Pemphigus Disease Area Index Score Research Articles

Establishing minimal clinically important differences forthe Pemphigus Disease Area Index.

Pemphigus is a rare autoimmune blistering disease with potentially life-threatening consequences. Establishing minimal clinically important differences (MCIDs) for disease severity scores like the Pemphigus Disease Area Index (PDAI) is crucial for assessing treatment efficacy. To calculate MCIDs for both improvement and deterioration in PDAI scores in patients with pemphigus vulgaris (PV) and pemphigus foliaceus (PF), using the anchor-based method. A total of 41 patients with pemphigus were recruited, with 35 meeting the MCID analysis criteria. The anchor-based method was used to calculate MCIDs for PDAI scores against the 15-point Likert scale and the Physician Global Assessment visual analogue scale (PGA-VAS) anchors. Receiver operating characteristic curves were employed to determine optimal MCID cutpoints with the highest Youden Index (J). The 15-point Likert scale scores the change in disease severity spanning from -7 to +7, designed to quantify the extent of disease improvement/deterioration since the preceding visit. The MCID for improvement in PDAI activity scores was 2.65 points using the 15-point Likert scale (78.7% correct classification; sensitivity 75.9%; specificity 73.5%) and 2.5 points using the PGA-VAS as the anchor (78.0% correct classification; sensitivity 84.4%; specificity 68.2%). Given the slightly higher correct classification rate using the 15-point Likert scale anchor, the MCID of 2.65 points was selected for PDAI activity score improvement. In contrast, the MCID for deterioration consistently remained at 2.5 points for the 15-point Likert scale anchor (81.0% correct classification; sensitivity 72.7%; specificity 81.0%) and 2.5 points for the PGA-VAS anchor (70.9% correct classification; sensitivity 69.6%; specificity 76.9%). This study marks the inaugural attempt at MCID determination for PDAI scores in pemphigus, filling a critical knowledge gap. The study's calculated MCIDs provide essential benchmarks for clinical trials, treatment evaluation and research design optimization. Future studies should explore international collaborations, to examine potential cross-cultural variations in MCIDs.

TARC/CCL17 reflects the severity of pemphigus (pemphigus vulgaris and pemphigus foliaceus) at the initial presentation

AbstractBackgroundPemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases targeting desmoglein 3 (Dsg3) or desmoglein 1 (Dsg1). The current scoring system, pemphigus disease area index (PDAI), has limitations including inter‐ and intra‐evaluator variability, as well as a time‐consuming evaluation process.ObjectivesTo identify objective and quantitative surrogate markers for assessing disease severity in PV and PF.MethodsEleven PV and PF patients were included. PDAI scores and candidate biomarkers [anti‐Dsg3 antibody, anti‐Dsg1 antibody, thymus and activation‐regulated chemokine (TARC)/CCL17, immunoglobulin E (IgE) levels and eosinophil counts] were measured before oral corticosteroid treatment. Pearson's correlation coefficient was used for correlations between PDAI and candidate biomarkers.ResultsSerum TARC/CCL17 levels significantly correlated with PDAI scores in PV and PF (R = 0.72, p = 0.02), while anti‐Dsg3 antibody levels correlated with PDAI scores for PV (R = 0.86, p = 0.012). No significant correlations were observed for anti‐Dsg1 antibody, eosinophil counts or IgE levels.ConclusionsSerum TARC/CCL17 may be a quantitative and unbiased disease biomarker in pemphigus patients, although further studies involving larger patient cohorts are needed.

Clinical and immunological predictors of post-rituximab paradoxical pemphigus flare: A prospective cohort study.

