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Pemphigus

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https://www.readbyqxmd.com/read/28543318/correlation-between-salivary-and-serum-anti-desmoglein-1-and-3-antibody-titres-using-elisa-and-between-anti-desmoglein-levels-and-disease-severity-in-pemphigus-vulgaris
#1
D De, G Khullar, S Handa, N Joshi, B Saikia, R W Minz
ELISA for anti-desmoglein antibodies (Dsg) is commonly used for diagnosis and assessment of treatment response in pemphigus vulgaris (PV). The present study was conducted to assess the relationship between salivary and serum Dsg1 and Dsg3 levels, and whether salivary Dsg1 and Dsg3 levels correlate with clinical disease severity of oral mucosal lesions in PV. In total 43, patients with PV with predominantly mucosal involvement were recruited. Both serum and salivary samples were collected from the cases, and salivary samples were also collected from five controls...
May 22, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28536732/pemphigus-vulgaris-and-pemphigus-foliaceus-differences-in-epidemiology-and-mortality
#2
Khalaf Kridin, Shira Zelber-Sagi, Reuven Bergman
Little is known about differences in epidemiological features and prognosis between pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The objective of this study was to compare PV and PF patients regarding ethnic variations and mortality rates. Mortality of PV and PF patients was compared with age- and gender-matched control subjects in the general population. The study cohort comprised 207 patients with PV and 30 with PF diagnosed during the period 2000 to 2015. The incidence rate of PV among Jews was 3...
May 24, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28536684/gastrointestinal-cytomegalovirus-disease-in-a-patient-with-pemphigus-vulgaris-treated-with-corticosteroid-and-mycophenolate-mofetil
#3
Luiza Barbosa Oliveira, Celina Wakisaka Maruta, Denise Miyamoto, Fernanda Aburesi Salvadori, Claudia Giuli Santi, Valeria Aoki, Amaro Nunes Duarte-Neto
Pemphigus vulgaris is an autoimmune disease characterized by the formation of suprabasal intra-epidermal blisters on the skin and mucosal surfaces. Infectious diseases are the main cause of death in patients with pemphigus due to the disrupture of the physiological skin barrier, immune dysregulation, and the use of immunosuppressive medications leaving the patient prone to acquire opportunistic infections. We report the case of a 67-year-old woman diagnosed with pemphigus vulgaris, who was irregularly taking prednisone and mycophenolate mofetil...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28534443/cd59-polymorphisms-are-associated-with-gene-expression-and-different-sexual-susceptibility-to-pemphigus-foliaceus
#4
Amanda Salviano-Silva, Maria Luiza Petzl-Erler, Angelica Beate Winter Boldt
Pemphigus foliaceus (PF) is an autoimmune disease, endemic in Brazilian rural areas, characterized by acantholysis and accompanied by complement activation, with generalized or localized distribution of painful epidermal blisters. CD59 is an essential complement regulator, inhibiting formation of the membrane attack complex, and mediating signal transduction and activation of T lymphocytes. CD59 has different transcripts by alternative splicing, of which only two are widely expressed, suggesting the presence of regulatory sites in their noncoding regions...
May 23, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28534318/association-between-inflammatory-skin-disease-and-cardiovascular-and-cerebrovascular-co-morbidities-in-us-adults-analysis-of-nationwide-inpatient-sample-data
#5
Michael C Kwa, Jonathan I Silverberg
BACKGROUND: Psoriasis, atopic dermatitis or eczema (AD-E), pemphigus, bullous pemphigoid (BP), and hidradenitis are chronic inflammatory skin disorders associated with systemic immune activation, considerable symptom burden, stigma, functional disturbances, and mental health symptoms. All of these might increase cardiovascular risk. OBJECTIVE: The objective of this study was to determine whether these inflammatory skin diseases are associated with increased cardiovascular/cerebrovascular risk and/or disease...
May 22, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28523900/pediatric-pemphigus-herpetiformis-case-report-and-review-of-the-literature
#6
Caitlin M Peterman, Sophie Vadeboncoeur, Birgitta A Schmidt, Stephen E Gellis
Pemphigus herpetiformis (PH) is a rare autoimmune bullous condition usually seen in adults and likely underrecognized in children. We describe a 2-year-old girl who presented with a chronic, generalized, blistering rash consistent with pediatric PH based on histology and immunohistochemistry and review the literature. Prognosis is generally more favorable in children than adults. Prednisone and dapsone should be considered as first-line agents to induce remission.
