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Dermatology Online Journal Aug 2020Pemphigus has been associated with other autoimmune and autoinflammatory disorders. Specifically, some case reports in the literature document coexistence of pemphigus... (Meta-Analysis)
Meta-Analysis
Pemphigus has been associated with other autoimmune and autoinflammatory disorders. Specifically, some case reports in the literature document coexistence of pemphigus with psoriasis, but this association is lacking larger scale investigation. With this in mind, we conducted a systematic review and meta-analysis to evaluate the association between pemphigus and psoriasis. In doing so, we found an association between the two conditions. Pemphigus was more common in patients with psoriasis than in controls (OR 2.64, 95% CI 1.24-5.59, P=0.01), with heterogeneity (I2=94%). We go on to propose pathophysiologic mechanisms and its relevance for diagnostic and management considerations.
Topics: Humans; Pemphigus; Psoriasis
PubMed: 32941727
DOI: No ID Found -
Pediatric Dermatology Jul 2016We aimed to better understand the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs). We searched Medline,... (Review)
Review
We aimed to better understand the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs). We searched Medline, Embase, PubMed, Latin American and Caribbean Health Sciences Literature, and reference lists of identified articles. Inclusion criteria were articles published from 1946 to December 2014 in any language. Exclusion criteria were age greater than 4 weeks and no confirmed AIBD diagnosis. We identified 51 cases of neonatal AIBDs: 34 cases of pemphigus (31 pemphigus vulgaris [PV], 3 pemphigus foliaceus [PF]) and 17 cases of pemphigoid diseases (9 bullous pemphigoid [BP], 5 linear immunoglobulin A bullous dermatosis [LABD], 1 BP and LABD, 1 epidermolysis bullosa acquisita, 1 bullous systemic lupus erythematosus). Pemphigoid diseases had a higher male predominance (male:female ratio 4.6:1) than pemphigus (male:female ratio 1:1.06) (p = 0.004). Pemphigus had a higher proportion presenting at birth (79.4%) than pemphigoid diseases (29.4%) (p = 0.008). The most common sites involved were the trunk (63.0%), followed by the head and neck (60.9%). The mucosal membranes were involved in 32.6% of cases (27.6% in pemphigus, 41.6% in pemphigoid diseases). Only 33.3% used systemic therapy, and 75.5% achieved control within 3 weeks. Most PV, PF, and BP cases, but not LABDs, reported maternal disease. In pemphigus cases, 75.0% of mothers had active disease and 25.0% were in control. Pregnant women with PV, PF, and PG of any severity can passively transfer autoantibodies leading to neonatal AIBD. Pemphigoid diseases are more likely to present after birth and may be more male predominant. The presentation of LABDs may be different from that of all other AIBDs.
Topics: Autoimmune Diseases; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Male; Pregnancy; Prognosis; Skin Diseases, Vesiculobullous
PubMed: 27086740
DOI: 10.1111/pde.12859 -
Immunologic Research Feb 2019There is little consensus regarding the association between pemphigus and psoriasis. The aim of the current study is to synthesize existing data on the prevalence of... (Meta-Analysis)
Meta-Analysis
There is little consensus regarding the association between pemphigus and psoriasis. The aim of the current study is to synthesize existing data on the prevalence of psoriasis in patients with pemphigus and on the association between the two conditions. We performed a systematic review and meta-analysis of observational studies in Medline, Embase, and Web of Science (1900-2018). Reference lists of included studies were also searched for eligible studies. Quality of evidence was assessed using Newcastle-Ottawa scale (NOS). A meta-analysis was performed using random-effects models to estimate pooled prevalence rates and odds ratios (ORs) with 95% confidence intervals (CI). Subgroup and sensitivity analyses were also conducted. Twelve eligible studies comprising 12,238 patients with pemphigus were included in the quantitative synthesis. The overall random-effects pooled prevalence of psoriasis among patients with pemphigus was 2.4% (95% CI, 1.0-4.4) across all studies. The overall pooled multivariate OR for psoriasis in patients with pemphigus was significantly increased and estimated at 3.5 (95% CI, 1.6-7.6). In conclusion, a significant association was found between pemphigus and psoriasis. Physicians managing patients with pemphigus may be aware of this comorbidity. Further studies are warranted to establish the precise mechanisms underlying this relationship.
Topics: Comorbidity; Humans; Israel; Odds Ratio; Pemphigus; Prevalence; Psoriasis
PubMed: 30338449
DOI: 10.1007/s12026-018-9031-6 -
Frontiers in Immunology 2023Pemphigus foliaceus (PF) differs from pemphigus vulgaris (PV) in that it affects only the skin and mucous membranes are not involved. Pemphigus is commonly treated with...
