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https://www.readbyqxmd.com/read/27917914/t-cells-mediate-autoantibody-induced-cutaneous-inflammation-and-blistering-in-epidermolysis-bullosa-acquisita
#1
Katja Bieber, Mareike Witte, Shijie Sun, Jennifer E Hundt, Kathrin Kalies, Sören Dräger, Anika Kasprick, Trix Twelkmeyer, Rudolf A Manz, Peter König, Jörg Köhl, Detlef Zillikens, Ralf J Ludwig
T cells are key players in autoimmune diseases by supporting the production of autoantibodies. However, their contribution to the effector phase of antibody-mediated autoimmune dermatoses, i.e., tissue injury and inflammation of the skin, has not been investigated. In this paper, we demonstrate that T cells amplify the development of autoantibody-induced tissue injury in a prototypical, organ-specific autoimmune disease, namely epidermolysis bullosa acquisita (EBA) - characterized and caused by autoantibodies targeting type VII collagen...
December 5, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27909641/a-case-series-of-complex-recalcitrant-wounds-treated-with-epidermal-grafts-harvested-from-an-automated-device
#2
Stephen S Cai, Arvind U Gowda, Karan Chopra, Rachel Waldman, Ronald P Silverman, Yvonne M Rasko
INTRODUCTION: Epidermal grafting has several advantages over full-thickness or split-thickness grafts in the treatment of complex non-healing wounds. These include the low risk of donor site complications, minimal patient discomfort, and abstention from the operating room. Traditionally, the lack of reliable epidermal harvesting techniques has limited its clinical utilization. The development of an automated suction blister epidermal graft (SBEG) harvesting device may facilitate clinical utilization of this technique...
October 30, 2016: Curēus
https://www.readbyqxmd.com/read/27904583/the-role-of-intravenous-immunoglobulin-in-treatment-of-mucous-membrane-pemphigoid-a-review-of-literature
#3
REVIEW
Soheil Tavakolpour
BACKGROUND: Mucous membrane pemphigoid (MMP) is considered an autoimmune blistering disease that predominantly affects mucous membranes. Various treatments are available for controlling the diseases, but not all of them may respond. MATERIALS AND METHODS: PubMed and Google Scholar were searched for all the associated studies until 2015, using the keywords such as "cicatricial pemphigoid" or "ocular pemphigoid" or "mucous membrane pemphigoid" or "MMP" and "intravenous immunoglobulin" or "IVIg" to find all the relevant studies...
2016: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/27902996/pemphigus-pathogenesis-to-treatment
#4
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
https://www.readbyqxmd.com/read/27901296/novel-forest-decline-triggered-by-multiple-interactions-among-climate-an-introduced-pathogen-and-bark-beetles
#5
Carmen M Wong, Lori D Daniels
Novel forest decline is increasing due to global environmental change, yet the causal factors and their interactions remain poorly understood. Using tree ring analyses, we show how climate and multiple biotic factors caused the decline of whitebark pine (Pinus albicaulis) in 16 stands in the southern Canadian Rockies. In our study area, 72% of whitebark pines were dead and 18% had partially dead crowns. Tree mortality peaked in the 1970s; however, the annual basal area increment of disturbed trees began to decline significantly in the late 1940s...
November 5, 2016: Global Change Biology
https://www.readbyqxmd.com/read/27899325/dystrophic-epidermolysis-bullosa-col7a1-mutation-landscape-in-a-multi-ethnic-cohort-of-152-extended-families-with-high-degree-of-customary-consanguineous-marriages
#6
Hassan Vahidnezhad, Leila Youssefian, Sirous Zeinali, Amir Hossein Saeidian, Soheila Sotudeh, Nikoo Mozafari, Maryam Abiri, Abdolmohammad Kajbafzadeh, Mohammadreza Barzegar, Adam Ertel, Paolo Fortina, Jouni Uitto
Dystrophic epidermolysis bullosa (DEB) is a heritable skin disease manifesting with sub-lamina densa blistering, erosions and chronic ulcers. COL7A1, encoding type VII collagen, has been identified as the candidate gene for DEB. In this study, we have identified COL7A1 mutations in a large multi-ethnic cohort of 152 extended Iranian families with high degree of consanguinity. The patients were diagnosed by clinical manifestations, histopathology and immunoepitope mapping. Mutation detection consisted of a combination of a single nucleotide polymorphism-based whole genome homozygosity mapping, Sanger sequencing and gene targeted next generation sequencing...
