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https://www.readbyqxmd.com/read/28338219/human-orf-complicated-by-epidermolysis-bullosa-acquisita
#1
E Zuelgaray, C Salle de Chou, J Gottlieb, M Battistella, M D Vignon-Pennamen, M Bagot, F Guibal, J D Bouaziz
Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after Orf infection including erythema multiforme. A few cases of auto-immune bullous dermatosis complicating Orf disease have been reported to date, usually characterized by tense blisters eruptions with or without mucosal involvement, linear deposition of C3, IgG and/or IgA along the basement membrane and negativity of indirect immunofluorescence analysis and ELISA assays (performed in 4 of 11 reported cases) against target antigens of bullous pemphigoid, mucous membrane pemphigoid or epidermolysis bullosa acquisita, except one case of mucosal pemphigoid with antilaminin-332 antibodies...
March 24, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28322362/immunopathogenic-oral-diseases-%C3%A2-an-overview-focusing-on-pemphigus-vulgaris-and-mucous-membrane-pemphigoid
#2
Liviu Feller, Raoul Ballyram, Razia Ag Khammissa, Mario Altini, Johan Lemmer
Pemphigus vulgaris, mucosal pemphigoid (mucous membrane pemphigoid), lichen planus, discoid lupus erythematosus and erythema multiforme are a group of immune-mediated mucocutaneous disorders characterised clinically by the formation of blisters, erosions or ulcers. The oral mucosa is often affected, and sometimes the disease is limited to the mouth. The target antigens, autoreactive immune responses, microscopic features, treatment and prognosis vary from one disease to the other. Treatment aims to eliminate exogenous risk factors, suppress the pathogenic immuno-inflammatory reactions, promote healing and prevent infection...
March 20, 2017: Oral Health & Preventive Dentistry
https://www.readbyqxmd.com/read/28321838/porphyria-cutanea-tarda-an-intriguing-genetic-disease-and-marker
#3
REVIEW
Nancy S Handler, Marc Z Handler, Matthew P Stephany, Glenn A Handler, Robert A Schwartz
Porphyrias are a group of intriguing genetic diseases of the heme pathway, of which porphyria cutanea tarda (PCT) is the most common. Resulting from a defect in enzymes in the porphyria pathway, PCT has been linked to several conditions. Recent studies have demonstrated a change in thinking regarding the human immunodeficiency virus (HIV) and development of PCT. The exacerbation of PCT with contraction of HIV is now believed to result from coinfection from the hepatitis C virus (HCV). Blistering of sun-exposed skin, a classic presenting sign of PCT, is not exclusive to the condition...
March 21, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28317060/prognostic-factors-for-mortality-in-patients-with-bullous-pemphigoid-a-meta-analysis
#4
Yi-Di Liu, Yan-Hong Wang, Yi-Cong Ye, Wen-Ling Zhao, Li Li
Bullous pemphigoid (BP) is a chronic debilitating autoimmune blistering disease that frequently occurs in the elderly population. Previous studies have suggested a high morbidity and mortality associated with BP. However, relatively few studies have investigated prognostic factors of BP mortality, and they showed considerably various results. This meta-analysis aimed to quantitatively assess the association between several potential prognostic factors and risk of mortality in bullous pemphigoid. A comprehensive search was performed using Pubmed, Embase, and Cochrane Library...
March 19, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28303283/-bullous-sweet-s-syndrome-with-pulmonary-involvement
#5
A Sesti, K Rappersberger, C Posch
Acute febrile neutrophilic dermatosis (Sweet's syndrome) is a rare dermatosis characterized by painful papules and plaques accompanied by cutaneous infiltration with neutrophilic granulocytes. Bullous changes are observed in some cases. We report about a patient with osteomyelofibrosis who developed fever accompanied by painful plaques and confluent papules on both arms and thighs. The course of the disease was complicated by blistering and pulmonary infiltrates. After the diagnosis of bullous Sweet's syndrome was established, systemic therapy with glucocorticoids was successful in treating skin lesions and dyspnea...
