Autoimmune Bullous Skin Disorder Intensity Score Research Articles

The Effect of Vitamin D Supplementation on Autoimmune Bullous Skin Disorder Intensity Score on Bullous Pemphigoid and Acute Kidney Injury: A Case Report

Introduction: Bullous pemphigoid is the most common bullous-type autoimmune disease. Hypovitaminosis D is often associated with autoimmune disease and found in 86,7% bullous pemphigoid patients. Vitamin D supplementation in bullous pemphigoid patients ought to ameliorate its clinical symptoms.
 Case presentation: A-58 years old male patient with bullous pemphigoid and acute kidney injury admitted with a chief complaint of infected blisters all over the body. The patient had been treated for 1 week with worsening swallowing pain and low intake. On the 12th day of hospitalization, the patient was given calcitriol supplementation of 0,25 mcg per day. There was an improvement in clinical symptoms shown on autoimmune bullous skin disorder intensity score (ABSIS) from 57 on inpatient day 7 to 17.5 on inpatient day 20. Patient discharged on the 20th day of hospitalization with no emerging blisters and the ability to swallow minced food by mouth.
 Conclusion: Calcitriol supplementation of 0,25 mcg per day for 10 days helps to improve the ABSIS score in bullous pemphigoid patient with acute kidney injury. Administration of calcitriol is considered safe and well tolerated.

Nail changes in pemphigus and bullous pemphigoid: A single-center study in China

Common autoimmune bullous diseases (AIBDs) include pemphigus and bullous pemphigoid (BP), which are primarily caused by IgG autoantibodies against the structural proteins of desmosomes at the cell–cell junction and hemidesmosomes at the epidermal–dermal junction. Few studies have assessed nail changes in patients with pemphigus or BP. In the present study, we collected the clinical data of 191 patients with AIBDs (108 patients with pemphigus and 83 patients with BP) and 200 control subjects. Nail changes were observed in 77.0% (147/191), 77.8% (84/108), and 75.9% (63/83) of patients with AIBDs, pemphigus, and BP, respectively, and 14.5% (29/200) of control subjects. Beau's lines and paronychia were the most common nail involvement, observed in 22.5% (43/191) and 22.5% (43/191) of patients with AIBDs, 25.0% (27/108) and 25.9% (28/108) of patients with pemphigus, 19.3% (16/83) and 18.1% (15/83) of patients with BP, respectively. The autoimmune bullous skin disorder intensity score (ABSIS) and the onset time of patients with pemphigus or BP with nail changes were different. Onychomycosis accounted for 21.5% (41/191) of all patients with AIBDs. The ABSIS was correlated with nail involvement in patients with BP (r = 0.46, p < 0.001), and weakly correlated with nail involvement in patients with AIBDs (r = 0.37, p < 0.001), pemphigus (r = 0.29, p = 0.009), and pemphigus vulgaris (PV; r = 0.35, p = 0.008). No correlation was observed between nail involvement and disease antibody titers. In conclusion, nail changes are frequently observed in patients with pemphigus and BP. The type and onset time of nail changes may indicate the severity of pemphigus and BP, which warrants the attention of dermatologists.

ODSS vs. ABSIS and PDAI oral parts in pemphigus vulgaris: inter-rater reliability and testing times.

The existence of standard methods for diagnosis and measuring the severity of diseases leads to a more accurate severity assessment, the possibility of following up, and the possibility of comparing the results of studies. This study aimed to compare different pemphigus vulgaris (PV) assessment methods regarding inter-observer reliability and testing times-focusing on oral parts. Two dermatologists evaluated orally involved PV patients by oral parts of Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), Pemphigus Disease Area Index (PDAI), and Oral Disease Severity Score (ODSS). Seventy patients completed the study. The intraclass correlation coefficient showed the evaluators' agreements on ABSIS, PDAI, and ODSS with 0.98, 0.94, and 0.95, respectively. Reliability analyses showed near-perfect relationships between each scoring methods pairs. There was no association between lesion sites and disease severity. The PDAI scoring duration was significantly shorter, and the ABSIS scoring duration was significantly longer. ODSS is valid for evaluating oral involvement in patients with PV and relates to ABSIS and PDAI almost perfectly. Besides, it was shown that the evaluation of patients' oral involvement based on PDAI and ODSS is done in about 1 min, which seems clinically reasonable.

