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Pemphigus

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https://www.readbyqxmd.com/read/28211055/pemphigus-a-pathomechanism-of-acantholysis
#1
REVIEW
Masutaka Furue, Takafumi Kadono
Autoantibodies to the desmosomal proteins desmoglein 1 and 3 cause pemphigus foliaceus and pemphigus vulgaris, which are characterised by keratinocyte dissociation (acantholysis) and intraepidermal blister formation. The passive transfer of pathogenic anti-desmoglein antibodies induces blisters in mice in vivo and the loss of keratinocyte adhesion in vitro. The pathogenetic mechanisms of acantholysis due to anti-desmoglein autoantibodies are not fully understood. However, recent studies have revealed that signalling-dependent and signalling-independent pathways are operative in the loss of cell adhesion...
February 17, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/28211049/low-dose-rituximab-and-concurrent-adjuvant-therapy-for-pemphigus-protocol-and-single-centre-long-term-review-of-nine-patients
#2
Aaron J Robinson, Mi Vu, Gary A Unglik, George A Varigos, Laura Scardamaglia
Pemphigus is an autoimmune B-cell mediated blistering disease associated with significant morbidity and mortality. Rituximab has proven effective for the treatment of steroid-refractory pemphigus, although there is controversy over the optimum dosing protocol. Additionally, effective disease control often requires long-term immunosuppression, even in disease-free periods. We present a case series of a single-centre long-term follow up of nine patients with pemphigus, treated with two 500-mg doses of rituximab separated by 14 days along with concurrent adjuvant therapy...
February 17, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/28211027/the-inpatient-burden-of-autoimmune-blistering-disease-in-us-children-analysis-of-nationwide-inpatient-sample-data
#3
Ziyou Ren, Derek Y Hsu, Nanette B Silverberg, Jonathan I Silverberg
BACKGROUND: Little is known about the epidemiology of pediatric autoimmune blistering disorders (PAIBD). OBJECTIVE: We sought to determine the inpatient burden and comorbidities of PAIBD. METHODS: We analyzed data from the Nationwide Inpatient Sample from 2002 to 2012, which contained a representative 20% sample of all US hospitalizations. RESULTS: The most common PAIBD with a primary admission was pemphigus (8.0 per million), whereas the most common secondary diagnosis of PAIBD was dermatitis herpetiformis (DH; 9...
February 16, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28207020/localized-pemphigus-foliaceus
#4
Ashley Walker, Tracy Favreau
No abstract text is available yet for this article.
January 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28197992/study-of-the-association-between-human-leukocyte-antigens-hla-and-pemphigus-vulgaris-in-brazilian-patients
#5
Julio M Gil, Raimar Weber, Claudia B Rosales, Helcio Rodrigues, Luiz U Sennes, Jorge Kalil, Azis Chagury, Ivan D Miziara
BACKGROUND: Pemphigus vulgaris is a mucocutaneous blistering autoimmune disease that manifests as painful blisters or erosions on the skin and/or mucosal surfaces. IgG autoantibodies target desmoglein, playing a major role in disease pathogenesis. Genetic predisposal to pemphigus vulgaris, especially the HLA DR and DQ alleles, has been known since the 1980s. The unique constitution of the Brazilian population favors exploratory genetic studies. METHODS: The study group included 51 patients with a confirmed diagnosis of pemphigus vulgaris from a tertiary hospital in Sao Paulo city, Sao Paulo, southeast Brazil...
February 15, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28186358/adjuvant-rituximab-treatment-for-pemphigus-a-retrospective-study-of-45-patients-at-a-single-center-with-long-term-follow-up
#6
Tae Hyung Kim, Yuri Choi, Sang Eun Lee, Jung Min Lim, Soo-Chan Kim
To evaluate the long-term outcomes of rituximab in the treatment of pemphigus and the influence of disease duration and different dose of rituximab on the clinical response, 45 patients with refractory pemphigus treated with at least one cycle of two infusions of rituximab (375 mg/m(2) per infusion weekly) were retrospectively studied. All patients were followed up for more than 2 years. All patients achieved complete or partial remission within 8 months of the first cycle. Thirty-four (76%) patients relapsed at a median of 17 months...
February 10, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28184292/rituximab-therapy-in-pemphigus-and-other-autoantibody-mediated-diseases
#7
REVIEW
Nina A Ran, Aimee S Payne
Rituximab, a monoclonal antibody targeting the B cell marker CD20, was initially approved in 1997 by the United States Food and Drug Administration (FDA) for the treatment of non-Hodgkin lymphoma. Since that time, rituximab has been FDA-approved for rheumatoid arthritis and vasculitides, such as granulomatosis with polyangiitis and microscopic polyangiitis. Additionally, rituximab has been used off-label in the treatment of numerous other autoimmune diseases, with notable success in pemphigus, an autoantibody-mediated skin blistering disease...
