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pemphigus vulgaris

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https://www.readbyqxmd.com/read/27902996/pemphigus-pathogenesis-to-treatment
#1
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/27872890/koebner-phenomenon-in-pemphigus-vulgaris-patients
#2
Kamran Balighi, Maryam Daneshpazhooh, Arghavan Azizpour, Vahide Lajevardi, Fariba Mohammadi, Cheyda Chams-Davatchi
No abstract text is available yet for this article.
September 2016: JAAD Case Reports
https://www.readbyqxmd.com/read/27869434/-childhood-pemphigus-vulgaris-a-case-report
#3
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/27829102/correlation-of-serum-levels-of-ige-autoantibodies-against-bp180-with-bullous-pemphigoid-disease-activity
#4
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/27814654/a-reciprocal-hla-disease-association-in-rheumatoid-arthritis-and-pemphigus-vulgaris
#5
Vincent Van Drongelen, Joseph Holoshitz
  Human leukocyte antigens (HLA) have been extensively studied as being antigen presenting receptors, but many aspects of their function remain elusive, especially their association with various autoimmune diseases. Here we discuss an illustrative case of the reciprocal relationship between certain HLA-DRB1 alleles and two diseases, rheumatoid arthritis (RA) and pemphigus vulgaris (PV). RA is strongly associated with HLA-DRB1 alleles that encode a five amino acid sequence motif in the 70-74 region of the DR beta chain, called the shared epitope (SE), while PV is associated with the HLA-DRB1*04:02 allele that encodes a different sequence motif in the same region...
January 1, 2017: Frontiers in Bioscience (Landmark Edition)
https://www.readbyqxmd.com/read/27800178/the-prevalence-of-s-aureus-skin-and-soft-tissue-infections-in-patients-with-pemphigus
#6
Zeinab Fagheei Aghmiyuni, Ahmad Khorshidi, Rezvan Moniri, Tahereh Soori, Seyed Gholam Abbas Musavi
Pemphigus vulgaris are autoimmune blistering diseases that may result in significant morbidity and death. Immunosuppressive therapy of pemphigus vulgaris would predispose the patients to infections. The aim of this study was to assess the prevalence of S. aureus infection and PVL gene in patients with pemphigus admitted to dermatology clinic. Materials and Methods. This descriptive study was conducted on 196 pemphigus vulgaris patients (119 males, 77 females) admitted to dermatology clinic between 2014 and 2015...
2016: Autoimmune Diseases
https://www.readbyqxmd.com/read/27796136/rituximab-treatment-of-pemphigus-in-women-of-childbearing-age-experience-with-two-patients
#7
Eden P Lake, Yu-Hui Huang, Iris K Aronson
Pemphigus vulgaris is an autoimmune blistering disorder treated with systemic steroids and immunosuppressive agents. Treatment of this disorder in young women of childbearing age must take into consideration the patient's desire for pregnancy and effects of the treatment on both mother and child. We report two young women with pemphigus, initially treated with standard immunosuppressive medications, who expressed their wishes for pregnancy. The immunosuppressive agents were tapered and both patients were treated with Rituximab and IVIG, permitting discontinuation of other medications, conception and pregnancy without any oral steroids or immunosuppressive agents...
November 20, 2016: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/27792691/-hla-class-ii-in-mexican-patients-with-pemphigus-vulgaris-shared-epitope-for-autoimmunity
#8
Lucía Rangel-Gamboa, María Elisa Vega-Memije, Víctor Acuña-Alonzo, Julio Granados-Arriola
INTRODUCTION: Pemphigus is an autoimmune blistering disease of skin and mucous membranes characterized by presence of IgG antibodies against desmoglein 3, and 1. Desmoglein 3 and 1 are presented in pemphigus vulgaris and pemphigus foliaceous, respectively. Desmoglein are transmembrane proteins that form part of cellular junctions called desmosomes. Major histocompatibility complex class II molecules have been related to autoimmune disease; in pemphigus vulgaris, different human lymphocyte antigens (HLA) were associated among different ethnic groups, such as HLA-DR4, HLA-DR14, and HLA-DR1...