Elevated Serum Levels of Interleukin-15 in Pemphigus Vulgaris Patients: a Potential Therapeutic Target.

IntroductionPemphigus vulgaris (PV) is a rare autoimmune disease that causes painful blistering. Interleukin-15 (IL-15) as a member of the immunoregulatory cytokines family is associated with the development of the chronic inflammatory or autoimmune disease. There is not much information available in the literature on the exact role IL-15 plays in PV.ObjectivesThe goal of this study was to evaluate the serum levels of IL-15 in patients with PV and assess the association of IL-15 with anti-desmoglein antibodies and the severity of the disease.MethodsFifty-three individuals affected with active PV and 38 age- and gender-matched healthy controls were participated in this study. Disease severity was assessed using Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Serum levels of IL-15 (pg/mL) and anti-desmoglein antibodies (Dsg1, 3) were determined.ResultsIn the patient group, IL-15 serum levels were statistically higher than those in the control group (3.71 ± 1.5 vs. 0.79 ± 1.03, P < 0.001). A positive correlation was found between serum levels of IL-15 and ABSIS (r = 0.5, P = 0.04). We found no significant correlation between serum concentrations of IL-15 and antidesmoglein antibodies (Dsg1 or Dsg3).ConclusionsAn increase in serum level of IL-15 in patients with PV and its relationship with disease severity suggest that this cytokine possibly contributes to the pathogenesis of the disease and targeting IL-15 will likely provide a new insight into the treatment of this disease.

European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part I.

This guideline on mucous membrane pemphigoid (MMP) has been elaborated by the Task Force for Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology (EADV) with a contribution of physicians from all relevant disciplines and patient organizations. It is a S3 consensus‐based guideline encompassing a systematic review of the literature until June 2019 in the MEDLINE and EMBASE databases. This first part covers methodology, the clinical definition of MMP, epidemiology, MMP subtypes, immunopathological characteristics, disease assessment and outcome scores. MMP describes a group of autoimmune skin and mucous membrane blistering diseases, characterized by a chronic course and by predominant involvement of the mucous membranes, such as the oral, ocular, nasal, nasopharyngeal, anogenital, laryngeal and oesophageal mucosa. MMP patients may present with mono‐ or multisite involvement. Patients’ autoantibodies have been shown to be predominantly directed against BP180 (also called BPAG2, type XVII collagen), BP230, laminin 332 and type VII collagen, components of junctional adhesion complexes promoting epithelial stromal attachment in stratified epithelia. Various disease assessment scores are available, including the Mucous Membrane Pemphigoid Disease Area Index (MMPDAI), the Autoimmune Bullous Skin disorder Intensity Score (ABSIS), the ‘Cicatrising Conjunctivitis Assessment Tool’ and the Oral Disease Severity Score (ODSS). Patient‐reported outcome measurements (PROMs), including DLQI, ABQOL and TABQOL, can be used for assessment of quality of life to evaluate the effectiveness of therapeutic interventions and monitor disease course.

Beyond the skin: disease parameters in pemphigus.

Pemphigus represents a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and mortality. Because of the painful chronic-recurring blisters and/or erosions on skin and mucosa, pemphigus can impair quality of life (QOL). Therapeutic modalities, anxiety and depression can also have an additional negative impact in the QOL of the pemphigus patients. Since the nature and course of the pathology and the fact that pemphigus worsens the quality of life of affected patients, scoring systems to objectively evaluate the clinical activity of the disease and to correlate that with the QOL are needed. Nowadays the most used global scales to assess the clinical activity of pemphigus are the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), the Pemphigus Disease Area Index (PDAI) and the Pemphigus Visual Activity Scale (PVAS). To evaluate the patient's generic QOL the most used score is the Dermatology Life Quality Index (DLQI), but all the sponsered clinical trials in pemphigus are using ABQOL this rather than DLQI.

