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Revista Gaucha de Enfermagem 2020To identify the primary bandages recommended in the literature to dress the wounds of people with pemphigus vulgaris and to describe the positive or negative... (Review)
Review
OBJECTIVE
To identify the primary bandages recommended in the literature to dress the wounds of people with pemphigus vulgaris and to describe the positive or negative repercussions related to these bandages.
METHOD
Integrative literature review, using the descriptors "dressing", "nursing care", "nursing", "skin diseases / vesiculobollous" and "pemphigus"; inclusion criteria: articles that address primary bandages for pemphigus vulgaris skin lesion dressing; published between 2010 and 2017; in Portuguese, English or Spanish; indexed in BDENF, LILACS, PubMed, and/or Scopus.
RESULTS
eight articles were selected, seven had evidence levels 4 and 5 and one had evidence level 3. The primary bandages covered were: silver gel, hydrocolloid, hydrogel, silver-containing hydrofiber, antibiotics, sterilized vaseline, corticoid, and vitamin E ointment Conclusion: Sterile gauze with vaseline, prepared according to protocol, was the bandage presented in the study with the most significant level of evidence.
Topics: Bandages; Humans; Pemphigus; Treatment Outcome
PubMed: 32491149
DOI: 10.1590/1983-1447.2020.20190259 -
The Journal of Investigative Dermatology Jun 2017Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal... (Review)
Review
Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in Lübeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases. Considerable advances have been made in describing incidences and prevalences of these diseases and linking comorbidities with autoantibody reactivities and clinical variants, for example, dipeptidyl peptidase-IV inhibitor-associated noninflammatory bullous pemphigoid. Although new entities are still being described, diagnosis of most autoimmune blistering diseases can now be achieved using standardized and widely available serological test systems. Various experimental mouse models of pemphigus and pemphigoid disease are increasingly being used to understand mechanisms of central and peripheral tolerance and to evaluate more specific treatment approaches for these disorders, such as molecules that target autoreactive T and B cells and anti-inflammatory mediators, that is, dimethyl fumarate, phosphodiesterase 4, and leukotriene B4 inhibitors in pemphigoid disorders, and chimeric antigen receptor T cells in pemphigus. Very recent experimental data about the immunopathology and the determinants of autoantibody formation and keratinocyte susceptibility in pemphigus were discussed. With regard to cellular mechanisms leading to the loss of cell-cell adhesion, new ideas were shared in the field of signal transduction. Major steps were taken to put the various partly contradictory and controversial findings about the effects of pemphigus autoantibodies and other inflammatory mediators into perspective and broaden our view of the complex pathophysiology of this disease. Finally, two investigator-initiated multicenter trials highlighted doxycycline and dapsone as valuable medications in the treatment of bullous pemphigoid.
Topics: Animals; Autoantibodies; Autoimmune Diseases; Consensus; Female; Germany; Humans; Male; Mice; Pemphigoid, Bullous; Pemphigus; Prognosis; Risk Assessment
PubMed: 28390814
DOI: 10.1016/j.jid.2017.01.028 -
Frontiers in Immunology 2018Pemphigus is a severe autoimmune-blistering disease of the skin and mucous membranes caused by autoantibodies reducing desmosomal adhesion between epithelial cells.... (Review)
Review
Pemphigus is a severe autoimmune-blistering disease of the skin and mucous membranes caused by autoantibodies reducing desmosomal adhesion between epithelial cells. Autoantibodies against the desmosomal cadherins desmogleins (Dsgs) 1 and 3 as well as desmocollin 3 were shown to be pathogenic, whereas the role of other antibodies is unclear. Dsg3 interactions can be directly reduced by specific autoantibodies. Autoantibodies also alter the activity of signaling pathways, some of which regulate cell cohesion under baseline conditions and alter the turnover of desmosomal components. These pathways include Ca, p38MAPK, PKC, Src, EGFR/Erk, and several others. In this review, we delineate the mechanisms relevant for pemphigus pathogenesis based on the histology and the ultrastructure of patients' lesions. We then dissect the mechanisms which can explain the ultrastructural hallmarks detectable in pemphigus patient skin. Finally, we reevaluate the concept that the spectrum of mechanisms, which induce desmosome dysfunction upon binding of pemphigus autoantibodies, finally defines the clinical phenotype.
Topics: Animals; Autoantibodies; Desmosomes; Humans; Pemphigus; Phenotype
PubMed: 29449846
DOI: 10.3389/fimmu.2018.00136 -
The Pan African Medical Journal 2022Pemphigus vulgaris (PV) is an autoimmune mucocutaneous disorder of the oral cavity and is the most common subtype of pemphigus. The etiology remains obscure, although...
