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Dermatologic Therapy Nov 2021Since the emergence of the new coronavirus disease 19 (COVID-19) pandemic, there has been a concern for the patients with chronic autoimmune diseases including...
Since the emergence of the new coronavirus disease 19 (COVID-19) pandemic, there has been a concern for the patients with chronic autoimmune diseases including dermatological conditions over the potential exacerbation of these underlying conditions after infection with severe acute respiratory syndrome coronavirus-2 (SARS-CoV2). We performed a systematic review to evaluate presentations, postinfection change in the manifestation, diagnosis, and management of flare-ups of underlying dermatologic disease in patients with COVID-19. A total of 17 articles were recovered reporting on flare-ups of dermatological disease including pemphigus vulgaris, psoriasis, subacute cutaneous lupus erythematosus, acrodermatitis continua of Hallopeau, systemic sclerosis sine scleroderma, and Sézary syndrome (SS). Out of these, psoriasis and alopecia areata were the most common conditions. However, most cases of psoriasis could have been attributed to either antimalarial agents that were initially used for the treatment of COVID-19 or discontinuation of treatment following SARS-CoV2 infection.
Topics: COVID-19; Humans; Pandemics; Psoriasis; RNA, Viral; SARS-CoV-2
PubMed: 34453380
DOI: 10.1111/dth.15113 -
Diagnostics (Basel, Switzerland) Aug 2021Desquamative Gingivitis (DG) comprises heterogeneous clinical manifestations of numerous immune-mediated muco-cutaneous diseases. Optical Coherence Tomography (OCT) has...
Desquamative Gingivitis (DG) comprises heterogeneous clinical manifestations of numerous immune-mediated muco-cutaneous diseases. Optical Coherence Tomography (OCT) has been proposed as a valuable diagnostic support even if, to date, there are no standardized OCT-diagnostic patterns applicable to DGs. A systematic review was performed to detect existing data on in vivo OCT diagnostic patterns of the most common immune-mediated DGs (i.e., pemphigus vulgaris, mucous membrane pemphigoid and oral lichen planus). It has been found that OCT exhibits specific patterns that address the diagnosis of DG by pemphigus vulgaris (i.e., intraepithelial unilocular blister, reduced epithelial thickness, presence of acantholytic cells in the blister) and by mucous membrane pemphigoid (i.e., subepithelial multilocular blister, presence of inflammatory infiltrate), but not by oral lichen planus. These patterns could offer an attractive diagnostic OCT framework to support the clinical preliminary assessment and monitoring of these complex pathological conditions.
PubMed: 34441387
DOI: 10.3390/diagnostics11081453 -
Experimental Dermatology Feb 2022Pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering disease characterised by cell-cell detachment or acantholysis. The mechanisms which follow antibody... (Review)
Review
Pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering disease characterised by cell-cell detachment or acantholysis. The mechanisms which follow antibody (Ab) binding and culminate in acantholytic changes and skin/mucosal blistering have not been fully clarified. Current treatment strategies are not specific to PV pathophysiology and although life-saving, harbour considerable side effects. We aimed to systematically assess the molecules amenable to targeted treatments that follow Ab binding and are associated with PV acantholysis. The resulting scoping review was conducted under PRISMA-ScR guidelines with clear inclusion and exclusion criteria and focused specifically on kinases, caspases, proteases, hydrolytic enzymes and other molecules of interest postulated to take part in the pathophysiology of PV. The review process resulted in the identification of 882 articles, of which 56 were eligible for qualitative synthesis. From the included articles, the majority (n = 42) used PV-IgG as the pathogenic agent, mainly via in vitro (n = 16) and in vivo (n = 10) models. Twenty-five molecules were found to play a pathogenic role in PV, including uPA, ADAM10, EGFR, Src, PKC, cdk2, ERK, PLC, calmodulin, NOS, p38MAPK and caspase-3. Selective inhibition of these molecules resulted in varying degrees of reduction in acantholysis and blistering. The pathogenic molecules identified in this review represent potential candidates for clinical translation.
Topics: Acantholysis; Autoantibodies; Blister; Humans; Pemphigus; Signal Transduction; p38 Mitogen-Activated Protein Kinases
PubMed: 34435386
DOI: 10.1111/exd.14453 -
Immunopharmacology and Immunotoxicology Oct 2021Rituximab is a FDA-approved monoclonal antibody for adults with moderate to severe potentially life-threatening pemphigus vulgaris. Recent studies have focused on...