A Cross-Sectional Study to Correlate Disease Severity in Bullous Pemphigoid Patients with Serum Levels of Autoantibodies Against BP180 and BP230.

Context:Enzyme-linked immunosorbent assay (ELISA) for BP 180 and 230 antibodies is commonly done in patients with bullous pemphigoid. We could not find much data regarding the usefulness of this test to predict the disease severity in Indian population.Aims:We studied the correlation of IgG anti BP180 and anti BP230 antibody titer with disease severity and clinical features in bullous pemphigoid.Settings and Design:This cross-sectional study was conducted at a tertiary care center in western India.Materials and Methods:Forty-two clinically diagnosed treatment-naive cases of bullous pemphigoid were enrolled and investigated with skin punch biopsy, IgG anti BP180, and anti BP230 ELISA, direct immunofluorescence, and indirect immunofluorescence tests. Disease severity was assessed by calculating modified Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) score. Thirty patients with a final diagnosis of bullous pemphigoid were included in the statistical analysis. Pearson's correlation coefficient (r) was used to study correlation.Results:The mean ABSIS skin score was 32.81 when both tests were negative, 42.13 when only BP230 was positive, 76.28 when only BP180 was positive, and 78.16 when both were positive. Pearson's correlation coefficient (r) for BP180 and ABSIS skin score was 0.6 (P value: 0.0005), and for BP230 was -0.055 (P value: 0.600).Conclusions:BP antibody titers correlate partially with disease severity. Anti-BP180 antibody is associated with more severe disease. Anti-BP230 antibody titer does not correlate with disease severity.

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.

Pemphigus Oral Lesions Intensity Score (POLIS): A Novel Scoring System for Assessment of Severity of Oral Lesions in Pemphigus Vulgaris

Background: Many patients with pemphigus vulgaris (PV) in India present with predominant/exclusive oral mucosal lesions. Current validated scoring systems for pemphigus do not adequately represent the clinical variability of oral lesions.Objective: To develop and validate a novel scoring system exclusively for oral lesions in PV.Methods: In this cross-sectional study, the Delphi method was used to build an initial scale that was administered in 115 patients with PV. Exploratory factor analysis was used to examine the underlying factor structure of the new scale. The psychometric properties of the new scale were studied. Correlations between the new scale and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), Pemphigus Disease Area Index (PDAI), and Physician Global Assessment (PGA) were also assessed.Results: Content validity of the initial scale was established with an average content validity index (CVI) of 0.8. Exploratory factor analysis resulted in a 3-factor structure with a total of 9 items. Corrected item-total correlation, a measure of data quality, was more than 0.30 for all items in the new oral mucosal scale—Pemphigus Oral Lesions Intensity Score (POLIS). Significant correlations were observed between POLIS and oral ABSIS (r = 0.85, p < 0.001), mucosal PDAI (r = 0.70, p < 0.001), and PGA (r = 0.60, p < 0.001). POLIS was also reliable with good internal consistency (Cronbach's α = 0.86) and strong inter-rater agreement.Limitations: The study cohort included participants from a single center. Usability and time taken to administer the scale were not assessed.Conclusions: The new scale, POLIS, has adequate validity and reliability. It includes both quality of life and clinical disease severity parameters, assessing disease severity holistically. Further studies evaluating the scale's responsiveness to change are in progress.

Oral health related qualıty of lıfe and dısease severıty ın autoımmune bullous dıseases.

There is an increased risk of long-term dental and periodontal disease in autoimmune bullous diseases (AIBD). In this cross-sectional study, we aimed to determine whether the oral health-related quality of life status (OHRQoL) was associated with disease severity and activity in patients with AIBD. 67 patients with AIBD were enrolled in this study. Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) was used to evaluate the disease severity. The score was categorized as a significant course (≥17) and moderate course (<17). Oral health impact profile-14 (OHIP-14) questionnaire was filled to assess the OHRQoL. Self-reported oral health status and oral lesion related pain score were also evaluated in the study group. OHIP-14 score was significantly higher in active patients (42.28 ± 13.66) than inactive patients (29.08 ± 12.25) (P = 0.004) and it was correlated with the pain score (6.33 ± 2.78; r = 0.409, P = 0.013). Furthermore, OHIP-14 score was higher in patients with a significant disease course (45.18 ± 15.08) (P = 0.010) than in patients with a moderate course (36.09 ± 9.73). OHRQoL may be useful in the disease management and treatment. Since it can be affected by both presence of oral erosions and disease severity, a collaboration between dermatologists and dentists could be crucial to the disease management in AIBD.