Background Paradoxical flare of pemphigus following rituximab infusion has been reported previously, however, its incidence or risk factors have not been studied in detail. Objectives To evaluate the clinical and immunological predictors associated with post-rituximab paradoxical pemphigus flare. Materials and Methods This was a prospective cohort study including adult patients with pemphigus vulgaris or foliaceus who were treated with rituximab. Patients were administered 1000 mg of intravenous rituximab on days 0 and 14 (Rheumatoid arthritis (RA) protocol), with or without oral prednisolone and/or conventional immunosuppressive agents. Baseline clinical and immunological predictors of post-rituximab pemphigus flares were assessed. Results Fifty patients (mean age 40.44 ± 12.36 years) with a mean pemphigus disease area index (PDAI) score of 27.8 ± 15.48 were administered rituximab. Post-rituximab flare occurred in 10 (20%) patients after a mean of 14.1 ± 4.33 days after the first rituximab infusion. The mean baseline PDAI score (36.4 ± 11.7 vs. 25.6 ± 15.7, P = 0.02) and serum anti-Dsg1 levels (1216.8 ± 850.1 vs. 592 ± 562.12 RU/mL, P = 0.03) were statistically significantly higher in patients experiencing a flare. Using ROC-curve analysis, a PDAI score of 328 (OR 8.3, 95% CI 1.5-44.7) was 80% sensitive and 67.5% specific in predicting post-rituximab flare, while serum anti-Dsg1 level of 31137.78 RU/ml had a sensitivity of 60% and specificity of 85%. There was no significant difference in terms of affected body surface area, type of pemphigus, starting prednisolone dose, oral immunosuppressive adjuvant, serum anti-Dsg3, serum anti-AchRM3, and peripheral CD19+ B cell population. Limitations Our study is limited by a relatively small sample size. Immunological factors were not evaluated at the time of pemphigus flare. Though these unexpected pemphigus flares are likely to be associated with rituximab infusion, the possibility of spontaneous disease exacerbation cannot be entirely excluded. Conclusions Patients with more severe pemphigus or high serum anti-Dsg1 are at risk of post-rituximab paradoxical flare, and may benefit from rituximab administration under close monitoring.

Rituximab treatment for refractory and moderate-to-severe pemphigus patients in the pandemic era: A single-center retrospective study

Abstract Background: The management of refractory pemphigus in the pandemic conditions is challenging. There are limited data comparing the safety of rituximab and other immunosuppressive therapies during the COVID-19 pandemic. Objectives: We evaluated the safety and efficacy of rituximab and conventional treatments in patients with pemphigus who received treatment during the COVID-19 pandemic period. Methods: The patients with pemphigus treated with immunosuppressive treatments in a university hospital between September 2020 and May 2023 were retrospectively evaluated. All patients initially received conventional treatments. Fifteen of them who were refractory to prior treatments or could not use prednisolone due to adverse effects received rituximab. Eighteen patients were treated with only conventional treatments. Results: Thirty-three patients with a mean age of 50 ± 15 years were included in the study. Twenty-two of the patients (66.7%) were female. The baseline mean pemphigus disease area index (PDAI) score of the patients was 37 ± 34 (range: 4–168). The mean follow-up time was 13.8 ± 6.8 months. The mean PDAI scores were significantly lower after treatment in both rituximab and conventional treatment groups (P: 0.001, P < 0.001; respectively). In the rituximab group, the monthly mean prednisolone dose was statistically reduced after rituximab treatment compared to before treatment (P < 0.001). There was no statistically significant difference in the relative odds of COVID-19 positivity or death between the groups (3.06 (95% [confidence interval (CI): 0.68–13.79]; 0.57 [95% CI: 0.05–7.00], respectively). Of 11 patients diagnosed with COVID-19, one patient (1/7, 14.3%) in the rituximab group and two patients (2/4, 50%) in the conventional treatment group died from SARS-CoV-2. Conclusion: We observed that rituximab treatment was effective and safe for treatment-resistant moderate-to-severe pemphigus patients during the COVID-19 pandemic.

Sexual quality of life in patients with pemphigus: A case-control study.

Pemphigus, an autoimmune blistering disease that affects the skin and mucous membranes, significantly impairs the quality of life (QoL) of affected individuals. While there are a variety of QoL measurement tools available for assessing this disease, there is a lack of studies that specifically evaluate the sexual QoL of patients with pemphigus. This case-control study aims to investigate the impact of the disease on sexual activity as well as its overall effect on QoL. Fifty pemphigus patients, who were referred to the Dermatology Department at the University Hospital of Brescia in the period March 2019-September 2021, completed several QoL surveys, including the 36-item Short Form Health survey (SF-36), the 12-Item General Health Questionnaire (GHQ-12), the Autoimmune Bullous Disease Quality of Life (ABQOL) and either the International Index of Erectile Function (IIEF) or the Female Sexual Function Index (FSFI). The severity of the disease was assessed using the Pemphigus Disease Area Index (PDAI). Differences in QoL surveys between the case and control groups were analysed using either the t-test or the Wilcoxon-Mann-Whitney test. The correlation between QoL surveys in pemphigus patients and disease severity were analysed using Spearman's coefficient (r). The results revealed a marked impairment in overall QoL among patients with pemphigus compared to the healthy control subjects. Significant differences were observed in various domains of QoL, including physical health, mental well-being, social functioning and, notably, sexual health. Furthermore, disease severity as evaluated by the PDAI showed correlations with specific aspects of health status, and disease-specific QoL demonstrated associations with nearly all domains of health status. No significant correlations were found between sexual activity, mucosal involvement or steroid therapy and PDAI scores or disease-specific QoL measures. These findings emphasize the significant impact of pemphigus on patients' well-being, with particular attention to the impaired sexual activity.