BACKGROUND
Rituximab is a FDA-approved monoclonal antibody for adults with moderate to severe potentially life-threatening pemphigus vulgaris. Recent studies have focused on assessments of efficacy and safety of low-dose rituximab (<2 gram in each cycle).
METHOD
Databases were searched from 2010 to 2020 (last update: 1 June 2020).
RESULT
Nine studies were entered; including180 cases (92: women, 88: men, age range: 9-83 years). The dosages of each Rituximab cycle varied between ultra-low-dose (≤500 mg for a cycle, either multiple infusions or a single infusion), low-dose (2 × 375 mg/m or 2 × 500 mg) and modified-dose (3 × 375 mg/m or 3 × 500 mg). The efficacy and safety of Rituximab in the studies are known by the recovery time, relapse time, and side events. According to the studies, 2 × 500 can lead to complete remission in a broad range, from 35 to 82%. These differences might be explained by different end-points and variable cumulative corticosteroid dosage after RTX administration. Although the studies showed that low dose RTX is efficient, there are some controversies regarding the choosing low-dose for severe patients.
CONCLUSION
Considering the effectiveness of low-dose, intermediate dose, and ultra-low-dose protocols of Rituximab in inducing remission in pemphigus disease and considering factors such as cost of therapy, and the need to induce a minimum of immunosuppression for a minimum duration in the COVID-19 pandemic, suggested to use low-dose Rituximab protocol (2 infusions of 500 mg Rituximab: interval of 2 weeks) to induce the remission in mild-to-moderate pemphigus patients.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; COVID-19; Child; Female; Humans; Immunocompromised Host; Immunologic Factors; Male; Middle Aged; Pemphigus; Remission Induction; Risk Factors; Rituximab; SARS-CoV-2; Treatment Outcome; Young Adult
PubMed: 34287098
DOI: 10.1080/08923973.2021.1953063 -
Dermatology (Basel, Switzerland) 2022The common inflammatory scalp diseases, such as psoriasis, seborrheic dermatitis, lichen planopilaris, discoid lupus erythematosus, contact dermatitis, or pemphigus may... (Review)
Review
BACKGROUND
The common inflammatory scalp diseases, such as psoriasis, seborrheic dermatitis, lichen planopilaris, discoid lupus erythematosus, contact dermatitis, or pemphigus may share similar clinical features.
OBJECTIVE
To identify and systematically review the available evidence on the accuracy of trichoscopy in inflammatory scalp disorders.
METHODS
A systematic review was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 58 articles were included in the analysis.
RESULTS
The following trichoscopy features were found to show the highest specificity for the respective diseases: in psoriasis: diffuse scaling, simple and twisted red loops, red dots and globules, and glomerular vessels; in seborrheic dermatitis: atypical vessels, thin arborizing vessels, and structureless red areas; in discoid lupus erythematosus: follicular plugs and erythema encircling follicles; in lichen planopilaris: milky red areas or fibrotic patches; in contact dermatitis: twisted red loops; in pemphigus foliaceus: white polygonal structures and serpentine vessels; in pemphigus vulgaris: red dots with whitish halo and lace-like vessels; and in dermatomyositis: lake-like vascular structures.
LIMITATIONS
Different nomenclature and variability in parameters, which were analyzed in different studies.
CONCLUSION
This systemic analysis indicates that trichoscopy may be used with high accuracy in the differential diagnosis of inflammatory scalp diseases.
Topics: Dermatitis, Contact; Dermatitis, Seborrheic; Dermoscopy; Humans; Lichen Planus; Lupus Erythematosus, Discoid; Pemphigus; Psoriasis; Scalp; Scalp Dermatoses
PubMed: 34265772
DOI: 10.1159/000517516 -
Expert Opinion on Biological Therapy Dec 2021: This review assesses current evidence supporting dose de-escalated rituximab therapy in pemphigus vulgaris, compared to standard protocols. Primary outcome measures...
: This review assesses current evidence supporting dose de-escalated rituximab therapy in pemphigus vulgaris, compared to standard protocols. Primary outcome measures were remission and relapse rates. Adverse effects, cumulative steroid dosages, and serological markers of disease activity were also reported.: A literature search was performed to look for reports describing the use of de-escalated rituximab therapy in pemphigus vulgaris. Results from heterogenous studies showed a large variation in remission and relapse rates. Complete remission rates from de-escalated treatment ranged from 41.7 to 100.0%, while rates in the control groups ranged from 60.0 to 90.9%. Relapse rates varied from 8.0 to 81.3% in the de-escalated group and from 0.0 to 72.4% in the control group. Of the 165 patients included in this report, only two major adverse effects were reported.: Overall, dose de-escalated rituximab protocols reported to date appear effective and safe. However, it is unclear if treatment effect parallels that of standard regimens in regard to disease control in the long term. A lower limit of effective dosing for rituximab in pemphigus vulgaris has not yet been reached or defined. The role for and timing of repeated cycles of low-dose rituximab therapy require further exploration.