October 27, 2016: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27893465/accelerated-resolution-of-inflammation-underlies-sex-differences-in-inflammatory-responses-in-humans
#7
Krishnaraj S Rathod, Vikas Kapil, Shanti Velmurugan, Rayomand S Khambata, Umme Siddique, Saima Khan, Sven Van Eijl, Lorna C Gee, Jascharanpreet Bansal, Kavi Pitrola, Christopher Shaw, Fulvio D'Acquisto, Romain A Colas, Federica Marelli-Berg, Jesmond Dalli, Amrita Ahluwalia
BACKGROUND: Cardiovascular disease occurs at lower incidence in premenopausal females compared with age-matched males. This variation may be linked to sex differences in inflammation. We prospectively investigated whether inflammation and components of the inflammatory response are altered in females compared with males. METHODS: We performed 2 clinical studies in healthy volunteers. In 12 men and 12 women, we assessed systemic inflammatory markers and vascular function using brachial artery flow-mediated dilation (FMD)...
November 28, 2016: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27878870/epidermolysis-bullosa-simplex-in-sibling-eurasier-dogs-is-caused-by-a-plec-non-sense-variant
#8
Elizabeth A Mauldin, Ping Wang, Thierry Olivry, Paula S Henthorn, Margret L Casal
BACKGROUND: Plectin, a large linker protein found in many tissues, acts to connect components of the cytoskeleton to each other. In the epidermis, plectin binds keratin intermediate filaments to hemidesmosomes. A deficiency of plectin in the skin leads to blister formation in the basal layer and the disease epidermolysis bullosa simplex (EBS). HYPOTHESIS/OBJECTIVES: To describe a novel blistering disease that arose spontaneously in a litter of puppies. ANIMALS: Two female and one male 20-day-old Eurasier puppies, from a litter of six, were presented for evaluation of failure to thrive and then euthanized due to poor prognosis...
November 7, 2016: Veterinary Dermatology
https://www.readbyqxmd.com/read/27878477/paraneoplastic-pemphigus-and-autoimmune-blistering-diseases-associated-with-neoplasm-characteristics-diagnosis-associated-neoplasms-proposed-pathogenesis-treatment
#9
REVIEW
Saritha Kartan, Vivian Y Shi, Ashley K Clark, Lawrence S Chan
Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes is particularly helpful in cases of skin lesions presenting as the first sign of the malignancy, and the underlying malignancy can be treated in a timely manner. Autoimmune paraneoplastic and neoplasm-associated bullous skin syndromes are characterized by blister formation due to an autoimmune response to components of the epidermis or basement membrane in the context of a neoplasm...
November 22, 2016: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/27874879/bullous-pemphigoid-associated-with-a-lymphoepithelial-cyst-of-the-pancreas
#10
Preston W Chadwick, Francis R Spitz, Daniel M Kwa, Waine C Johnson, Warren R Heymann
Bullous pemphigoid (BP) is an acquired, autoimmune, subepidermal blistering disorder. A possible paraneoplastic association has been suggested; however, debate remains regarding the precise relationship of these neoplasms with BP. We present a case of recalcitrant BP in a 67-year-old man with a pancreatic neoplasm that was found to be a lymphoepithelial cyst. Immunoperoxidase staining of the cyst demonstrated C3d along the basement membrane of the stratified squamous epithelium, suggesting that the BP may have involved the lymphoepithelial cyst itself...