September 2016: Gaceta Médica de México
https://www.readbyqxmd.com/read/27767277/zeitlicher-verlauf-der-avaskul%C3%A3-ren-nekrose-des-h%C3%A3-ftkopfes-bei-patienten-mit-pemphigus-vulgaris
#9
Kamran Balighi, Maryam Daneshpazhooh, Nessa Aghazadeh, Vahide Saeidi, Farzam Shahpouri, Pardis Hejazi, Cheyda Chams-Davatchi
HINTERGRUND UND ZIELE: Pemphigus vulgaris (PV) wird in der Regel mit systemischen Corticosteroiden und Immunsuppressiva behandelt. Avaskuläre Nekrose (AVN) des Hüftkopfes ist eine gut bekannte schwerere Komplikation einer Corticosteroid-Therapie. Die Charakteristika dieser schweren Komplikation bei PV sind nach wie vor unbekannt. PATIENTEN UND METHODEN: Nicht kontrollierte, retrospektive Untersuchung aller PV-bedingten AVN-Fälle, die in einer iranischen Klinik für bullöse Autoimmunerkrankungen zwischen 1985 und 2013 diagnostiziert wurden...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/27767268/temporal-course-of-avascular-femoral-head-necrosis-in-patients-with-pemphigus-vulgaris
#10
Kamran Balighi, Maryam Daneshpazhooh, Nessa Aghazadeh, Vahide Saeidi, Farzam Shahpouri, Pardis Hejazi, Cheyda Chams-Davatchi
BACKGROUND AND OBJECTIVES: Pemphigus vulgaris (PV) is typically treated with systemic corticosteroids and immunosuppressive agents. Avascular necrosis (AVN) of the femoral head is a well-recognized major complication of corticosteroid therapy. The characteristics of this serious complication in PV remain unknown. PATIENTS AND METHODS: Uncontrolled, retrospective study of all PV-related AVN cases diagnosed at an Iranian autoimmune bullous disease clinic between 1985 and 2013...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/27755423/pemphigus-vulgaris-how-to-perform-an-oral-biopsy-properly
#11
Aneliza de Fatima Moraes da Silva, Daniel Galera Bernabé, Glauco Issamu Miyahara, Eder Ricardo Biasoli, Renata Callestini, Kellen Cristine Tjioe
Oral biopsy of vesiculobullous diseases such as pemphigus vulgaris often raises questions due to some particularities involving this procedure. The adequate selection of the area to be biopsied defines if the final diagnosis will be reached, being the Achiles heel of the proper management of the patient. Here, the authors report a case of a woman who sought for treatment of generalized oral blisters and ulcers that caused severe pain. She had undergone a previous biopsy by other professionals that was inconclusive...
October 14, 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27750289/pemphigus-autoantibodies-induce-blistering-in-human-conjunctiva
#12
Franziska Vielmuth, Vera Rötzer, Eva Hartlieb, Christoph Hirneiß, Jens Waschke, Volker Spindler
Purpose: The autoimmune blistering skin disease pemphigus vulgaris (PV) is caused by autoantibodies against desmosomal adhesion molecules. Patients may suffer conjunctival involvement, yet the underlying mechanisms are largely unclear. We characterized human and murine conjunctiva with respect to the PV autoantigens, and evaluated the effects and mechanisms of PV autoantibodies applied to human conjunctiva ex vivo. Methods: We obtained human conjunctiva specimens from surgical explants and established a short-term culture model to study the alterations induced by antibody fractions of PV patients (PV-IgG)...
August 1, 2016: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/27721634/oral-pemphigus-vulgaris-a-case-report-with-direct-immunofluorescence-study
#13
Sangeetha Jeevan Kumar, S P Nehru Anand, Nandhini Gunasekaran, Rajkumar Krishnan
Pemphigus vulgaris (PV) is a chronic, autoimmune, intraepidermal blistering disease of the skin and mucous membranes. The initial clinical manifestation is frequently the development of intraoral lesions, and later, the lesions involve the other mucous membranes and skin. The etiology of this disease still remains obscure although the presence of autoantibodies is consistent with an autoimmune disease. These antibodies are targeted against the adhesion proteins of keratinocytes, leading to acantholysis (disruption of spinous layer, leading to intraepidermal clefting) and blister formation...
September 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
https://www.readbyqxmd.com/read/27698327/efficacy-of-dapsone-in-the-treatment-of-pemphigus-vulgaris-a-single-center-case-study
#14
Sharon Baum, Assaf Debby, Sarit Gilboa, Henri Trau, Aviv Barzilai
BACKGROUND: Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease. Most patients require long-term therapy with systemic steroids, and a steroid-sparing agent is usually also utilized. Dapsone is a chemotherapeutic agent with anti-inflammatory properties that is used as a steroid-sparing agent in PV. OBJECTIVE: The aim of the present study was to evaluate the efficacy of dapsone as an adjuvant therapy in patients with PV. METHODS: A retrospective analysis of patients' files was performed...