Topics: Antibodies, Monoclonal; Antineoplastic Agents; Humans; Immunologic Factors; Pemphigus; Rituximab; Treatment Outcome
PubMed: 34092162
DOI: 10.1080/14712598.2021.1937118 -
Orvosi Hetilap Mar 2021Összefoglaló. Bevezetés: Az időalku (time trade-off) egy nemzetközileg széles körben alkalmazott életminőség- és egészséghasznosság-mérési módszer. Az...
UNLABELLED
Összefoglaló. Bevezetés: Az időalku (time trade-off) egy nemzetközileg széles körben alkalmazott életminőség- és egészséghasznosság-mérési módszer. Az időalkuval mért hasznosságértékek az egészségügyi technológiák költséghatékonysági vizsgálatakor az életminőséggel korrigált életév (quality-adjusted life year, QALY) mutatószám számítására használhatók. Kevés ismerettel rendelkezünk arról, hogy Magyarországon milyen betegségekben állnak rendelkezésre időalkuval mért hasznosságértékek. Célkitűzés: Célunk szisztematikus irodalmi áttekintés keretében összefoglalni és katalogizálni az időalku módszerrel mért hasznosságértékeket közlő publikációkat Magyarországon. Módszer: 2020 januárjában szisztematikus folyóirat-keresést végeztünk a PubMed, Web of Science és Matarka elektronikus adatbázisokban. Beválogatási kritériumaink a következők voltak: (1) eredeti közlemények, melyek (2) időalku módszerrel mértek hasznosságértéket, és (3) magyarországi mintán mért adatokat közöltek. Eredmények: 9 eredeti közleményt válogattunk be, amelyek összesen 7 krónikus betegségben (Crohn-betegség, időskori maculadegeneratio, krónikus migrén, pemphigus, psoriasis, primer dysmenorrhoea és rheumatoid arthritis) 23 egészségi állapot hasznosságát határozták meg. A kutatások mintanagysága 108 és 1996 fő között alakult. Két kutatás betegcsoportokat vizsgált, kettő az általános populációt, és három vizsgálatban szerepelt mindkettő. Hat kutatás használta a hagyományos időalku valamelyik formáját, egy pedig az összetett időalku módszert. Egy kutatásban szerepelt 'rosszabb a halálnál' válaszlehetőség. A leggyakrabban alkalmazott időtáv a standard 10 év volt (71%). Az egyes állapotok hasznosságának átlaga 0,34 (kezeletlen pemphigus vulgaris) és 0,94 (enyhe primer dysmenorrhoea) között változott. A 'non-traderek' aránya az egyes kutatásokban 0 és 29% között változott. Következtetések: Egyre több krónikus betegségben elérhetők a magyar társadalom vagy betegek preferenciáin alapuló egészséghasznosság-értékek. A hazai időalku-vizsgálatok többsége megfelel a nemzetközi minőségi követelményeknek. Az időalku módszer alkalmazása javasolt más krónikus állapotokban is, a felmért hasznosságértékek segíthetik az egészségügyi technológiákkal kapcsolatos finanszírozói döntéshozatalt. Orv Hetil. 2021; 162(14): 542-554.
INTRODUCTION
Time trade-off (TTO) is a widely used method to assess health-related quality of life and health utilities for economic evaluations of health technologies. Little is known about the use of TTO in the Hungarian context.
OBJECTIVE
To systematically summarize the existing literature on the method in Hungary.
METHOD
In January 2020, we conducted a systematic literature search in three electronic databases (MEDLINE, Web of Science and the Hungarian Periodicals Table of Contents Database). Our inclusion criteria were: (1) original publications, which (2) measured utilities by using TTO, (3) from a Hungarian sample.