Environmental factors in autoimmune bullous diseases with a focus on seasonality: new insights.

Autoimmune bullous diseases are a heterogeneous group of rare conditions clinically characterized by the presence of blisters and/or erosions on the skin and the mucous membranes. Practically, they can be divided into two large groups: the pemphigoid group and the pemphigus group, depending on the depth of the autoimmune process on the skin. A family history of autoimmune diseases can often be found, demonstrating that genetic predisposition is crucial for their development. Moreover, numerous environmental risk factors, such as solar radiation, drugs, and infections, are known. This study aimed to evaluate how seasonality can affect the trend of bullous pemphigoid and pemphigus vulgaris, especially considering the number of hospitalizations recorded over the course of individual months. The total number of hospitalizations in the twelve months of the year was evaluated. Moreover, blood chemistry assay and, for some patients, enzyme-linked immunosorbent assay were executed to evaluate antibodies. Regarding the severity of the disease, the bullous pemphigoid area index and the pemphigus disease area index score systems were used. Results showed a complex interplay between environmental factors such as seasons and autoimmune conditions.

The Importance of Patient-Focused Drug Development in Pemphigus and Pemphigoid

The Importance of Patient-Focused Drug Development in Pemphigus and Pemphigoid

How to decrease systemic corticosteroids in pemphigus patients under rituximab.

How to decrease systemic corticosteroids in pemphigus patients under rituximab.

Assessment of anti-desmoglein antibodies levels and other laboratory indexes as objective comprehensive indicators of patients with pemphigus vulgaris of different severity: a single-center retrospective study.

The aim of this study is to assess the utility of anti-desmoglein (Dsg) antibodies levels, hematological biomarkers levels, the albumin to globulin (A/G) ratio, blood lipids levels, and lymphocyte subpopulation percentages as objective laboratory indicators of disease severity of pemphigus vulgaris (PV). A retrospective study of 187 PV patients with 256 medical records between January 2013 and December 2020. PV patients were divided into three groups by disease severity according to the pemphigus disease area index (PDAI) score: mild (0-8), moderate (9-24), and severe (≥ 25). The levels of anti-Dsg antibodies, hematological biomarkers, A/G ratio, blood lipids, and the percentage of lymphocyte subpopulations were measured. We assessed the correlations of quantitative variables by Pearson correlation (r). Multivariable linear regression was used to identify the variables associated with the disease severity of PV (PDAI score). The results show that the levels of Dsg1 (r = 0.294, P < 0.001) and Dsg3 (r = 0.206, P = 0.011), monocyte count (r = 0.210, P = 0.001), neutrophil-to-lymphocyte ratio (NLR) (r = 0.123, P = 0.049), and platelet-to-lymphocyte ratio (PLR) (r = 0.170, P = 0.006) were positively correlated with the PDAI score. However, the A/G ratio (r = -0.399, P < 0.001), and the levels of total serum cholesterol (r = -0.140, P = 0.026) and HDL (r = -0.143, P = 0.023) were negatively correlated with the PDAI score. Multiple linear regression showed that the factors associated with the PDAI score were higher level of anti-Dsg1 antibody (P = 0.001), a higher NLR (P = 0.005), and a lower A/G ratio (P < 0.001). The linear regression equation was Y(PDAI) = 32.798 + 0.058X(Dsg1) + 0.846 X(NLR)-16.472 X(A/G) (R2 = 0.586). Therefore, high levels of anti-Dsg1 antibody and NLR combined with a low A/G ratio could explain the PDAI score. These findings might provide a more comprehensive and objective evaluation system for reflecting the disease severity of PV based on laboratory indicators.