Validation of an Oral Disease Severity Score (ODSS) tool for use in oral mucous membrane pemphigoid.

Mucous membrane pemphigoid (MMP) is a rare autoimmune bullous disease predominantly affecting the oral mucosa. Optimal management relies upon thorough clinical assessment and documentation at each visit. The primary aim of this study was to validate the Oral Disease Severity Score (ODSS) for the assessment of oral involvement in MMP. We also compared its inter- and intraobserver reliability with those of the oral parts of the Mucous Membrane Pemphigoid Disease Area Index (MMPDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Physician's Global Assessment (PGA). Fifteen patients with mild-to-moderately severe oral MMP were scored for disease severity by 10 oral medicine clinicians from four U.K. centres using the ODSS, the oral sections of MMPDAI and ABSIS, and PGA. Two clinicians rescored all patients after 2 h. In terms of reliability, the interobserver ODSS total score intraclass correlation coefficient (ICC) was 0·97, MMPDAI activity 0·59 and damage 0·15, ABSIS total 0·84, and PGA 0·72. The intraobserver ICCs (two observers) for ODSS total were 0·97 and 0·93; for MMPDAI activity 0·93 and 0·70 and damage 0·93 and 0·79; for ABSIS total 0·99 and 0·94; and for PGA 0·92 and 0·94. Convergent validity between ODSS and MMPDAI was good (correlation coefficient 0·88). The mean ± SD time for completion of ODSS was 93 ± 31 s, with MMPDAI 102 ± 24 s and ABSIS involvement 71 ± 18 s. The PGA took < 5 s. This study has validated the ODSS for the assessment of oral MMP. It has shown superior interobserver agreement over MMPDAI, ABSIS and PGA, and superior intraobserver reliability to MMPDAI. It is quick and easy to perform. What's already known about this topic? There are no validated scoring methodologies for oral mucous membrane pemphigoid (MMP). Proposed disease activity scoring tools for MMP include the Mucous Membrane Disease Area Index (MMPDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). The Oral Disease Severity Score (ODSS) has been validated for use in oral pemphigus vulgaris (PV). It has been shown to be reliable and sensitive in both lichen planus (LP) and MMP. What does this study add? The ODSS has been shown to be a thorough, sensitive and reproducible, yet quick scoring tool for the assessment of oral involvement in MMP. Its versatility for use in oral PV, MMP and LP is an added advantage over other scoring methodologies. What are the clinical implications of this work? We propose that the ODSS be used as a clinical scoring tool for monitoring activity in oral MMP in clinical practice as well as for use in multicentre studies.

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.

Therapeutic Targeting of Th17/Tc17 Cells Leads to Clinical Improvement of Lichen Planus.

