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pemphigus vulgaris

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https://www.readbyqxmd.com/read/28633752/rituximab-administration-in-a-patient-with-pemphigus-vulgaris-following-reactivation-of-occult-hepatitis-b-virus-infection
#1
Soheil Tavakolpour, Tahereh Soori, Pedram Noormohammadpour, Kamran Balighi, Hamidreza Mahmoudi, Maryam Daneshpazhooh
Immunosuppressive drugs are the milestone of treatment of autoimmune diseases, but they can lead to serious complications, including hepatitis B virus reactivation in HBV carriers as well as in patients with occult HBV infection (OBI). A 36-year-old man with OBI was diagnosed with pemphigus vulgaris. He was prescribed prednisolone and his hepatitis B surface antigen turned positive. Viral replication was successfully controlled by lamivudine and adefovir. Mycophenolate mofetil and intravenous immunoglobulin  were not effective in controlling the pemphigus vulgaris...
June 20, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28632917/miniaturization-of-sebaceous-glands-a-novel-histopathological-finding-in-pemphigus-vulgaris-and-pemphigus-foliaceus-of-the-scalp
#2
Marta Sar-Pomian, Joanna Czuwara, Lidia Rudnicka, Malgorzata Olszewska
BACKGROUND: Intraepidermal acantholysis is a characteristic histopathological feature of pemphigus. The histopathology of scalp biopsies in pemphigus has not been widely discussed in the literature, although the scalp is commonly involved in pemphigus. METHODS: A total of 32 consecutive patients with scalp involvement (seventeen with pemphigus vulgaris and fifteen with pemphigus foliaceus) were enrolled into the study. The histopathological examination of scalp biopsies was performed in all patients...
June 20, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28631092/rituximab-treatment-in-pemphigus-vulgaris-effect-on-circulating-tregs
#3
Bakr El-Zawahry, Dalia Bassiouny, Rehab Hegazy, Heba Gawdat, Suzan Shalaby, Mervat Khorshied, Marwah Adly Saleh
Rituximab (RTX) has been used successfully to treat refractory pemphigus. We aimed to assess the response of pemphigus vulgaris (PV) cases to RTX therapy and its effect on CD4(+)CD25(+) (T regulatory) cells level. Sixteen PV patients were included in this study, each received one cycle of two RTX infusions (1000 mg on days 1 and 15). Five PV patients served as controls. All cases were on prednisolone ± adjuvant therapy. Pemphigus disease area index (PDAI), autoimmune bullous skin intensity score (ABSIS), anti-desmoglein antibodies, CD4, CD8, CD20 and CD4(+)CD25(+) levels were assessed at baseline, 3, 6 and 12 months after therapy...
June 19, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28620161/different-signaling-patterns-contribute-to-loss-of-keratinocyte-cohesion-dependent-on-autoantibody-profile-in-pemphigus
#4
Elias Walter, Franziska Vielmuth, Lukas Rotkopf, Miklós Sárdy, Orsolya N Horváth, Matthias Goebeler, Enno Schmidt, Rüdiger Eming, Michael Hertl, Volker Spindler, Jens Waschke
Pemphigus is an autoimmune blistering skin disease caused primarily by autoantibodies against desmoglein (Dsg)1 and 3. Here, we characterized the mechanisms engaged by pemphigus IgG from patients with different clinical phenotypes and autoantibody profiles. All pemphigus vulgaris (PV) and pemphigus foliaceus (PF) IgG and AK23, a monoclonal mouse antibody against Dsg3, caused loss of cell cohesion, cytokeratin retraction and p38MAPK activation. Strong alterations in Dsg3 distribution were caused by mucosal (aDsg3 antibodies), mucocutaneous (aDsg1 + aDsg3) as well as atypical (aDsg3) PV-IgG...