Treatment of pemphigus vulgaris and foliaceus with efgartigimod, a neonatal Fc receptor inhibitor: a phase II multicentre, open‐label feasibility trial*

Pemphigus vulgaris and pemphigus foliaceus are potentially life-threatening autoimmune disorders triggered by IgG autoantibodies against mucosal and epidermal desmogleins. There is an unmet need for fast-acting drugs that enable patients to achieve early sustained remission with reduced corticosteroid reliance. To investigate efgartigimod, an engineered Fc fragment that inhibits the activity of the neonatal Fc receptor, thereby reducing serum IgG levels, for treating pemphigus. Thirty-four patients with mild-to-moderate pemphigus vulgaris or foliaceus were enrolled in an open-label phase II adaptive trial. In sequential cohorts, efgartigimod was dosed at 10 or 25mgkg-1 intravenously with various dosing frequencies, as monotherapy or as add-on therapy to low-dose oral prednisone. Safety endpoints comprised the primary outcome. The study is registered at ClinicalTrials.gov (identifier NCT03334058). Adverse events were mostly mild and were reported by 16 of 19 (84%) patients receiving efgartigimod 10mgkg-1 and 13 of 15 (87%) patients receiving 25mgkg-1 , with similar adverse event profiles between dose groups. A major decrease in serum total IgG and anti-desmoglein autoantibodies was observed and correlated with improved Pemphigus Disease Area Index scores. Efgartigimod, as monotherapy or combined with prednisone, demonstrated early disease control in 28 of 31 (90%) patients after a median of 17days. Optimized, prolonged treatment with efgartigimod in combination with a median dose of prednisone 0·26mgkg-1 per day (range 0·06-0·48) led to complete clinical remission in 14 of 22 (64%) patients within 2-41weeks. Efgartigimod was well tolerated and exhibited an early effect on disease activity and outcome parameters, providing support for further evaluation as a therapy for pemphigus.

Comorbid diseases associated with pemphigus: a case-control study.

Pemphigus has been associated with physical and psychiatric comorbid diseases. This study aims to further investigate these associations in patients with pemphigus, and to analyze the relationships of comorbid conditions with sex and age, pemphigus disease area index score, diagnostic delay and cutaneous/mucous involvement. Patients with pemphigus were matched by age, gender and area of residence with eight controls each. The odds of comorbid conditions in patients vs. matched controls was determined using univariate conditional logistic regression models. Comorbid diseases significantly associated with the diagnosis of pemphigus at P<0.05 in univariate models were subsequently included in a multivariable conditional logistic regression model with a forward procedure. The study sample included 163 patients with pemphigus. Cardiovascular diseases, hyperlipidemia, autoimmune thyroid disorders, dermatological autoimmune/inflammatory conditions and cancer were the most prominent conditions at the time of diagnosis. In the multiple conditional regression analysis, the two diagnoses independently associated with patients with pemphigus were cancer and dermatological autoimmune/inflammatory conditions. In sensitivity analyses excluding four patients with paraneoplastic pemphigus, these associations were still significant. Cancer and dermatological autoimmune/inflammatory conditions may represent possible triggering conditions for pemphigus and should be considered as early warning signs for the disease.

Punctate Pattern and Pemphigus: Is There Any Evidence of Punctate Pattern Among Iranian Patients?

To examine the prevalence of this novel pattern among Iranian patients with pemphigus and peruse the relationship between the presence of a punctate pattern with clinical severity of disease and histopathological findings. One hundred recently diagnosed patients with pemphigus were enrolled. DIF evaluation and routine light microscopy were performed on their biopsy specimens. Disease severity was determined using the Pemphigus Disease Area Index. Serum samples were collected to measure autoantibody titers using enzyme-linked immunosorbent assay. All the samples evaluated by DIF showed a continuous linear pattern of intercellular IgG deposition, whereas none of them had a punctate pattern. Despite a significant correlation between the Pemphigus Disease Area Index score and autoantibody values, no association between histopathological findings and disease severity has been found. We could not detect any punctate pattern among Iranian patients with pemphigus. The importance of this pattern in the diagnosis of pemphigus might be different among patients with different ethnic and genetic factors.