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28498563/identification-of-factors-associated-with-treatment-refractoriness-of-oral-lesions-in-pemphigus-vulgaris
#7
S Kumar, D De, S Handa, R K Ratho, S Bhandari, A Pal, P Kamboj, S Sarkar
BACKGROUND: Oral mucosal lesions of pemphigus vulgaris (PV) are known to show more treatment refractoriness than skin lesions. OBJECTIVES: To identify the role of different clinical and laboratory parameters in the treatment refractoriness in the oral lesions of PV. METHODS: This prospective study recruited 50 adult patients of PV having oral lesions. Patients were given treatment appropriate for overall disease severity. Treatment refractoriness was defined arbitrarily as less than 75% reduction in oral objective Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) after treatment for 6 months...
May 12, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28497856/case-of-shift-from-linear-immunoglobulin-a-bullous-dermatosis-to-pemphigus-herpetiformis-for-a-short-period-of-time
#8
Hiroshi Koga, Norito Ishii, Takashi Hashimoto, Takekuni Nakama
Pemphigus herpetiformis (PH) is a rare variant of pemphigus characterized by erythemas and vesicles, tending to present with annular-shaped lesions. Immunologically, immunoglobulin (Ig)G deposition at the keratinocyte cell surfaces is observed. Linear IgA bullous dermatosis (LABD) is a rare subepidermal blistering disease with linear IgA deposits at the epidermal basement membrane zone (BMZ). The annular-shaped skin lesions in PH mimic clinical manifestation of other autoimmune bullous diseases, including LABD, although PH and LABD have different immunological and histopathological features...
February 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28495498/venous-thromboembolism-in-patients-with-pemphigus-a-cohort-study
#9
Yael Anne Leshem, Lihi Atzmony, Israel Dudkiewicz, Emmilia Hodak, Daniel Mimouni
BACKGROUND: Venous thromboembolism (VTE) has been reported to be a significant cause of death in patients with pemphigus. OBJECTIVE: We sought to assess the incidence and characteristics of VTE in patients with pemphigus. METHODS: A retrospective study following a cohort of 172 patients with newly diagnosed pemphigus for the development of VTE was conducted. RESULTS: Over a mean follow-up time of 4 years, 10 patients (6%) had development of VTE at a median of 4 months from pemphigus diagnosis...
May 8, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28492232/pemphigus
#10
REVIEW
Michael Kasperkiewicz, Christoph T Ellebrecht, Hayato Takahashi, Jun Yamagami, Detlef Zillikens, Aimee S Payne, Masayuki Amagai
Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. The sites of blister formation can be physiologically explained by the anti-desmoglein autoantibody profile and tissue-specific expression pattern of desmoglein isoforms...
May 11, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28492227/pemphigus
#11
REVIEW
(no author information available yet)
No abstract text is available yet for this article.
May 11, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28492025/validation-studies-of-outcome-measures-in-pemphigus
#12
REVIEW
Sarah Hanna, Minhee Kim, Dedee F Murrell
Pemphigus is a group of rare and potentially fatal autoimmune blistering diseases that are associated with auto-antibodies that target intercellular adhesion molecules. Incidence of pemphigus varies among populations, with the lowest incidence in Switzerland and Finland at 0.6-0.76 per million per year and the highest in Jewish communities at 16.1-32 per million per year. Pemphigus is associated with devastating morbidity and despite advancements in our understanding of the disease and a widening array of therapeutic options, no cure exists...
December 2016: International Journal of Women's Dermatology
https://www.readbyqxmd.com/read/28492006/evaluation-of-prolactin-levels-in-patients-with-newly-diagnosed-pemphigus-vulgaris-and-its-correlation-with-pemphigus-disease-area-index
#13
Vahideh Lajevardi, Zahra Hallaji, Maryam Daneshpazhooh, Narges Ghandi, Peyman Shekari, Sepideh Khani
BACKGROUND: Prolactin is a hormone; in addition to it known roles, it has immunomodulatory effects on lymphocytes maturation and immunoglobulins production. Hyperprolactinemia has been demonstrated in various autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, type I diabetes mellitus, and Graves' disease. In view of the prolactin immunomodulatory roles, studying prolactin levels in pemphigus as an autoimmune blistering disease may introduce new ways of understanding disease etiology and developing treatment strategies...