Lichen planus (LP) is a common, chronic relapsing inflammatory disorder of the skin and mucous membranes which often poses a major therapeutic challenge due to its refractory course. Novel pathogenesis-based therapies are urgently needed. As several studies have shown that IL-17 may contribute to LP pathogenesis, we investigated whether therapeutic targeting of IL-17+ T cells leads to clinical improvement of mucosal and cutaneous LP lesions. A total of five patients with lichen planus were treated in a compassionate use trial with either secukinumab (anti-IL-17; 3 patients with acute and chronic recalcitrant muco-cutaneous LP), ustekinumab (anti-IL-12/IL-23; 1 patient with recalcitrant oral LP) or guselkumab (anti-IL-23; 1 patient with recalcitrant oral LP). The clinical course of the patients was assessed by the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) reflecting both extent and severity of disease and functional sequelae of oral involvement for at least 12 weeks. The inflammatory infiltrate in lesional and post-lesional skin was analyzed by immunohistochemistry before and after treatment. Furthermore, the cytokine profile of peripheral blood T cells from the treated patients was assessed by flow cytometry and/or ELISpot assay. Treatment with secukinumab induced rapid and prolonged clinical amelioration of muco-cutaneous LP. Clinical improvement was accompanied by a strong reduction of the Th1 and Th17/Tc17 cellular mucosal and cutaneous infiltrate. Moreover, long-term treatment of one patient with recalcitrant oral LP with ustekinumab led to healing of the ulcerative oral lesions and a reduction of peripheral blood and lesional IL-17+ T cells. Finally, treatment with guselkumab led to a marked clinical improvement in a patient with recalcitrant erosive oral LP. These findings show for the first time that therapeutic targeting of Th17/Tc17 cells leads to a pronounced clinical amelioration of mucosal and cutaneous LP and strongly suggests that IL-17-producing T cells are central to disease pathogenesis. Thus, therapeutic targeting of Th17/Tc17 cells opens new therapeutic avenues in the treatment of recalcitrant LP.

Patient-assigned health utility values for controlled and uncontrolled pemphigus vulgaris and foliaceus.

The assessment of health-related quality of life (HRQoL) in patients with pemphigus is now of increasing interest due to the availability of highly effective new therapies. Preference-based HRQoL values or health utilities required for medical and financial decision-making are not yet available directly from pemphigus patients. To obtain health utility values for current health and hypothetical health states from the perspective of pemphigus patients. A cross-sectional questionnaire survey was carried out with pemphigus patients. Disease severity was rated by Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Patients were asked to evaluate their current health as well as three common hypothetical pemphigus health states [uncontrolled pemphigus vulgaris (PV), uncontrolled pemphigus foliaceus (PF) and controlled PV/PF] by using composite time trade-off (cTTO). Multiple regression was applied to explore determinants of utility values. Responses of 108 patients (64.8% women, mean age 57.4years) were analysed. Mean ABSIS score was 11.6. The mean utility values for the hypothetical uncontrolled PV, uncontrolled PF and controlled PV/PF health states were 0.41, 0.52 and 0.66 with cTTO. The mean cTTO scores for current health were higher compared with the hypothetical health states (0.76; P<0.001). Patients with higher ABSIS, worse pain intensity scores and those having a caregiver reported lower utility values for current health (P<0.05). In pemphigus, HRQoL impairment expressed in utility values seems to be considerable, especially in comparison with other chronic dermatological conditions (e.g. psoriasis, atopic eczema, chronic hand eczema). These health utilities inform physicians, policymakers and funders about the overall extent of health loss in pemphigus and provide evidence to guide medical decisions and cost-effectiveness analyses of treatment strategies. Future research is needed to evaluate the caregiver burden in pemphigus.

The elevated level of osteopontin in patients with pemphigus vulgaris: A cytokine-like protein with a therapeutic potential.

Pemphigus vulgaris (PV) is a life-threatening autoimmune disease with no certain treatment. Anticytokine therapy is being increasingly discussed in multiple autoimmune diseases. Osteopontin (OPN) is a glycoprotein produced by a variety of immune cells. Increased OPN serum levels have been reported in several autoimmune diseases, with targeting OPN considered as a promising therapy in these diseases. However, the role of OPN in PV has not been well studied so far. To investigate whether OPN level is elevated in PV patients in the active stage of the disease and to examine its possible relationship with disease severity and anti-desmoglein (anti-Dsg) antibodies levels. This study included 53 consecutive subjects affected by PV and 38 age- and sex-matched healthy controls. Clinical characteristics and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) were assessed. Serum OPN levels (pg/mL) and anti-Dsg antibodies were also measured. The serum OPN level of the patient group proved to be statistically higher than that of the control group (11.08 ± 5.24 vs 8.47 ± 5.68; p = .02). No significant relationship were detected between the serum OPN level and anti-Dsg1 or anti-Dsg3 antibodies (r = 0.1, p = .2 and r = 0.1, p = .4), respectively. In addition, no correlation was found between serum OPN levels and severity of PV as measured by ABSIS (r = 0.08 and p = .5). The growth observed in OPN levels in pemphigus patients suggests the role of OPN in pemphigus pathogenesis, but there is a need for more extensive studies to show how OPN can be associated with the PV pathogenesis and whether OPN could be used as an important therapeutic target in pemphigus disease.