March 16, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28300888/vancomycin-associated-linear-iga-disease-mimicking-toxic-epidermal-necrolysis
#6
Amanda Regio Pereira, Luis Henrique Barbizan de Moura, Jhonatan Rafael Siqueira Pinheiro, Victor Pavan Pasin, Milvia Maria Simões E Silva Enokihara, Adriana Maria Porro
Linear IgA dermatosis is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. In the last three decades, many different drugs have been associated with the drug-induced form of the disease, especially vancomycin. We report a case of vancomycin-induced linear IgA disease mimicking toxic epidermal necrolysis. The aim of this work is to emphasize the need to include this differential diagnosis in cases of epidermal detachment and to review the literature on the subject and this specific clinical presentation...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28300855/longitudinal-tracking-of-autoantibody-levels-in-a-pemphigus-vulgaris-patient-support-for-a-role-of-anti-desmoglein-1-autoantibodies-as-predictors-of-disease-progression
#7
Nadia Y Abidi, Irene Lainiotis, Gretchen Malikowski, Kristina Seiffert-Sinha, Animesh A Sinha
Anti-desmoglein (Dsg) 1 and -Dsg3 antibody titers have an established role in the diagnosis of the autoimmune blistering skin disease pemphigus vulgaris (PV). However, their usefulness for disease monitoring has been controversial. A recent large-scale immunoprofiling study by our group indicated that anti-Dsg1 levels may be a better predictor of disease activity than anti-Dsg3 levels, with declining levels predicting progression from active phase of disease to early remission, irrespective of lesional subtypes...
February 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28298019/microsurgical-versus-endovascular-interventions-for-blood-blister-aneurysms-of-the-internal-carotid-artery-systematic-review-of-literature-and-meta-analysis-on-safety-and-efficacy
#8
Sumedh S Shah, Zachary C Gersey, Mohamed Nuh, Hesham T Ghonim, Mohamed Samy Elhammady, Eric C Peterson
OBJECTIVE Blood-blister aneurysms (BBAs) of the internal carotid artery (ICA) have a poor natural history associated with high morbidity and mortality. Currently, both surgical and endovascular techniques are employed to treat BBAs; thus, the authors sought to perform a meta-analysis to compare the efficacy and safety of these approaches. METHODS A literature search of PubMed, MEDLINE, and Google Scholar online databases was performed to include pertinent English-language studies from 2005 to 2015 that discussed the efficacy and safety of either surgical or endovascular therapies to treat BBAs...
February 17, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28295451/ccl20-neutralisation-by-a-monoclonal-antibody-in-healthy-subjects-selectively-inhibits-recruitment-of-ccr6-cells-in-an-experimental-suction-blister
#9
Gerben Bouma, Stefano Zamuner, Kirsty Hicks, Andrew Want, João Oliveira, Arpita Choudhury, Sara Brett, Darren Robertson, Leigh Felton, Virginia Norris, Disala Fernando, Michael Herdman, Ruth Tarzi
AIMS: GSK3050002, a humanized IgG1κ antibody with high binding affinity to human CCL20, was administered in a first-in-human study to evaluate safety, pharmacokinetics (PK) and pharmacodynamics (PD). An experimental skin suction blister model was employed to assess target engagement and the ability of the compound to inhibit recruitment of inflammatory CCR6 expressing cells. METHODS: This study was a randomized, double-blind (sponsor open), placebo-controlled, single-centre, single ascending intravenous dose escalation trial in 48 healthy male volunteers...
March 11, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28279484/doxycycline-versus-prednisolone-as-an-initial-treatment-strategy-for-bullous-pemphigoid-a-pragmatic-non-inferiority-randomised-controlled-trial
#10
Hywel C Williams, Fenella Wojnarowska, Gudula Kirtschig, James Mason, Thomas R Godec, Enno Schmidt, Joanne R Chalmers, Margaret Childs, Shernaz Walton, Karen Harman, Anna Chapman, Diane Whitham, Andrew J Nunn
BACKGROUND: Bullous pemphigoid is a blistering skin disorder with increased mortality. We tested whether a strategy of starting treatment with doxycycline gives acceptable short-term blister control while conferring long-term safety advantages over starting treatment with oral corticosteroids. METHODS: We did a pragmatic, multicentre, parallel-group randomised controlled trial of adults with bullous pemphigoid (three or more blisters at two or more sites and linear basement membrane IgG or C3)...