Pemphigus vulgaris (PV) is an autoimmune mucocutaneous disorder of the oral cavity and is the most common subtype of pemphigus. The etiology remains obscure, although the disease is characterized by autoantibodies directed against the desmoglein component of the keratinocytes. It manifests clinically as vesicle, bullae or erosions of skin and mucous membrane and histopathologically shows the presence of acantholysis. The presence of exclusive oral lesions initially increases the chances of misdiagnosing the disease as another condition, posing diagnostic, therapeutic and prognostic difficulties, consequently prompt diagnosis and treatment can prevent untoward consequences. Demonstration of IgG antibodies against desmoglein in Immunofluroscence confirms the diagnosis. In here we report a case of a 55-year-old female patient suffering from PV emphasizing the significance of clinical examination, pertinent investigations, treatment rendered and its outcome.
Topics: Acantholysis; Autoantibodies; Blister; Desmogleins; Female; Humans; Immunoglobulin G; Keratinocytes; Middle Aged; Pemphigus
PubMed: 36212921
DOI: 10.11604/pamj.2022.42.184.34184 -
La Tunisie MedicaleDesmoglein (Dsg) 1 and 3 are the 2 major autoantigens in pemphigus foliaceus (PF) and pemphigus vulgaris (PV).
BACKGROUND
Desmoglein (Dsg) 1 and 3 are the 2 major autoantigens in pemphigus foliaceus (PF) and pemphigus vulgaris (PV).
AIM
We aimed to determine anti-Dsg1 and 3 Abs'usefulness in the diagnosis of pemphigus and to assess the correlation of these antibodies (Abs) with clinical phenotype and disease activity in south Tunisian patients.
METHODS
We retrospectively analyzed 131 samples from 82 patients (52 with PF and 30 with PV) during follow-up. Anti-Dsg1 and 3 Abs were measured by ELISA. Consecutive anti-Dsg1 and 3 Abs were correlated with disease activity. Receiver operating characteristics (ROC) curve were calculated to determine anti-Dsg1 and 3 Abs'cut-offs with optimal sensitivity and specificity for disease activity.
RESULTS
Anti-Dsg1 and 3 levels were associated to in PF and PV patients respectively (p<0,001). Anti-Dsg1 and 3 Ab were associated with skin (95%) and mucosal (60%) lesions, respectively. A significant decrease of anti-Dsg1 Abs was observed in patients with PF in clinical remission (36 ± 62 U/mL; (p=0,04). No correlation was found between anti-Dsg3 Abs and the course of mucosal lesions in PV (p=0,3). During follow-up, anti-Dsg1 Abs correlated with relapses (177 ±60 U/mL ; p=0,04). The 161,5 U/mL cut-off for anti-Dsg1 Abs provided 100% specificity and 86,4% sensitivity in PF disease activity. The 30,7U/mL cut-off for anti-Dsg3 provided 89,5% sensitivity and 100% specificity in PV.
CONCLUSIONS
High anti-Dsg3 Abs values are not always associated with PV disease activity. Anti-Dsg1 Abs showed a closer relationship with skin activity in PS and should be therefore taken into account in management of pemphigus patients.
Topics: Autoantibodies; Desmoglein 1; Desmoglein 3; Humans; Pemphigus; Retrospective Studies
PubMed: 35288901
DOI: No ID Found -
Dermatologic Therapy Jan 2021Pemphigus vulgaris (PV), an autoimmune blistering disease is treated with immunosuppressive medications. As the immunosuppressive effect of rituximab, the first-line... (Review)
Review
Pemphigus vulgaris (PV), an autoimmune blistering disease is treated with immunosuppressive medications. As the immunosuppressive effect of rituximab, the first-line therapy of PV, lasts more than 6 months, many concerns have raised due to the ongoing novel coronavirus disease (COVID-19) pandemic. With this background, our objective was to review the currently available literature as well as important websites for the evidence related to rituximab, PV and COVID-19, adverse effects associated with drugs, and relevant guidelines. "PubMed" and "Google Scholar" database were systematically searched for retrieving all articles related to anti-CD20 therapy in pemphigus vulgaris and COVID-19 published up to 14 July 2020. A total of seven clinical studies are performed with anti-CD20 therapy in COVID-19, three of which are performed on pemphigus patients, and have shown concerns employing rituximab in patients with COVID-19. Evidence for treating PV patients with rituximab in COVID-19 pandemic is limited. Until sufficient evidence or guideline for pemphigus and COVID-19 treatment is available, we advocate caution commencing rituximab in patients with pemphigus, due to the reported adverse outcomes.