June 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28611998/recurrent-paronychia-as-a-presenting-manifestation-of-pemphigus-vulgaris-a-case-report
#5
Vijay Zawar, Manoj Pawar, Shrikant Kumavat
Nail involvement in pemphigus vulgaris (PV) is an unusual clinical finding. The most common clinical manifestations include chronic paronychia and onychomadesis. We report an adult female patient with PV who initially presented with chronic paronychia of multiple fingernails and toenails before the onset of vesiculobullous eruptions. Later on, after complete resolution, there was recurrence of pemphigus presenting as hemorrhagic paronychia of the left index finger. Systemic treatment with corticosteroids and azathioprine led to complete resolution of mucocutaneous lesions as well as nail manifestations...
March 2017: Skin Appendage Disorders
https://www.readbyqxmd.com/read/28611508/correlation-between-il36%C3%AE-and-il17-and-activity-of-the-disease-in-selected-autoimmune-blistering-diseases
#6
Agnieszka Żebrowska, Anna Woźniacka, Katarzyna Juczyńska, Kamila Ociepa, Elżbieta Waszczykowska, Izabela Szymczak, Rafał Pawliczak
Dermatitis herpetiformis (DH), bullous pemphigoid (BP), and pemphigus vulgaris (PV) are autoimmune bullous skin conditions with eosinophilic and neutrophilic infiltrations. While cytokines are crucial for the affinity and activation of different leukocyte cells in the inflammation and blister formation, there are no studies concerning a role of IL-36. The goal of the study was to analyze whether interleukin 36 is involved in pathogenesis of DH, BP, and PV. And the second aim of the study was the estimation of correlation between Il-36 and IL-17 and titers of specific antibodies in these diseases...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28608226/blocking-rhoa-rock-inhibits-the-pathogenesis-of-pemphigus-vulgaris-by-suppressing-oxidative-stress-and-apoptosis-through-tak1-nod2-mediated-nf-%C3%AE%C2%BAb-pathway
#7
Junqin Liang, Xuewen Zeng, Yilinuer Halifu, Wenjing Chen, Fengxia Hu, Peng Wang, Huan Zhang, Xiaojing Kang
Oxidative stress and apoptosis play critical roles in pemphigus vulgaris (PV). The main aim of the present study was to investigate the effects of RhoA/ROCK signaling on UVB-induced oxidative damage, and to delineate the molecular mechanisms involved in the UVB-mediated inflammatory and apoptotic response. In HaCaT cells, we observed that blockage of RhoA/ROCK signaling with the inhibitor CT04 or Y27632 greatly inhibited the UVB-mediated increase in intracellular reactive oxygen species (ROS). Additionally, inhibition of RhoA/ROCK signaling reduced UVB-induced apoptosis, as exemplified by a reduction in DNA fragmentation, and also elevated anti-apoptotic Bcl-2 protein, concomitant with reduced levels of pro-apoptotic protein Bax, caspase-3 cleavage and decreased PARP-1 protein...
June 12, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28600798/inhibition-of-p38mapk-signaling-prevents-epidermal-blistering-and-alterations-of-desmosome-structure-induced-by-pemphigus-autoantibodies-in-human-epidermis
#8
D T Egu, E Walter, V Spindler, J Waschke
BACKGROUND: Pemphigus vulgaris (PV) is a skin blistering disease caused by autoantibodies targeting the desmosomal adhesion proteins desmoglein (Dsg) 3 and 1. The mechanisms underlying pemphigus skin blistering are not fully elucidated but p38MAPK activation is one of the signaling events necessary for full loss of cell cohesion. However, it is unclear whether ultrastructural hallmarks of desmosome morphology as observed in patients' lesions are mediated by p38MAPK signaling. OBJECTIVE: In this study, we tested the relevance of p38MAPK for blister formation and the ultrastructural changes induced by PV autoantibodies in human skin...
June 10, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28597020/pemphigus-vulgaris-persistently-localized-to-the-nose-with-local-and-systemic-response-to-topical-steroids
#9
Connie Zhang, Ilana Goldscheider, Thomas Ruzicka, Miklós Sárdy
No abstract text is available yet for this article.
June 9, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28596152/bullous-pemphigoid-and-pemphigus-vulgaris
#10
Mahaz Kayani, Arif M Aslam
No abstract text is available yet for this article.