March 6, 2017: Lancet
https://www.readbyqxmd.com/read/28274366/pemphigus-vulgaris-and-pemphigus-foliaceus-determined-by-cd86-and-ctla4-polymorphisms
#11
Srdjan Tanasilovic, Svetlana Popadic, Ljiljana Medenica, Dusan Popadic
Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are rare autoimmune blistering diseases with presumed T-cell-dependent pathology. Activation of naïve T cells is dependent on antigen recognition, subsequent signaling through the T-cell receptor complex (signal 1), and various other interactions of T cells with antigen presenting cells that may be collectively designated as signal 2, which is unconditionally required for T-cell activation both in response to infection and to autoantigens. Among the best described interactions contributing to signal 2 are those mediated by B7 family molecules, such as CD80 and CD86 with their ligands; CD28, providing activation signals; and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4), conferring inhibition...
March 2017: Clinics in Dermatology
https://www.readbyqxmd.com/read/28273946/dysregulation-of-mcd46-and-scd46-contribute-to-the-pathogenesis-of-bullous-pemphigoid
#12
Pei Qiao, Erle Dang, Tianyu Cao, Hui Fang, Jieyu Zhang, Hongjiang Qiao, Gang Wang
Bullous pemphigoid (BP) is an autoimmune bullous disease caused by autoantibodies against BP180 in the epidermal basement membrane. Autoantibody-mediated complement activation is an important process in BP pathogenesis. CD46, a crucial complement regulatory protein in the complement activation, has been reported to be involved in several autoimmune diseases. In the present study, we investigated whether CD46 plays a role in BP development. We found that sCD46 expression was significantly increased in the serum and blister fluids of BP patients and correlated with the levels of anti-BP180 NC16A antibody and C3a...
December 2017: Scientific Reports
https://www.readbyqxmd.com/read/28271571/gene-editing-for-skin-diseases-designer-nucleases-as-tools-for-gene-therapy-of-skin-fragility-disorders
#13
Oliver P March, Julia Reichelt, Ulrich Koller
The current treatment of inherited blistering skin diseases, such as epidermolysis bullosa (EB) is largely restricted to wound care and pain management. More effective therapeutic strategies are urgently required and targeting the genetic basis of these severe diseases is now within reach. Here we describe current gene editing tools and their potential to correct gene function in monogenetic blistering skin diseases. We present the features of the most frequently used gene editing techniques TALEN and CRISPR/Cas9, determining their preferential application under specific genetic conditions, including the type of mutational inheritance, the targeting site within the gene or the possibility to specifically target the mutation...
March 7, 2017: Experimental Physiology
https://www.readbyqxmd.com/read/28267595/economic-burden-of-herpes-zoster-culebrilla-in-latin-america
#14
Emmanouil Rampakakis, Clare Pollock, Claudia Vujacich, Joao Toniolo Neto, Alejandro Ortiz Covarrubias, Homero Monsanto, Kelly D Johnson
BACKGROUND: Herpes zoster (HZ) is characterized by debilitating pain and blistering dermatomal rash. The most common complication of HZ is postherpetic neuralgia (PHN), a persistent pain that can substantially affects patients' quality of life. HZ has significant impact on patients' lives with considerable implications for healthcare systems and society. The purpose of this study was to evaluate the healthcare resource utilization (HCRU) and medical costs associated with HZ in Latin America...
March 3, 2017: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/28261030/neurodegenerative-disorders-bullous-pemphigoid-and-psoriasis-a-comparative-study-in-ethnic-poles-indicates-that-parkinson-s-disease-is-more-relevant-to-bullous-pemphigoid
#15
Paweł Bartkiewicz, Justyna Gornowicz-Porowska, Paweł P Pietkiewicz, Anna Świrkowicz, Monika Bowszyc-Dmochowska, Marian Dmochowski
INTRODUCTION: Bullous pemphigoid (BP) is an autoimmune blistering dermatosis of the elderly with autoimmunity to hemidesmosomal proteins, BP180 and BP230, which are expressed also in neuronal tissue. AIM: The aim here was to retrospectively compare the prevalence of neurodegenerative disorders (ND), particularly Parkinson's disease (PD), unspecified conditions manifesting as dementia and stroke, in two groups of ethnic Poles, with BP and with psoriasis (Ps), in order to obtain data whether BP is more prone to coexist with ND than Ps in the elderly...