October 2016: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/27871350/early-description-of-diet-induced-blistering-skin-diseases-in-medieval-persia-avicenna-s-point-of-view
#11
Fatemeh Atarzadeh, Babak Daneshfard, Ladan Dastgheib, Amir-Mohammad Jaladat, Gholamreza Amin
Pemphigus is an autoimmune blistering skin disease that is strongly associated with different environmental factors. Among these, nutritional factors are considered to trigger pemphigus; however, their role may be underestimated. Investigated more recently in conventional medicine, this causative bond between dietary factors and blistering skin diseases was mentioned by Persian scholars such as Avicenna a thousand years ago. Avicenna, a well-known Persian physician and philosopher, who could be considered a pioneer in dermatology, discussed skin diseases in a chapter in The Canon of Medicine...
2016: Skinmed
https://www.readbyqxmd.com/read/27869434/-childhood-pemphigus-vulgaris-a-case-report
#12
Ana G Bellelli, Natalia M Mantero, María L Rueda, Daniel Navacchia, Gabriel Cao, Leonardo De Lillo, Susana A Grees
Pemphigus vulgaris is a chronic disease that is characterized by flaccid blisters and erosions, caused by immunoglobulin G autoantibodies against epidermal components. It is highly unusual in the pediatric population; it represents the 1.4 - 2.9% of the total cases. We present a case of pemphigus vulgaris in a 12 year old girl, which has implied a challenge both in its diagnosis and in its treatment.
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27868136/increased-activity-and-apoptosis-of-eosinophils-in-blister-fluids-skin-and-peripheral-blood-of-patients-with-bullous-pemphigoid
#13
Judith Engmann, Urda Rüdrich, Georg Behrens, Eleni Papakonstantinou, Manuela Gehring, Alexander Kapp, Ulrike Raap
Bullous pemphigoid (BP) is an autoimmune blistering skin disease that is more common in elderly individuals. The aim of this study was to determine the functional activity of eosinophils in patients with BP compared with healthy donors. Blood, skin and blister-derived eosinophils were strongly activated in patients with BP, seen by increased surface expression of CD69 compared with controls. CD11b was also increased in BP blood eosinophils, which may explain the striking accumulation of eosinophils in BP (1×10-6 per ml blister fluid)...
November 21, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/27855302/the-possible-role-of-interleukin-35-and-its-therapeutic-potential-in-pemphigus
#14
REVIEW
Soheil Tavakolpour, Forough Kheiry, Hajar Sadat Mirsafaei, Meisam Akhlaghdoust
Pemphigus is an autoimmune disease that causes blistering and is life-threatening if left untreated. Nowadays, finding a promising treatment for pemphigus remains a serious challenge. Various treatments are currently recommended to treat this disease, but they rarely lead to complete and durable remission. Regulatory cells appear to have a critical role in numerous autoimmune diseases, so it is possible that promotion of these cells may induce remission. This study presents a new approach to treating pemphigus that has not been discussed to date...
November 14, 2016: International Immunopharmacology
https://www.readbyqxmd.com/read/27854192/blistering-disease-in-viewpoint-of-avicenna
#15
Fatemeh Atarzadeh, Majid Nimrouzi, Gholamreza Amin
No abstract text is available yet for this article.
November 2016: Journal of Integrative Medicine
https://www.readbyqxmd.com/read/27852057/novel-mutations-c-28g-t-p-ala10ser-and-c-189g-t-p-glu63asp-in-wdr62-associated-with-early-onset-acanthosis-and-hyperkeratosis-in-a-patient-with-autosomal-recessive-microcephaly-type-2
#16
Santasree Banerjee, Huishuang Chen, Hui Huang, Jing Wu, Zhiyun Yang, Weiping Deng, Dongna Chen, Jianlian Deng, Yan Su, Yang Li, Chao Wu, Ye Wang, Hao Zeng, Yiming Wang, Xunhua Li
Microcephaly (MCPH) is a developmental disorder characterized by reduced brain size and intellectual disability. A proportion of microcephaly is caused by defects in a single gene. Microcephaly 2 (MCPH2) is one of the most frequent subtypes of MCPH.WD repeat-containing protein 62 gene (WDR62) is the most frequently mutated gene in MCPH2 patients. Phenotypes involving dermatological changes in MCPH2 have not been reported. We have identified and investigated a 5-year-old Chinese girl with markedly reduced brain size (86% of normal size), intellectual disability and psychomotor developmental delay...