June 2016: International Journal of Women's Dermatology
https://www.readbyqxmd.com/read/28490623/genetics-based-interactions-of-foundation-species-affect-community-diversity-stability-and-network-structure
#14
Arthur R Keith, Joseph K Bailey, Matthew K Lau, Thomas G Whitham
We examined the hypothesis that genetics-based interactions between strongly interacting foundation species, the tree Populus angustifolia and the aphid Pemphigus betae, affect arthropod community diversity, stability and species interaction networks of which little is known. In a 2-year experimental manipulation of the tree and its aphid herbivore four major findings emerged: (i) the interactions of these two species determined the composition of an arthropod community of 139 species; (ii) both tree genotype and aphid presence significantly predicted community diversity; (iii) the presence of aphids on genetically susceptible trees increased the stability of arthropod communities across years; and (iv) the experimental removal of aphids affected community network structure (network degree, modularity and tree genotype contribution to modularity)...
May 17, 2017: Proceedings. Biological Sciences
https://www.readbyqxmd.com/read/28489243/nasal-pharyngeal-and-laryngeal-pemphigus-vulgaris-successfully-treated-with-rituximab
#15
Naveed Sami
Pemphigus vulgaris is a potentially fatal autoimmune blistering disease that can involve the nasopharyngeal and laryngeal tissues. The disease can be recalcitrant to conventional oral treatments, and treatment alternatives are limited. This retrospective study evaluated the efficacy of rituximab as a rescue agent in 5 patients with recalcitrant pemphigus vulgaris involving nasopharyngeal and laryngeal mucosa. All 5 patients were unresponsive to systemic steroids and at least one conventional oral immunosuppressive agent...
April 2017: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/28473193/corrigendum-to-first-line-treatment-of-pemphigus-vulgaris-with-a-novel-protocol-in-patients-with-contraindications-to-systemic-corticosteroids-and-immunosuppressive-agents-preliminary-retrospective-study-with-a-seven-year-follow-up-int-immunopharmacol-2016
#16
https://www.readbyqxmd.com/read/28471236/a-study-of-clinical-histopathological-and-direct-immunofluorescence-diagnosis-in-pemphigus-group-utility-of-direct-immunofluorescence
#17
Z Hrabovska, J Jautova, V Hrabovsky
AIMS: To determine the diagnostic accordance between histopathological and direct immunofluorescence diagnosis of patients with autoimmune vesiculobullous skin diseases. BACKGROUND: The term pemphigus refers to a group of autoimmune blistering diseases mediated by auto-antibodies directed against desmoglein proteins. The differentiation between the various bullous diseases is important for treatment and prognosis. Direct immunofluorescence microscopy is still the gold standard in differentiating these diseases...
2017: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/28469076/stat3-regulates-desmoglein-3-transcription-in-epithelial-keratinocytes
#18
Xuming Mao, Michael Jeffrey T Cho, Christoph T Ellebrecht, Eric M Mukherjee, Aimee S Payne
Pemphigus vulgaris (PV) is an epithelial blistering disease caused by autoantibodies to the desmosomal cadherin desmoglein 3 (DSG3). Glucocorticoids improve disease within days by increasing DSG3 gene transcription, although the mechanism for this observation remains unknown. Here, we show that DSG3 transcription in keratinocytes is regulated by Stat3. Treatment of primary human keratinocytes (PHKs) with hydrocortisone or rapamycin, but not the p38 MAPK inhibitor SB202190, significantly increases DSG3 mRNA and protein expression and correspondingly reduces phospho-S727 Stat3...
May 4, 2017: JCI Insight
https://www.readbyqxmd.com/read/28413387/successful-treatment-of-a-bullous-pemphigoid-patient-with-rituximab-who-was-refractory-to-corticosteroid-and-omalizumab-treatments
#19
Aslı Bilgiç Temel, Cumhur Ibrahim Bassorgun, Ayşe Akman-Karakaş, Erkan Alpsoy, Soner Uzun
Omalizumab is a humanized monoclonal antibody which is an FDA-approved treatment of severe allergic asthma and inhibits IgE binding to FcεRI. According to increasing evidence of IgE inhibition, omalizumab was suggested as a therapeutic approach for bullous pemphigoid (BP). Rituximab has been reported to be effective in various autoimmune diseases, including autoimmune bullous dermatoses. A specific protocol for the use of rituximab to treat BP patients is not yet available. There are only small case series and case reports about the efficacy and safety of rituximab in BP...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28401573/is-tocilizumab-a-potential-therapeutic-option-for-refractory-unicentric-castleman-disease
#20
Muhammad Bilal Abid, Rachel Peck, Muhammad Abbas Abid, Wesam Al-Sakkaf, Yuening Zhang, Giles S Dunnill, Konrad Staines, Iara-Maria Sequeiros, Lisa Lowry
Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. We present a case of a patient, negative for HIV and HHV-8, who had an unsuccessful surgical intervention and no response to radiotherapy and chemotherapy...
April 11, 2017: Hematological Oncology
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