Safety, Tolerability, and Activity of ALXN1830 Targeting the Neonatal Fc Receptor in Chronic Pemphigus

Pemphigus is a debilitating IgG-mediated autoimmune disease requiring better tolerated, more targeted, and rapid onset therapies. ALXN1830 is a humanized IgG4 antibody that blocks neonatal Fc receptor interactions with IgG. A multicenter, open-label safety and tolerability phase 1b/2 trial (NCT03075904) was conducted in North America from July 2017 to January 2019 and included patients aged ≥18 years with a confirmed diagnosis of pemphigus (vulgaris or foliaceus) and active disease. Dosing included five weekly intravenous doses of ALXN1830 (10 mg/kg) and follow-up through day 112 (study termination). Pharmacokinetics, pharmacodynamics, safety, and efficacy, as evaluated by determining the change in the median pemphigus disease area index, were determined. In this pilot study of eight patients, five weekly infusions of ALXN1830 produced a rapid improvement in the pemphigus disease area index score within 14 days of the first dose. Pemphigus disease area index improvement increased further together with reductions in IgG, circulating immune complexes of IgG, and anti-desmoglein antibodies without affecting albumin, IgM, IgA, or C-reactive protein levels. ALXN1830 was well-tolerated, with headache as the most common adverse event. This study reveals the importance of neonatal Fc receptor in the biology of pemphigus and the potential for use of ALXN1830 in pemphigus treatment.

Serum IL-21 level in patients with pemphigus: Before and after treatment.

Interleukin-21 (IL-21) is believed to play a pathogenic role in the development of pemphigus. Our aim was to assess serum IL-21 levels in pemphigus patients before and after treatment. Twenty patients with pemphigus (pemphigus group) and 10 healthy controls (control group) were enrolled. Patients were assessed clinically using the Pemphigus Disease Area Index (PDAI) at presentation and 2 months after treatment. Serum samples were collected to measure serum IL-21 levels by enzyme-linked immunosorbent assay (ELISA), at presentation and after 2 months of treatment. The mean age of the participants was 41.9 ± 13.5 years (7 men and 13 women). The PDAI score at presentation was 37.35 ± 14.206, which decreased significantly after treatment to 11.70 ± 6.729 (P-value <.001). On the other hand, serum IL-21 levels increased after treatment (589.03 ± 131.86 ng/L at first presentation vs 627.10 ± 304.05 ng/L after treatment: P-value .444). No significant correlation was found between the PDAI severity scores and serum IL-21 levels at the first presentation or 2 months after treatment.

Estimated cut-off values for pemphigus severity classification according to pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and anti-desmoglein 1 autoantibodies

BackgroundPemphigus is a potentially fatal disease if left untreated. Valid scoring systems and defined cut-off values for classification of patients would help with better management through specified pharmaceutical and non-pharmaceutical treatments.MethodsIn this study, pemphigus patients who were receiving immunosuppressive treatments and had recent disease relapse were recruited for examination of pemphigus disease area index(PDAI), autoimmune bullous skin disorder intensity score (ABSIS), physician global assessment (PGA), autoimmune bullous disease quality of life (ABQoL), anti-desmoglein 1 (anti-Dsg1), and anti-Dsg3 autoantibody titers from December-2017 to February-2018. Cut-off values were estimated using model-based clustering classification and the 25th and 75th percentiles approach, performed separately for the exclusive cutaneous, exclusive mucosal, and mucocutaneous groups.ResultsIn the 109 included patients, the 25th and 75th percentiles cut-offs were 6.2 and 27 for PDAI score, and 4 and 29.5 for ABSIS score. The model-based analysis resulted in two groups (cut-point:15) for PDAI score, and three groups (cut-points:6.4 and 31.5) for ABSIS score. The groups were significantly different for the PDAI, ABSIS, PGA, and ABQoL values. Based on anti-Dsg1 autoantibody values, the model-based analysis cut-point was 128 and the 25th and 75th percentiles cut-offs were 98 and 182. Anti-Dsg3 autoantibody values did not differentiate between pemphigus severity classes.ConclusionsEstimated cut-off values based on the anti-Dsg1 level, PDAI, and ABSIS scoring systems could be used to classify patients into different severity grades for better management and prognosis.