BACKGROUND
Pemphigus foliaceus (PF) differs from pemphigus vulgaris (PV) in that it affects only the skin and mucous membranes are not involved. Pemphigus is commonly treated with systemic corticosteroids and immunosuppressive agents (ISAs). More recently, biologics have been used. The current literature on biologic therapy often combines treatment of PF with PV, hence it is often difficult for clinicians to isolate the treatment of PF from PV. The purpose of this review was to provide information regarding the use of current biological therapy, specifically in PF.
MATERIALS AND METHODS
A search of PubMed, Embase, and other databases was conducted using keywords pemphigus foliaceus (PF), rituximab (RTX), intravenous immunoglobulin (IVIg), and biologics. Forty-one studies were included in this review, which produced 105 patients with PF, treated with RTX, IVIg, or a combination of both. Eighty-five patients were treated with RTX, eight patients with IVIg, and 12 received both RTX and IVIg.
RESULTS
Most patients in this review had PF that was nonresponsive to conventional immunosuppressive therapies (CIST), and had significant side effects from their use. RTX treatment resulted in complete remission (CR) in 63.2%, a relapse rate of 39.5%, an infection rate of 19.7%, and a mortality rate of 3.9%. Relapse was greater in the lymphoma (LP) protocol than the rheumatoid arthritis (RA) protocol (p<0.0001). IVIg led to CR in 62.5% of patients, with no relapses or infections. Patients receiving both biologics experienced better outcomes when RTX was first administered, then followed by IVIg. Follow-up durations for patients receiving RTX, IVIg, and both were 22.1, 24.8, and 35.7 months, respectively.
DISCUSSION
In pemphigus foliaceus patients nonresponsive to conventional immunosuppressive therapy or in those with significant side effects from CIST, RTX and IVIg appear to be useful agents. Profile of clinical response, as well as relapse, infection, and mortality rates in PF patients treated with RTX were similar to those observed in PV patients. The data suggests that protocols specific for PF may produce better outcomes, less adverse effects, and improved quality of life.
Topics: Humans; Pemphigus; Immunoglobulins, Intravenous; Quality of Life; Immunosuppressive Agents; Rituximab; Recurrence; Biological Products
PubMed: 37901249
DOI: 10.3389/fimmu.2023.1267668 -
Dermatologic Therapy Nov 2022Pemphigus is a group of autoimmune blistering diseases. Given the ulcerative nature of the diseases, the full healing of ulcers and erosions might be challenging. The... (Review)
Review
Pemphigus is a group of autoimmune blistering diseases. Given the ulcerative nature of the diseases, the full healing of ulcers and erosions might be challenging. The aim of this study was to determine various topical wound care options for pemphigus patients, the advantage of each alternative, and to compare their efficacy, safety, and feasibility. In accordance with PRISMA guideline, we performed a systematic search in PubMed/Medline, web of science, and Scopus databases for articles published from inception until February 2, 2022. Out of 703 initially retrieved articles, 11 full texts were included. Our findings were divided into the following categories: silver-containing dressings, paraffin-embedded tulle nets, topical insulin, EPIFIBROIN 0039, platelet gel, and Biobrane®. The most commonly used topical care in pemphigus patients was silver-containing dressings in six studies. All of the included studies reported acceptable outcomes without any severe adverse effects. Due to the few available studies in this field, a definite suggestion cannot be made. We recommend larger randomized controlled studies to identify the best topical care modality in pemphigus patients.
Topics: Humans; Bandages, Hydrocolloid; Silver; Pemphigus; Wound Healing
PubMed: 36070217
DOI: 10.1111/dth.15808 -
Drugs Feb 2015While a variety of intervention options have been described for pemphigus vulgaris, the optimal treatment strategy has not been established. (Review)
Review
BACKGROUND
While a variety of intervention options have been described for pemphigus vulgaris, the optimal treatment strategy has not been established.
OBJECTIVES
The objective of this systematic review is to assess the literature on the efficacy and safety of interventions for the treatment of pemphigus vulgaris.
DATA SOURCES
Five electronic databases were searched, including The Cochrane Skin Group's Specialized Register, The Cochrane Central Register of Controlled Trials (CENTRAL), EMBASE, MEDLINE and Latin American and Caribbean Health science Information database (LILACS). Five trial registers as well as reference lists of included RCTs were also searched.