June 8, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/28591691/naringenin-protects-keratinocytes-from-oxidative-stress-injury-via-inhibition-of-the-nod2-mediated-nf-%C3%AE%C2%BAb-pathway-in-pemphigus-vulgaris
#11
Junqin Liang, Yilinuer Halipu, Fengxia Hu, Buwajieer Yakeya, Wenjing Chen, Huan Zhang, Xiaojing Kang
Naringenin is known to have anti-oxidative activity; however, the effect of naringenin on the progression of pemphigus vulgaris (PV) still remains unclear. This study aims to analyze the effect of naringenin on HaCaT cell apoptosis and oxidative damage under the treatment of PV serum. The results showed that PV serum significantly induced cell apoptosis compared with the control group; whereas, comparing with PV group, naringenin inhibited cell apoptosis. Moreover, PV serum increased the expression of bax and caspase-3, and decreased the expression of bcl-2; but naringenin significantly suppressed the expression of bax and caspase-3, induced the expression of bcl-2...
June 4, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28571245/rituximab-a-magic-bullet-for-pemphigus
#12
V Anandan, W Afthab Jameela, R Sowmiya, M Mani Surya Kumar, P Lavanya
INTRODUCTION: Pemphigus, an autoimmune disease, was fatal before the era of corticosteroids. With the advent of steroids, mortality decreased but morbidity was present due to the side effects of high dose steroids. Newer drugs targeted at the molecular level are said to have fewer side effects and improved effectiveness. AIM: The aim of our study was to assess the effectiveness of one such drug, Rituximab, a biological, in treating pemphigus vulgaris and to identify common adverse events...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28559814/the-rare-case-of-pemphigus-vegetans-in-association-with-malnutrition-children-in-the-multidisciplinary-management
#13
Oki Suwarsa, Endang Sutedja, Hartati Purbo Dharmadji, Pramita Kusuma, July Rahardja, Reti Hindritiani, Hendra Gunawan
Pemphigus vegetans is a clinical variant of pemphigus vulgaris, accounting for 1-2% of all very rare pemphigus cases in children. The involvement of the oral mucosa in this disease is usually accompanied by severe pain that aggravates the patient's malnourished condition. Conversely, malnutrition may also reduce vulnerability towards autoimmune diseases. Although pemphigus vegetans has never been reported to develop in a child with marasmus before, we encountered a case of pemphigus vegetans in a severely malnourished patient...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28559810/atypical-clinical-and-serological-manifestation-of-pemphigus-vegetans-a-case-report-and-review-of-the-literature
#14
REVIEW
Rebecca Mergler, Andreas Kerstan, Enno Schmidt, Matthias Goebeler, Sandrine Benoit
Pemphigus vegetans (PVeg) is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. Exceptional manifestations may be misdiagnosed resulting in delayed diagnosis and treatment. Diagnosis is confirmed by immunofluorescence and detection of anti-desmoglein (Dsg) 3 and/or anti-Dsg1 antibodies. We herein report an unusual manifestation of PVeg. At the time of first presentation, lesions were restricted to the right ring finger's tip...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28546112/pemphigus-vulgaris-induced-by-5-aminolaevulinic-acid-based-photodynamic-therapy
#15
Qian Zhou, Peiru Wang, Linglin Zhang, Bo Wang, Lei Shi, Uma Keyal, Anil Kumar Bhatta, Guolong Zhang, Xiuli Wang
Pemphigus vulgaris (PV) is an autoimmune disorder resulting from the interaction between autoantibodies and desmoglein. Here, we report a case of PV developed after 5-aminolaevulinic acid-based photodynamic therapy (ALA-PDT). The harmful and deleterious effects of UV radiation on the onset, during course, and perpetuation of PV have been observed for decades. Correlation between reactive oxygen species (ROS) and PV have also been reported. Oxidative proteins, which are modified by ROS, and subsequent production of antibodies by immune system seem to be responsible for PV developed following ALA-PDT...
May 22, 2017: Photodiagnosis and Photodynamic Therapy
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
#16
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
#17
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
#18
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/28498563/identification-of-factors-associated-with-treatment-refractoriness-of-oral-lesions-in-pemphigus-vulgaris
#19
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/28492232/pemphigus
#20
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
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