Therapeutic outcomes of pemphigus in a referral service in Northern Brazil: a retrospective study of 32 patients

Background Pemphigus are rare chronic autoimmune blistering disorders with challenging therapeutic management. Objective To investigate the therapeutic outcomes of pemphigus patients admitted to a dermatology referral service in northern Brazil. Materials and methods We conducted a retrospective analysis of 32 patients with histopathological diagnosis of pemphigus between 2010 and 2016. Clinical and epidemiological data were collected. Therapeutic outcome was evaluated according to the criteria proposed by the International Pemphigus Committee. Pemphigus Disease Area Index (PDAI) was used to quantify disease severity. Results Of the 32 patients, 68.75% had pemphigus vulgaris (PV) and 31.25% had pemphigus foliaceus (PF). Female-to-male ratio was 1:1. The average age was 49.5 years. All patients received oral prednisone as the first-line therapy. Adjuvant immunosuppressive drugs were gradually added in refractory and/or severe cases. After 24 months of treatment, disease control and complete remission rates were 37.5% and 25%, respectively. The mortality rate was 9.37%. PDAI score was significantly lower at 24 months of therapeutic follow-up (p < .0001). Conclusion Therapeutic management with corticosteroids and/or immunosuppressive drugs was able to induce disease control/complete remission in most of patients. PDAI was a useful tool for objective assessment of disease severity during therapeutic follow-up.

Comparative retrospective evaluation of efficacy of rituximab therapy with dexamethasone cyclophosphamide pulse therapy in patients of pemphigus vulgaris at a tertiary care hospital

Background: Popular treatment modalities for pemphigus vulgaris are dexamethasone cyclophosphamide pulse (DCP) therapy and rituximab. However, no previous studies are available which have compared the efficacy of the two modalities. Aim: This study aims to study the response of pemphigus vulgaris to DCP and rituximab therapy and to compare the efficacy and safety of the two modalities over a period of 1½ years. Materials and Methods: This was an observational retrospective study. The medical records of a total of 14 pemphigus vulgaris patients from July 2016 to July 2018 were retrieved for analysis who were treated with either DCP therapy or rituximab (7 in each group). Patients treated with any other modality except DCP/rituximab were excluded from the study. The pemphigus disease area index (PDAI) scores at baseline, 1 month, 6 months, 12 months, and 18 months were analyzed using statistical software (Stata Corp. 2013. Stata Statistical Software: Release 13. College Station, TX, Stata Corp. LP, Texas, USA). Outcome Measures: Time to achieve the end of consolidation phase, improvement in PDAI scores, adverse effects, and relapse. Results: Rituximab group achieved early remission; however, there was no statistically significant difference in the two groups in the time when they reached the end of consolidation phase. Repeated measure anova showed significant difference in PDAI scores within the two groups over time. One patient in DCP group relapsed following discontinuation of cyclophosphamide due to complications. Overall, the scoring of disease severity was markedly reduced in both the groups. Conclusion: Both DCP therapy and rituximab were found to be extremely and equally effective in inducing and maintaining remission.

Phosphorylated p38 mitogen-activated kinase in patients with pemphigus vulgaris: a case–controlled study

Background Although the exact pathogenic mechanisms leading to blister formation in pemphigus vulgaris (PV) have not been fully elucidated, intracellular signaling following antibody binding has been found to be necessary for inducing cell-cell dissociation. The phosphorylated p38 mitogen-activated protein kinase (P38 MAPK) signaling pathway plays a major role in the modulation of immune-mediated inflammatory responses and therefore has been linked with several autoimmune diseases. Studies showed that activation of P38 MAPK plays a role in the acantholytic process in PV through changes in the quaternary structure and cytoskeletal rearrangements. Objective To determine the levels of p38 MAPK and its substrate [MAP kinase-activated protein kinase 2 (MAPKAPK2)] in the peripheral blood mononuclear cells of patients with PV in relation to both the clinical score and the antidesmoglein 3 antibodies. Patients and methods This study included 25 Egyptian patients with PV and 25 healthy controls, who were recruited from Cairo University Hospitals. Detailed history was documented, and the severity of the diseases was determined by pemphigus disease area index score in each patient. Blood samples were obtained from all participants. Then, determination of the expression of p38 MAPK and its substrate MAPKAPK2 on peripheral mononuclear cells was done using flow cytometry, with measurement of antidesmoglein 3 antibody using enzyme-linked immunosorbent assay technique. Results We detected that P38 MAPK and MAPKAPK2 were upregulated in patients with PV in comparison with controls (P=0.024 and P=0.002, respectively). There was a positive correlation between the anti-Dsg3 antibodies and P38 MAPK (r=0.591, P=0.004) and between disease severity (pemphigus disease area index score) and P38 MAPK (r=0.617, P=0.002) as well. Conclusion This study concluded that P38 MAPK and its substrate MAPKAPK2 have a role in the pathogenesis of PV. Moreover, P38 MAPK can be used as a marker for disease activity follow-up, as it was correlated with both disease severity and autoantibody level.