February 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28259440/suction-blistering-the-lesional-skin-of-vitiligo-patients-reveals-useful-biomarkers-of-disease-activity
#16
James P Strassner, Mehdi Rashighi, Maggi Ahmed Refat, Jillian M Richmond, John E Harris
BACKGROUND: Vitiligo is an autoimmune disease of the skin with limited treatment options; there is an urgent need to identify and validate biomarkers of disease activity to support vitiligo clinical studies. OBJECTIVE: To investigate potential biomarkers of disease activity directly in the skin of vitiligo subjects and healthy subjects. METHODS: Patient skin was sampled via a modified suction-blister technique, allowing for minimally invasive, objective assessment of cytokines and T-cell infiltrates in the interstitial skin fluid...
March 1, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28258944/caesarean-delivery-in-a-pregnant-woman-with-epidermolysis-bullosa-anaesthetic-challenges
#17
M Araújo, R Brás, R Frada, L Guedes-Martins, P Lemos
Epidermolysis bullosa is a heterogeneous group of hereditary diseases characterised by extreme fragility of skin and mucosa, with blister and lesion formation spontaneously or in response to trauma. Anaesthetic management of these patients is challenging with respect to positioning, monitoring, use of medical devices and airway management. These challenges are increased when managing labour. We report an elective caesarean delivery in a nulliparous woman with autosomal recessive dystrophic epidermolysis bullosa, managed successfully with spinal anaesthesia...
February 3, 2017: International Journal of Obstetric Anesthesia
https://www.readbyqxmd.com/read/28248822/mucous-membrane-pemphigoid-causing-central-airway-obstruction
#18
Bilal A Jalil, Yara G Abdou, Stephanie A Rosen, Tarek Dammad
Mucous membrane pemphigoid (MMP) is a rare variant of the skin disease pemphigoid, which predominantly involves the mucous membranes. This rare autoimmune disease that infrequently affects the respiratory tract is characterized by subepithelial blister formation that may result in scarring. Immunopathologic examination of mucous membranes reveals the deposition of immunoglobulins and complement within the subepithelial basement membrane. We describe a patient with undiagnosed MMP, with a near-fatal presentation of central airway obstruction causing acute respiratory distress...
February 28, 2017: Journal of Bronchology & Interventional Pulmonology
https://www.readbyqxmd.com/read/28247089/bullous-pemphigoid-a-review-of-its-diagnosis-associations-and-treatment
#19
REVIEW
Philippe Bernard, Frank Antonicelli
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries, and typically affects the elderly. BP is immunologically characterized by tissue-bound and circulating autoantibodies directed against either the BP antigen 180 (BP180, or BPAG2) or the BP antigen 230 (BP230, or BPAG1e), or even both, which are components of hemidesmosomes involved in the dermal-epidermal cohesion. Risk factors for BP include old age, neurologic diseases (dementia, Parkinson's disease, cerebrovascular disease), and some particular drugs, including loop diuretics, spironolactone and neuroleptics...
February 28, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28241331/gabapentin-induced-bullous-pemphigoid
#20
Avram Flamm, Sarina Sachdev, Francois Dufresne
Bullous pemphigoid is an autoimmune blistering dermatosis with separation of the epidermis from the dermis. This disease process is common among elderly patients and manifests with subepidermal vesicles and tense bullae. Patients with bullous pemphigoid are more likely to have also received a previous diagnosis of a neurologic disorder. Gabapentin is an antiepileptic that is used to manage neuropathic pain. The authors describe, to their knowledge, the first report of gabapentin-induced bullous pemphigoid in an elderly man with no history of rashes or reactions to other medications...
March 1, 2017: Journal of the American Osteopathic Association
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