STUDY ELIGIBILITY CRITERIA
Any published randomised controlled trial (RCT) on intervention for pemphigus vulgaris was included, provided the diagnosis of pemphigus vulgaris was confirmed with appropriate clinical features, histopathology and immunofluorescence studies. Studies which included forms of pemphigus other than pemphigus vulgaris were excluded.
INTERVENTIONS
Altogether 18 RCTs were identified including 16 distinct interventions.
STUDY APPRAISAL AND SYNTHESIS METHODS
Included studies were assessed for patient selection, methods of randomisation, blinding, follow-up and selective reporting.
RESULTS
Current evidence is incomplete and inconclusive. The interventions which appear promising, but will require further evaluation include adjuvant mycophenolate mofetil (MMF), azathioprine, intravenous immunoglobulins (IVIG), sulfasalazine and pentoxifylline, infliximab, epidermal growth factor and pimecrolimus. Interventions with inconclusive evidence include high (120-180 mg) versus low (45-60 mg) prednisone dosage, pulsed dexamethasone, cyclophosphamide, dexamethasone-cyclophosphamide pulse therapy (DCP), cyclosporine, dapsone, etanercept, acyclovir and tacrolimus.
LIMITATIONS
Our review is limited by the small number of high-quality RCTs and variety of outcome measures, precluding the performing of a meta-analysis.
CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS
The optimal treatment strategy for pemphigus vulgaris remains unclear. Higher quality RCTs are required in the future to re-evaluate many interventions and to explore other unstudied interventions.
Topics: Anti-Inflammatory Agents, Non-Steroidal; Dose-Response Relationship, Drug; Glucocorticoids; Humans; Immunologic Factors; Pemphigus; Randomized Controlled Trials as Topic; Treatment Outcome
PubMed: 25655250
DOI: 10.1007/s40265-015-0353-6 -
JAMA Dermatology Dec 2021There is a lack of evidence synthesis on the association between bullous skin disease and depression.
IMPORTANCE
There is a lack of evidence synthesis on the association between bullous skin disease and depression.
OBJECTIVE
To synthesize and interpret the current evidence on the association between bullous skin disease and depression.
EVIDENCE REVIEW
This review was conducted according to PRISMA guidelines and reviewed literature related to bullous skin disease and depression in the PubMed, Embase, PsycInfo, and Cochrane databases published between 1945 and February 2021. The quality of each included article was assessed via the Newcastle-Ottawa Scale. This review was registered with PROSPERO (CRD42021230750).
FINDINGS
A total of 17 articles were identified that analyzed a total of 83 910 patients (55.2% female; specifically, 6951 patients with bullous pemphigoid, 1669 patients with pemphigus, and 79 patients with epidermolysis bullosa were analyzed). The prevalence of depressive symptoms among patients with bullous dermatoses ranged from 40% to 80%. The prevalence of depression diagnosis among patients with bullous dermatoses ranged from 11.4% to 28%.
CONCLUSIONS AND RELEVANCE
In this systematic review, high rates of depression and depressive symptoms existed among patients with bullous skin disease. Adequate treatment of bullous dermatoses may be associated with a decrease in mental health burden on patients.
Topics: Depression; Epidermolysis Bullosa; Female; Humans; Male; Pemphigoid, Bullous; Pemphigus; Skin Diseases, Vesiculobullous
PubMed: 34668929
DOI: 10.1001/jamadermatol.2021.4055 -
Experimental Dermatology Jul 2023Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety and treatment durability of... (Review)
Review
A systematic review on efficacy, safety and treatment durability of intravenous immunoglobulin in autoimmune bullous dermatoses: Special focus on indication and combination therapy.
Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety and treatment durability of intravenous immunoglobulin (IVIg) as an alternative treatment should be explored to systematically review the available literature regarding treatment outcomes with IVIg in AIBD patients. The predefined search strategy was incorporated into the following database, MEDLINE/PubMed, Embase, Scopus and Web of Science on 18 July 2022. Sixty studies were enrolled using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. The use of IVIg alone or combined with rituximab was reported in 500 patients with pemphigus, 82 patients with bullous pemphigoid, 146 patients with mucous membranes pemphigoid and 19 patients with epidermolysis bullosa acquisita. Disease remission with IVIg therapy and RTX + IVIg combination therapy were recorded as 82.8% and 86.7% in pemphigus, 88.0% and 100% in bullous pemphigoid and 91.3% and 75.0% in mucous membrane pemphigoid, respectively. In epidermolysis bullosa acquisita, treatment with IVIg led to 78.6% disease remission; no data were available regarding the treatment with RTX + IVIg in this group of patients. Among all the included patients, 37.5% experienced at least one IVIg-related side effect; the most common ones were headaches, fever/chills and nausea/vomiting. The use of IVIg with or without rituximab had a favourable clinical response in patients with AIBDs. IVIg has no major influence on the normal immune system, which makes its utilization for patients with AIBDs reasonable.