Topics: COVID-19; Humans; Immunologic Factors; Pandemics; Pemphigus; Rituximab; SARS-CoV-2
PubMed: 33051960
DOI: 10.1111/dth.14405 -
Acta Dermato-venereologica May 2022Pemphigus vulgaris is a rare autoimmune skin disease. Although herpes simplex virus has been associated with autoimmune diseases, evidence regarding its association with...
Pemphigus vulgaris is a rare autoimmune skin disease. Although herpes simplex virus has been associated with autoimmune diseases, evidence regarding its association with pemphigus vulgaris exacerbations is scarce. This retrospective cohort study aimed to characterize the epidemiological and clinical features of patients with pemphigus vulgaris who were herpes simplex-positive, compared with those who were herpes simplex-negative, during disease onset. Of 62 patients with pemphigus vulgaris who underwent PCR testing for herpes simplex virus, 25 (40.3%) were positive, with a mean age of 56.1 ± 15.5 years; 35.5% were male. The herpes-positive group had significantly elevated levels of C-reactive protein, Pemphigus Disease Activity Index score, and shorter time to relapse. The time to remission, number of exacerbations per year, and remission status were non-significantly elevated in the herpes-positive group. Thus, routine testing lesions from patients with pemphigus for herpes simplex virus should be performed. If positive, antiviral treatment should be initiated; and preventive antiviral treatment should be considered in severe cases.
Topics: Adult; Aged; Antiviral Agents; Female; Herpes Simplex; Humans; Male; Middle Aged; Pemphigus; Polymerase Chain Reaction; Retrospective Studies; Simplexvirus
PubMed: 35393625
DOI: 10.2340/actadv.v102.917 -
Cells Jun 2022(1) Background: Pemphigus is a blistering autoimmune disease of the skin and/or mucous membranes, characterised by the presence of specific autoantibodies directed...
(1) Background: Pemphigus is a blistering autoimmune disease of the skin and/or mucous membranes, characterised by the presence of specific autoantibodies directed against structural proteins of the human skin. Recent reports indicate that new haematological parameters, termed Extended Inflammation Parameters (EIP), can be used to assess the activation of immune cells during active inflammation. These include parameters assessing both neutrophil activation (NEUT-RI, NEUT-GI) and the number of activated lymphocytes (RE-LYMP). The aim of this study was to investigate the relationship between changes in NEUT-RI, NEUT-GI and RE-LYMP and the disease activity in patients with pemphigus. (2) Results: The study involved 32 patients with diagnosed different types of pemphigus. Neutrophil activation parameters (NEUT-RI and NEUT-GI) and lymphocytes (RE-LYMP) were significantly higher in these patients compared to the parameters in healthy participants (respectively p = 0.0127, p = 0.0011 and p = 0.0033). The increased quantity of activated lymphocytes (RE-LYMP) also correlated significantly with the extent of skin and/or mucosal lesions in patients assessed by the PDAI scale (p < 0.02). (3) Conclusions: The NEUT-RI, NEUT-GI and RE-LYMP parameters proved to be appropriate markers of inflammation severity in pemphigus, also in relation to local lesions, which was not possible with the inflammation markers (CRP, ESR) used so far on a routine basis.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Inflammation; Pemphigus; Severity of Illness Index
PubMed: 35741041
DOI: 10.3390/cells11121912 -
Frontiers in Immunology 2022Oral lichen planus (OLP) is an inflammatory disease of the oral mucosa. Clinically, two main subsets are described, namely non-erosive and erosive OLP. While non-erosive... (Review)
Review
Oral lichen planus (OLP) is an inflammatory disease of the oral mucosa. Clinically, two main subsets are described, namely non-erosive and erosive OLP. While non-erosive OLP is usually responsive to local therapies, erosive OLP is often refractory also to systemic therapies and extremely reduces the quality of life of the patients. Furthermore, in some erosive OLP cases different autoantibodies have been detected, including anti-desmoglein 1 and 3 autoantibodies, and anti-bullous pemphigoid 180 and 230 autoantibodies. However, their potential role is still not clear. In this paper, we reviewed the literature about the detection of autoantibodies against desmoglein 1 and 3, the main target antigens of pemphigus vulgaris, in patient with OLP, summarizing the more recent insights on this topic.
Topics: Humans; Lichen Planus, Oral; Quality of Life; Mouth Mucosa; Autoantibodies; Pemphigus
PubMed: 36263026
DOI: 10.3389/fimmu.2022.1001970 -
Canadian Medical Association Journal Dec 1970
Review
Topics: Antigen-Antibody Reactions; Autoantibodies; Autoimmune Diseases; Fluorescent Antibody Technique; Humans; Methods; Microscopy, Electron; Pemphigus; Skin Diseases
PubMed: 4922169
DOI: No ID Found