2017: F1000Research
https://www.readbyqxmd.com/read/28174139/positive-correlation-of-anti-herpes-simplex-type-i-virus-antibody-levels-with-pemphigus-vulgaris-disease-status-and-activity-in-a-large-patient-cohort
#8
Philip Senger, Nadia Abidi, Diana M Lin, Kristina Seiffert-Sinha, Animesh A Sinha
It is well accepted that pemphigus vulgaris (PV) is genetically linked to specific HLA class II subtypes. Environmental factors, including the role of herpes simplex virus (HSV1) in disease manifestation, have also been implicated, but in a limited number of patients and with inconsistent results. To clarify an association between HSV1 and PV in a large data set, including a stratification by dynamic and static clinical parameters, including disease activity, therapy status, HLA association, and gender. Serum HSV1 IgG levels from PV patients and healthy controls were measured by ELISA...
February 3, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28164651/anti-desmoglein-1-and-3-autoantibody-levels-in-endemic-pemphigus-foliaceus-and-pemphigus-vulgaris-from-brazil
#9
Liana A Oliveira, Alfredo Marquart-Filho, Gerson Trevilato, Rodolfo P Timoteo, Maira Mukai, Ana M F Roselino, Maria L Petzl-Erler
BACKGROUND: Pemphigus is a group of autoimmune blistering diseases of which the major forms are pemphigus foliaceus (PF) and vulgaris (PV). In Brazil, PF occurs in an endemic form also known as fogo selvagem. The main autoantibody in PF is against desmoglein 1 (DSG1), while in PV the main antibody is anti-desmoglein 3 (DSG3), but often anti-DSG1 is also present. The aim of the present study was to analyze the levels of anti-DSG1 and antiDSG3 autoantibodies in Brazilian PF and PV patients, considering different stages of the disease for PF patients and comparing these levels to those of healthy individuals living in and outside the endemic regions...
July 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28158888/-dysphonia-as-a-symptom-of-a-laryngeal-involvement-in-pemphigus-vulgaris
#10
D Crisan, K Scharffetter-Kochanek, T K Hoffmann, S Brosch, R Reiter
No abstract text is available yet for this article.
January 2017: Laryngo- Rhino- Otologie
https://www.readbyqxmd.com/read/28129481/fatal-infection-of-pneumocystis-jiroveci-pneumonia-in-a-pemphigus-patient-treated-with-rituximab
#11
K C Wei, Y H Wang, W H Wang, W Chen
Rituximab is an effective treatment for pemphigus and can be considered as a first-line treatment for severe cases.(1-4) Rituximab increases the risk of infection with Pneumocystis jiroveci pneumonia (PJP), an opportunistic pathogen, especially in hematopoietic stem cell transplant and Wegener's granulomatosis, with a high associated mortality rate up to 30%.(5) The risk of PJP infection in pemphigus patients treated with rituximab has been underreported to date. This article is protected by copyright. All rights reserved...
January 27, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28127349/disseminated-strongyloidiasis-in-an-immunodeficient-patient-pemphigus-vulgaris-due-to-corticosteroid-therapy-a-case-report
#12
Mohsen Najjari, Mohammad Ebrahimipour, Amir Kaheh, Mohammadreza Karimazar
Strongyloidiasis is a frequent misdiagnosed parasitic infection in the world that caused by Strongyloides stercoralis. In Iran, the disease is predominantly reported from warm and humid climate provinces. The patient was a 54-yr-old man, originated from Khuzestan Province with a history of pemphigus and diabetes that was treated with high-dose of corticosteroid drugs before admission in a non-private hospital in Shiraz, Iran in 2014. After different primary diagnosis and administrating of several drugs, endoscopy and histopatholgical biopsy revealed a massive S...
July 2016: Iranian Journal of Parasitology
https://www.readbyqxmd.com/read/28115214/local-inflammation-exacerbates-cutaneous-manifestations-in-a-murine-autoimmune-pemphigus-model
#13
Sachiko Ono, Gyohei Egawa, Akihiko Kitoh, Teruki Dainichi, Atsushi Otsuka, Saeko Nakajima, Tetsuya Honda, Kenji Kabashima
Local inflammation enhances autoantibody deposition in the skin and exacerbates murine pemphigus manifestations as a representative of autoantibody-mediated disorders, therefore prevention of excessive external stimulants might be important for the disease management.