RESULTS
Nine publications containing seven original studies were included that reported utilities for 23 different health states in seven chronic diseases (age-related macular degeneration, chronic migraine, Crohn's disease, pemphigus, primary dysmenorrhoea, psoriasis and rheumatoid arthritis). Sample sizes ranged from 108 to 1996 respondents. Two studies used general population samples, another two used patient groups and three studies used both. Six studies used a form of conventional TTO and one used composite TTO method. The most frequent timeframe was 10 years (71%). The lowest mean utility was 0.34 (uncontrolled pemphigus vulgaris), while the highest was 0.94 (mild primary dysmenorrhoea). The overall proportion of non-traders ranged between 0 and 29% across studies.
CONCLUSIONS
A growing number of studies are using TTO to assess utilities for chronic conditions from the general population or patients in Hungary. The majority of Hungarian TTO studies have met international quality standards. The assessment of TTO utilities is recommended also in other chronic conditions to assist health technology assessment. Orv Hetil. 2021; 162(14): 542-554.
Topics: Chronic Disease; Facilities and Services Utilization; Humans; Hungary; Quality of Life; Technology Assessment, Biomedical; Time Factors
PubMed: 33784247
DOI: 10.1556/650.2021.32056 -
Journal of Oral Pathology & Medicine :... Sep 2021To assess the prevalence of oral mucosal lesions in patients with Pemphigus Vulgaris. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To assess the prevalence of oral mucosal lesions in patients with Pemphigus Vulgaris.
METHODS
Observational studies reporting the prevalence of oral lesions in pemphigus vulgaris patients, without restriction to language and year of publication, were selected in a two-phase process. Search strategies were applied to PubMed, Scopus, Livivo, Web of Science, LILACS, Google Scholar, and OpenGrey databases. Articles assessing the prevalence of oral lesions in patients with conditions other than pemphigus vulgaris were excluded. Risk of bias analysis was performed using the Joanna Briggs Institute's Critical Appraisal Checklist for Studies Reporting Prevalence Data. Synthesis of results was calculated by the software R Statistics version 4.0.2 (The R Foundation). Confidence in cumulative evidence was assessed with the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) criteria.
RESULTS
From 1957 studies identified, 40 were included in qualitative synthesis and 38 in meta-analyses. The pooled prevalence of patients with oral lesions solely or concurrent with other mucocutaneous lesions was 90.3%. The prevalence of patients with exclusive oral mucosal lesions was 50.8%. Risk of bias was considered low, and the certainty of evidence was very low.
CONCLUSION
Oral lesions were present in approximately nine out of 10 patients with pemphigus vulgaris. The oral mucosa was the most common site of disease onset. Further longitudinal studies are urged to assess the prevalence of oral lesions at different disease stages.
Topics: Humans; Mouth Mucosa; Pemphigus; Prevalence
PubMed: 33713362
DOI: 10.1111/jop.13167 -
Anais Brasileiros de Dermatologia 2021
Meta-Analysis
Topics: Humans; Lupus Erythematosus, Systemic; Pemphigus
PubMed: 33632594
DOI: 10.1016/j.abd.2020.07.009 -
Journal of Cosmetic Dermatology May 2021Hailey-Hailey disease is a rare disorder characterized by recurrent painful blistering, erosions, maceration in the intertriginous regions. Botulinum toxin has been used...
BACKGROUND
Hailey-Hailey disease is a rare disorder characterized by recurrent painful blistering, erosions, maceration in the intertriginous regions. Botulinum toxin has been used in the treatment of Hailey-Hailey disease.
AIMS
This study aimed to examine all published articles on botulinum toxin in the treatment of Hailey-Hailey disease, and to evaluate its efficacy and safety.
METHODS
PubMed, Embase, Cochrane Library, and Web of Science were used to identify eligible articles on August 8, 2020. The searching strategy was "(Hailey Hailey or Hailey-Hailey or pemphigus) and botulinum."
RESULTS
Sixteen articles including 38 patients described the use of botulinum toxin in treating Hailey-Hailey disease. Only one case had no response, while the other patients all had partial or complete remission. No side effects were reported. Nine articles including 10 patients mainly described other treatment options, and the patients were only treated with botulinum toxin previously. Their responses to botulinum toxin were limited: one was mild improvement, one was partial response, and the other eight failed.
CONCLUSION
Botulinum toxin is not almighty, but a promising alternative option. We recommend botulinum toxin as an adjuvant or supplemental treatment modality for severe and recalcitrant Hailey-Hailey disease. Larger studies are warranted to confirm its efficacy, safety, long-term effects, and cost performance.
Topics: Botulinum Toxins, Type A; Humans; Pemphigus, Benign Familial
PubMed: 33533135
DOI: 10.1111/jocd.13963