November 10, 2016: Oncotarget
https://www.readbyqxmd.com/read/27845416/psychiatric-and-neurological-disorders-are-associated-with-bullous-pemphigoid-a-nationwide-finnish-care-register-study
#17
Anna-Kaisa Försti, Jari Jokelainen, Hanna Ansakorpi, Allan Seppänen, Kari Majamaa, Markku Timonen, Kaisa Tasanen
Bullous pemphigoid (BP) is an autoimmune blistering skin disease with increasing incidence. BP is associated with neurological disorders, but it has not been established, what subtypes of dementia and stroke are associated with BP, and what is the temporal relation between these diseases. Also, the association between BP and psychiatric disorders is controversial. We conducted a retrospective nationwide study, using the Finnish Care Register for Health Care diagnoses between 1987 and 2013. The study population of 4524 BP patients were compared with 66138 patients with basocellular carcinoma (BCC), neurological and psychiatric comorbid disorders were evaluated for both groups, and associations were estimated by Cox regression and logistic regression analyses...
November 15, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27829102/correlation-of-serum-levels-of-ige-autoantibodies-against-bp180-with-bullous-pemphigoid-disease-activity
#18
Nina van Beek, Nadine Lüttmann, Franziska Huebner, Andreas Recke, Ingolf Karl, Franziska Sophie Schulze, Detlef Zillikens, Enno Schmidt
Importance: Bullous pemphigoid (BP) is by far the most frequent autoimmune blistering disease. The presence of IgE autoantibodies against the transmembrane protein BP antigen 2 (BP180, type XVII collagen) has previously been reported in 22% to 100% of BP serum samples, and the pathogenic relevance of anti-BP180 IgE has been suggested in various experimental models and by the successful use of omalizumab in individual patients with BP. Objectives: To determine the rate of anti-BP180-reactive IgE in BP, to evaluate the diagnostic relevance of anti-BP180 IgE in BP, and to correlate anti-BP180 IgE with disease activity and the clinical phenotype of patients with BP...
November 9, 2016: JAMA Dermatology
https://www.readbyqxmd.com/read/27828632/quality-of-life-depression-anxiety-and-loneliness-in-patients-with-bullous-pemphigoid-a-case-control-study
#19
Anargyros Kouris, Eftychia Platsidaki, Christos Christodoulou, Kalliopi Armyra, Panagiota Korkoliakou, Christina Stefanaki, Revekka Tsatovidou, Dimitrios Rigopoulos, George Kontochristopoulos
Background: Bullous pemphigoid (BP) is a chronic, autoimmune blistering skin disease that affects patients' daily life and psychosocial well-being. Objective: The aim of the study was to evaluate the quality of life, anxiety, depression and loneliness in BP patients. Methods: Fifty-seven BP patients and fifty-seven healthy controls were recruited for the study. The quality of life of each patient was assessed using the Dermatology Life Quality Index (DLQI) scale...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27827380/carriers-with-functional-null-mutations-in-lama3-have-localized-enamel-abnormalities-due-to-haploinsufficiency
#20
Katarzyna B Gostyńska, Wing Yan Yuen, Anna Maria Gerdina Pasmooij, Cornelius Stellingsma, Hendri H Pas, Henny Lemmink, Marcel F Jonkman
The hereditary blistering disease junctional epidermolysis bullosa (JEB) is always accompanied by structural enamel abnormalities of primary and secondary dentition, characterized as amelogenesis imperfecta. Autosomal recessive mutations in LAMA3, LAMB3 and LAMC2 encoding the heterotrimer laminin 332 (LM-332) are among the genes causing JEB. While examining pedigrees of JEB patients with LAMA3 mutations, we observed that heterozygous carriers of functional null mutations displayed subtle enamel pitting in the absence of skin fragility or other JEB symptoms...
November 9, 2016: European Journal of Human Genetics: EJHG
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