January 20, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28108048/bullous-pseudobullous-pustular-dermatoses
#14
Mark R Wick
Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Examples of such disorders include impetigo, Herpes virus infections, pemphigus, bullous pemphigoid and pemphigoid gestationis, epidermolysis bullosa acquisita, IgA-related dermatoses, inherited epidermolysis bullosa variants, Hailey-Hailey disease, and porphyria cutanea tarda...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28093721/application-of-autologous-hematopoietic-stem-cell-transplantation-for-pemphigus
#15
Menglei Wang, Can Cao, Jing Sun, Xuebiao Peng, Qifa Liu, Liang Huang, Yanyan Chai, Kuan Lai, Pingjiao Chen, Qingxiu Liu, Qian Li, Yusheng Peng, Hao Xiong, Jing Zhang, Minghua Chen, Kang Zeng
BACKGROUND: Pemphigus is a rare and fatal autoimmune disease for which the treatment options are limited. This study aimed to evaluate the efficacy of autologous peripheral hematopoietic stem cell transplantation (APHSCT) for pemphigus. METHODS: We conducted APHSCT for 12 pemphigus patients (seven males and five females, mean age 23.8 years) with life-threatening complications or who responded poorly to conventional therapy. Peripheral blood stem cells were mobilized with cyclophosphamide, granulocyte colony-stimulating factor, and rituximab, and purified autologous CD34(+) stem cells were infused...
March 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28093595/mortality-and-cause-of-death-in-israeli-patients-with-pemphigus
#16
Khalaf Kridin, Shira Zelber Sagi, Reuven Bergman
All-cause and cause-specific mortality among patients with pemphigus compared with the general population is yet to be established. This study investigated overall mortality and cause-specific mortality in a large immunopathologically validated cohort of patients with pemphigus. Mortality of patients with pemphigus was compared with age- and gender-matched control subjects in the general population. All-cause and cause-specific standardized mortality ratios (SMRs) were estimated. The study cohort included 245 patients newly-diagnosed with pemphigus between January 1990 and June 2016...
January 17, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28089115/correlation-of-antimuscarinic-acetylcholine-receptor-antibody-titers-and-antidesmoglein-antibody-titers-with-the-severity-of-disease-in-patients-with-pemphigus
#17
Manimegalai Jeyasekaran Dhanabhakya Lakshmi, Telanseri Jaykar Jaisankar, Medha Rajappa, Devinder Mohan Thappa, Laxmisha Chandrashekar, Dakshinamurthy Divyapriya, Malathi Munisamy, Gunaseelan Revathy
BACKGROUND: Acetylcholine receptor (AchR) antibody levels significantly correlate with disease severity at initial pemphigus diagnosis and during follow-up. However, it is not clear if they are just an epiphenomenon or a potential trigger of the known pathogenic process in pemphigus vulgaris. OBJECTIVE: We sought to assess the changes in anti-muscarinic (M3) AchR and anti-desmoglein (Dsg) antibody titers with therapy. METHODS: This was a hospital-based cohort study involving 45 patients with active pemphigus...
January 11, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28079925/pregnancy-outcome-after-rituximab-treatment-before-conception-in-patients-affected-by-severe-pemphigus-vulgaris-superficialis
#18
C Vassallo, S Grassi, E Tagliabue, A Piccolo, V Brazzelli
Pregnancy is rare among patients affected by pemphigus, especially in European countries, mainly because of older patients, lower fertility rates and possible teratogenic risk.(1) Avoidance of immunosuppressive medications during pregnancy has been recommended, but inadequate treatment of pemphigus may increase morbidity and mortality.(2) Corticosteroids remain the first-line agent for treatment of pemphigus in pregnancy when low dosages are sufficient for disease control; however, prednisone and its metabolites crosses the placenta barrier, and high levels in the serum have been associated with adverse fetal outcomes...
January 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28064226/salivary-samples-for-the-diagnosis-of-pemphigus-vulgaris-using-the-biochip-approach-a-pilot-study
#19
Irene Russo, Andrea Saponeri, Anna Michelotto, Mauro Alaibac
Pemphigus vulgaris (PV) is a rare autoimmune intraepithelial blistering skin disease characterized by the presence of circulating autoantibodies against desmoglein 3 (DSG3) and desmoglein 1 (DSG1), resulting in loss of the normal epithelial cell-to-cell adhesion, through a process called acantholysis. In recent years, a BIOCHIP-based indirect immunofluorescence technique for the determination of anti-DSG3 and anti-DSG1 autoantibodies has been described. Even though, the use of saliva anti-DSG3 and anti-DSG1 ELISA for the diagnosis of PV has been already reported, there are no studies concerning the utilization of saliva by the BIOCHIP approach...
2, 2017: In Vivo
https://www.readbyqxmd.com/read/28052410/can-immunohistochemistry-replace-immunofluorescence-in-diagnosis-of-skin-bullous-diseases
#20
COMPARATIVE STUDY
Hanan Al-Saeid Al-Shenawy
Autoimmune bullous diseases are distressing and sometimes risky bullous dermatoses characterized by the presence of antibodies focused against disease-specific target antigens. Recognition of these antibodies using immunofluorescence is used to be the only sure diagnostic method after reviewing the routine histopathological section. Because of many causes that make the using of immunofluorescence difficult, we tried to evaluate the role of immunohistochemistry in diagnosis of these bullous skin diseases; 40 pemphigus cases (30 pemphigus vulgaris and 10 pemphigus foliaceus) and 37 non-pemphigus cases (35 vesiculobullous skin diseases and 2 normal skin)...
February 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
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