Topics: Humans; Pemphigoid, Bullous; Immunoglobulins, Intravenous; Epidermolysis Bullosa Acquisita; Pemphigus; Rituximab; Autoimmune Diseases; Skin Diseases, Vesiculobullous; Pemphigoid, Benign Mucous Membrane
PubMed: 37150538
DOI: 10.1111/exd.14829 -
Cytokine May 2020Pemphigus is a rare and life-threatening group of blistering autoimmune diseases that affects the skin and mucous membranes. There are two major subtypes of pemphigus,...
Pemphigus is a rare and life-threatening group of blistering autoimmune diseases that affects the skin and mucous membranes. There are two major subtypes of pemphigus, including pemphigus vulgaris (PV) and pemphigus foliaceus (PF); each has different clinical manifestations. Pemphigus cannot be considered as a single disease and each patient may have a specific immunological profile. There are a lot of studies available regarding the role of different cytokines in the pathogenesis of pemphigus, although the data are not coherent between different studies. In this study, a systematic review from inception to December 25, 2019, through the MEDLINE/PubMed database had been performed to address several aspects of cytokines' roles in PV and PF. As a result, 57 studies from 352 initially found records, containing 26 cytokines had met the inclusion criteria. We found different pieces of evidence in favor of increased levels of TNF-α, TGF-β, interleukin (IL)-8, IL-10, IL-12, IL-17, IL-21; while decreased levels of IL-2 and IL-23 in pemphigus patients. Regarding other cytokines, such as IFN-γ, IL-5, IL-15, IL-22, there are controversial results. Different studies suggested the association of TNF-α and IL-6 with disease activity and autoantibodies values. However, there is uncertainty regarding the role of IFN-γ, IL-2, IL-15, IL-27, and IL-33. Treatments with immunosuppressive agents may decrease IL-1, IL-6, IL-8, IFN-γ, IL-33, IL-17 levels. In conclusion, cytokines are deeply involved in PV and PF pathogenesis, and targeting specific cytokines may lead to development of more promising treatments for pemphigus.
Topics: Animals; Autoantibodies; Autoimmune Diseases; Cytokines; Humans; Pemphigus
PubMed: 32058276
DOI: 10.1016/j.cyto.2020.155026 -
Cureus Apr 2024Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder characterized by the loss of intraepithelial adhesion, affecting the skin and mucous membranes. Both... (Review)
Review
Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder characterized by the loss of intraepithelial adhesion, affecting the skin and mucous membranes. Both males and females are affected, although it predominantly affects females in their fifth and sixth decades of life. Approximately 1.4 to 3.7% of PV cases occur in the pediatric population (≤18 years of age), and may be classified into childhood/pediatric PV, which affects individuals under 12 years old, and juvenile/adolescent PV, affecting those between 12 and 18 years old. Due to its rare occurrence in children and adolescents, there is often a delay in diagnosis and treatment in this age group. A systematic literature search was conducted on MEDLINE/PubMed, Web of Science, EMBASE, SCOPUS, and Cochrane Library databases to evaluate the efficacy of rituximab (RTX) in childhood and juvenile PV patients. The Joanna Briggs Institute (JBI) Critical Appraisal Checklist was employed to assess the risk of bias in case reports and series, while the Cochrane ROBINS-I tool was utilized for evaluating observational studies or non-randomized intervention studies. A total of 18 studies encompassing 46 juvenile or childhood PV patients in the pediatric and adolescent age groups were included for qualitative synthesis. The studies included nine case reports, two case series, five retrospective studies, one prospective study, and one open-label pilot study. Almost all cases of childhood and juvenile PV achieved either complete or partial remission after undergoing RTX treatment during the final follow-up periods. Furthermore, most cases reported no relapse, and only minor adverse events were noted in the RTX treatment group. Despite its potential benefits, the utilization of RTX in pediatric patients raises concerns due to the scarcity of evidence and the absence of controlled studies specific to this age group. Further exploration is necessary to establish a standardized treatment regimen for RTX in pediatric PV, which involves identifying the optimal dosage, frequency, treatment cycle duration, and maintenance therapy duration.
PubMed: 38752055
DOI: 10.7759/cureus.58288