October 4, 2016: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/27688439/significance-of-anti-cyclic-citrullinated-peptide-autoantibodies-in-immune-mediated-inflammatory-skin-disorders-with-and-without-arthritis
#15
Chander Grover, Bineeta Kashyap, Deepashree Daulatabad, Amit Dhawan, Iqbal R Kaur
BACKGROUND: Anti-cyclic citrullinated peptides (CCPs) are autoantibodies directed against citrullinated peptides. Rheumatoid factor (RF), an antibody against the Fc portion of IgG, is known to form immune complexes and contribute to the etiopathogenesis of various skin disorders. C-reactive protein (CRP), an acute-phase protein, increases following secretion of interleukin-6 from macrophages and T cells. Anti-CCP, RF, and CRP are well-established immune-markers, their diagnostic potential in immune-mediated skin disorders remains less widely studied...
September 2016: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/27631412/-involvement-of-mucous-membranes-in-autoimmune-bullous-diseases
#16
C Günther
Autoimmune bullous diseases are characterized by intraepidermal or subepidermal autoantibody deposition that leads to blisters and secondary erosion. Mucous membranes are frequently affected in pemphigus vulgaris and always involved in cicatricial and mucosal pemphigoid. Mucosal lesions are detected less frequently in patients with bullous pemphigoid or epidermolysis bullosa acquisita. The diagnosis of autoimmune bullous disorders is based on determination of the subtype of autoantibodies bound in the skin and the clinical picture...
October 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/27603326/rituximab-uses-in-dermatology
#17
K Gleghorn, J Wilson, M Wilkerson
Rituximab is an anti-CD20 monoclonal antibody with considerable potential in dermatology due to an increase in off-label indications. Chronic graft-versus-host disease and pemphigus vulgaris are two of the most promising indications for off-label use of rituximab. It is a generally safe alternative that should be considered when traditional therapy with corticosteroids or immunosuppressants has failed or caused significant intolerance. Currently, rituximab is only FDA-approved for treatment of follicular and diffuse large B-cell non-Hodgkin's lymphoma, rheumatoid arthritis, chronic lymphocytic leukemia, granulomatosis with polyangiitis (formerly Wegener's granulomatosis) and microscopic polyangiitis...
September 2016: Skin Therapy Letter
https://www.readbyqxmd.com/read/27600093/successful-monotherapy-of-pemphigus-vegetans-with-minocycline-and-nicotinamide
#18
A von Köckritz, S Ständer, C Zeidler, D Metze, T Luger, G Bonsmann
BACKGROUND: Pemphigus vegetans is a rare variant of pemphigus vulgaris, accounting for 1-2% of all pemphigus diseases. Systemic corticosteroids are the therapy of first choice in combination with immunosuppressants as steroid-sparing agents. OBJECTIVE: To highlight the exceptional but successful use of minocycline/nicotinamide monotherapy in pemphigus vegetans. METHODS: A review of the literature to date about pemphigus vegetans with special emphasis on therapy was performed...
September 7, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27599778/antigastric-parietal-cell-and-antithyroid-autoantibodies-in-patients-with-desquamative-gingivitis
#19
Julia Yu-Fong Chang, Chun-Pin Chiang, Yi-Ping Wang, Yang-Che Wu, Hsin-Ming Chen, Andy Sun
BACKGROUND: Desquamative gingivitis (DG) is principally associated with erosive oral lichen planus (EOLP), mucous membrane pemphigoid (MMP), and pemphigus vulgaris (PV). METHODS: Serum autoantibodies including antigastric parietal cell antibody (GPCA), antithyroglobulin antibody (TGA), and antithyroid microsomal antibody (TMA) were measured in 500 patients with DG, 287 EOLP without DG (EOLP/DG(-) ) patients, and 100 healthy control subjects. RESULTS: The 500 patients with DG were diagnosed as having EOLP in 455 (91%), PV in 40 (8%), and MMP in five (1%) patients...
September 7, 2016: Journal of Oral Pathology & Medicine
https://www.readbyqxmd.com/read/27576321/assessment-of-the-effects-of-rituximab-monotherapy-on-different-subsets-of-circulating-t-regulatory-cells-and-clinical-disease-severity-in-severe-pemphigus-vulgaris
#20
Rajsmita Bhattacharjee, Dipankar De, Sanjeev Handa, Ranjana W Minz, Biman Saikia, Neha Joshi
BACKGROUND: Robust evidence for the efficacy of rituximab monotherapy in pemphigus is lacking. The effects of rituximab on T-regulatory cells (Tregs) in pemphigus have not been studied. OBJECTIVE: The primary objective was to assess the efficacy of rituximab monotherapy in severe pemphigus vulgaris. The secondary objectives were to assess whether counts of different subsets of Tregs in the peripheral blood correlate with baseline clinical severity and whether clinical response in severe pemphigus is associated with an alteration in the Treg count...
September 1, 2016: Dermatology: International Journal for Clinical and Investigative Dermatology
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