with TV among South African HIV-positive men with genital ulcer disease. METHODS: We conducted a cross-sectional study with data from a randomised controlled trial conducted by the Centers for Disease Control and Prevention and the London School of Hygiene and Tropical Medicine...
August 1, 2017: BMJ Open
https://www.readbyqxmd.com/read/28761292/analysis-of-serum-cytokine-profile-in-pemphigus
#14
Sang Hee Lee, Won Jin Hong, Soo-Chan Kim
BACKGROUND: Pemphigus is a group of autoimmune blistering diseases affecting skin and mucous membranes. While pemphigus is an autoantibody mediated disease, the role of T cells and cytokines in the pathogenesis is being increasingly recognized. OBJECTIVE: This study was conducted to observe alterations in the serum cytokine levels of patients with pemphigus vulgaris (PV), pemphigus foliaceous (PF), paraneoplastic pemphigus (PNP) and compare with bullous pemphigoid (BP) and healthy subjects...
August 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28747266/igg4-complement-and-the-mechanisms-of-blister-formation-in-pemphigus-and-bullous-pemphigoid
#15
REVIEW
Teruki Dainichi, Zachary Chow, Kenji Kabashima
Autoimmune bullous diseases are at the forefront of the research field on autoimmune diseases. Pemphigus and pemphigoid were historical entities in the world of descriptive dermatology for a long time. Recently, however, dermatologists and skin biologists have elegantly explained the novel pathomechanism of pemphigus and pemphigoid diseases. IgG4 is the major subclass of autoantibodies in autoimmune bullous diseases and is known to have little activity to activate complement. It is quite acceptable for pemphigus, because acantholysis in pemphigus has been demonstrated to be complement-independent...
July 18, 2017: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28736835/peptide-mimetics-of-immunoglobulin-a-iga-and-fc%C3%AE-ri-block-iga-induced-human-neutrophil-activation-and-migration
#16
Marieke H Heineke, Lydia P E van der Steen, Rianne M Korthouwer, J Joris Hage, Johannes P M Langedijk, Joris J Benschop, Jantine E Bakema, Jerry W Slootstra, Marjolein van Egmond
The cross-linking of the IgA Fc receptor (FcαRI) by IgA induces release of the chemoattractant LTB4, thereby recruiting neutrophils in a positive feedback loop. IgA autoantibodies of patients with autoimmune blistering skin diseases therefore induce massive recruitment of neutrophils, resulting in severe tissue damage. To interfere with neutrophil mobilization and reduce disease morbidity, we developed a panel of specific peptides mimicking either IgA or FcαRI sequences. CLIPS technology was used to stabilize three-dimensional structures and to increase peptides' half-life...
July 24, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28726005/the-role-of-sleep-in-pemphigus-a-review-of-mechanisms-and-perspectives
#17
Matheus Negrao Pedroni, Camila Hirotsu, Adriana Maria Porro, Sergio Tufik, Monica Levy Andersen
Pemphigus is an autoimmune bullous disease that causes the development of blisters and erosions on the skin and/or mucosa. Its inflammatory process is mediated by cytokines, which interact with sleep in a bidirectional manner. Pain, a frequent symptom due to pemphigus lesions, is well known to impair sleep quality. Depression is also associated with pemphigus and pro-inflammatory cytokines and may impair sleep. Additionally, a common relationship among other dermatological diseases and sleep has increasingly been described...
July 19, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28718937/wound-healing-in-epidermolysis-bullosa
#18
L B Tuderman, J E Mellerio
Epidermolysis bullosa (EB) is a group of inherited diseases characterised by recurrent skin blistering due to impaired epidermal or dermo-epidermal adhesion. In some subtypes, damage to internal organs causes serious comorbidities, and an increased risk of early and aggressive squamous cell carcinoma (SCC) characterizes recessive dystrophic EB (RDEB). It is likely that EB will only be cured by mutation-correction interventions, but until that goal becomes a reality we need to pool and extend knowledge about wound management and other strategies to improve quality of life for people with EB...
July 18, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28711083/bedside-diagnostics-in-dermatology-parasitic-and-noninfectious-diseases
#19
REVIEW
Robert G Micheletti, Arturo R Dominguez, Karolyn A Wanat
In addition to aiding the diagnosis of viral, bacterial, and fungal diseases, mineral oil preparation, Tzanck smear, and other techniques can be used to diagnose parasitic infections, neonatal pustular dermatoses, blistering diseases, Stevens-Johnson syndrome, and a plethora of other benign and malignant conditions, including granulomatous diseases and tumors. In many cases, these techniques are specific, reliable, and easy to perform and interpret. In others, a certain amount of training and expertise are required...
August 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28697212/maxillary-implant-prosthodontic-treatment-using-digital-laboratory-protocol-for-a-patient-with-epidermolysis-bullosa-a-case-history-report
#20
Rubén Agustín-Panadero, Blanca Serra-Pastor, David Peñarrocha-Oltra, Miguel Peñarrocha-Diago
Epidermolysis bullosa belongs to a group of genetic diseases that present with skin disorders and is characterized by generalized blister formation in response to mechanical trauma. This article reports on the management of a recessive dystrophic epidermolytic patient with four remaining periodontally compromised maxillary teeth. Treatment involved placement of four maxillary implants and use of computer-aided design/computer-assisted manufacture techniques to fabricate a fixed full-arch implant-supported prosthesis...
July 2017: International Journal of Prosthodontics
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