Evaluation of methicillin-resistant Staphylococcus aureus infections in pemphigus vulgaris patients: cross-sectional study

Background Pemphigus vulgaris (PV) is a potentially fatal autoimmune vesiculobullous disease. Staphylococcus aureus is the most common cause of cutaneous bacterial infection in pemphigus. Methicillin-resistant S. aureus (MRSA) is a common multidrug-resistant bacterium. MRSA infection is high among dermatology inpatients and vesiculobullous diseases patients are the commonest infected.Objective This study aimed at determining the causative organisms of the secondary bacterial skin infections in PV patients, identifying MRSA, doing antibiotic sensitivity pattern for MRSA, and determining the risk factors for acquiring MRSA infection among PV patients in the locality.Patients and methods This cross-sectional study included 54 PV patients. All patients were subjected to history taking, general, and dermatological examinations. Pemphigus disease area index score was calculated. Three swabs for bacterial culture and antibiotic sensitivity testing were obtained (one from vesicle or bulla, one from pustule, and the third from the nose).Results In all, 51 (94.4%) patients showed positive cultures for S. aureus either as nasal carrier, skin colonization, or skin infection. There was overlap as some patients showed more than one type of positive culture at the same time. Of the patients, 19 out of 51 patients (37.25%) had positive cultures for MRSA. All MRSA strains were sensitive to vancomycin. Also, they had good sensitivity to trimethoprim–sulfamethoxazole, amikacin, ciprofloxacin, and erythromycin, while all MRSA strains were resistant to cefoxitin and ampicillin. Hospitalization, previous antibiotics use in the last 4 weeks prior to this study, length of hospital stay, history of hospital-acquired infections, and community-acquired infections were significantly higher among MRSA-positive patients.Conclusion There is high MRSA infection/colonization among PV patients. MRSA responds to cheap antibiotics as trimethoprim–sulfamethoxazole, amikacin, and ciprofloxacin.

Assessing the Correlation Between Disease Severity Indices and Quality of Life Measurement Tools in Pemphigus.

Pemphigus, an autoimmune blistering disease that affects the skin and mucous membranes, adversely impacts patients' quality of life (QOL). While there are various QOL measurement tools that can be used in this disease, few studies have assessed how a patient's change in disease severity can affect their QOL. This study aims to identify which disease severity index correlates best with the change in QOL. Fifty pemphigus patients completed QOL surveys with disease severity scored over two visits. QOL was assessed with the Autoimmune Bullous Disease Quality of Life (ABQOL), Dermatology Life Quality Index (DLQI), Skindex-29, and Short Form Survey 36 (SF-36). Disease severity was scored with the Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Correlations between the change in QOL scores and change in disease severity were analyzed using Spearman's coefficient (r). The change in PDAI showed a strong correlation (r = 0.60–0.79) with changes in the ABQOL, Skindex-29 symptoms (Skindex-S), and Skindex-29 functioning (Skindex-F) subscales for all patients (n = 50). For patients with mucosal disease (n = 24), the change in PDAI showed a strong correlation with changes in the ABQOL and Skindex-S subscale. For patients without mucosal disease, the change in PDAI showed a strong correlation with the Skindex-S. The change in ABSIS showed a strong correlation with Skindex-S for all patients and patients with no mucosal involvement, but showed no strong correlations for patients with mucosal involvement. The changes in PDAI always had a stronger correlation than the changes in ABSIS scores to changes in the ABQOL, DLQI, and Skindex-29 subscales, except where the PDAI and ABSIS scores were about the same for the Skindex-S subscale in patients with no mucosal involvement (r = 0.76 and r = 0.77, respectively). PDAI is superior to ABSIS in its correlation with validated QOL tools. The QOL tools that appear to be of most use in clinical trials and patient management are the Skindex-S and ABQOL.