Subjective well-being in patients with pemphigus: a path analysis

BackgroundPemphigus is a chronic autoimmune blistering disease of the skin and mucosa severely impairing patients’ health-related quality of life (HRQoL). To date, no studies have measured subjective well-being in terms of life satisfaction in pemphigus. Our main objective was to evaluate satisfaction with life in patients with pemphigus, and to analyse its relationship with clinical severity and HRQoL.MethodsA cross-sectional survey was carried out enrolling 77 patients with pemphigus. Subjective well-being was measured using the Satisfaction with Life Scale (SWLS). HRQoL was assessed by the Dermatology Life Quality Index (DLQI) and EQ-5D-5L. Disease severity was measured by Autoimmune Bullous Skin Disorder Intensity Score (ABSIS).ResultsMean ABSIS, DLQI, EQ-5D-5L and SWLS scores of patients were 11.7 (SD 17.3), 5.4 (6.8), 0.84 (0.22) and 4.76 (SD 1.52), respectively. The proportion of patients indicating extreme dissatisfaction, dissatisfaction, slightly below average in life satisfaction, average satisfaction, high satisfaction and very high satisfaction with life was 6 (7.8%), 5 (6.5%), 14 (18.2%), 16 (20.8%), 21 (27.3%) and 15 (19.5%), respectively. Life satisfaction was independent from age, gender, level of education and type of disease. A path analysis revealed that there was no direct relationship between ABSIS and SWLS (beta = − 0.09; p = 0.428); however, the following indirect path was confirmed: ABSIS → DLQI → EQ-5D-5L → SWLS.ConclusionsDisease severity and HRQoL measures regularly used to assess patients’ health status may be complemented with a measure of subjective well-being, such as SWLS, to achieve a more holistic assessment of patients’ lives and optimise pemphigus care.

Evaluation of the relation between T-Lymphocytes (CD4 + ve and CD8 + ve) and pemphigus vulgaris pathogenesis and severity

Background: Pemphigus vulgaris (PV) is an autoimmune bullous disease involving both the skin and mucus membranes. Infiltrating CD4+ and CD8+ T-lymphocytes may have a vital role in its pathogenesis. The autoimmune bullous skin disorder intensity score (ABSIS) was proposed for clinical evaluation and scoring of PV patients. Aims and Objectives: This work aimed to assess the role of CD4+ and CD8+ T-lymphocytes in the pathogenesis of PV and the correlation between number of T-lymphocytes infiltrate and clinical severity of the disease evaluated by ABSIS. Patients and Methods: Forty PV patients (patient group) and 10 healthy volunteers (control group) were enrolled. All patients were scored by ABSIS. Immunohistochemical staining was performed on skin biopsies for IgG, IgA, CD8, and CD4. Results: IgG was positive in 97.5% of patients, combined IgG and IgA were positive in 20% of patients, and only one case was negative for both antibodies. Thirty-three cases showed the predominance of CD4+ T cells, and seven cases showed predominance of CD8+ T cells. CD4/CD8 ratio was 2.578. Non-significant positive correlation was found between the total count of lymphocytic infiltrate and ABSIS. Conclusion: CD4+ and CD8+ T-lymphocytes share in the pathogenesis of PV and CD4+ are more expressed than CD8+ in the inflammatory infiltrate of PV lesions. The correlation between total lymphocytic count and ABSIS score may help to predict the clinical state of the patient from skin biopsy, and ABSIS score can predict the lymphocytic infiltration in PV cases. This correlation is in need to be reapplied on larger scales of patients to confirm its validity and reliability.

Validity of the EQ-5D in patients with pemphigus vulgaris and pemphigus foliaceus.

No studies to date have employed the EuroQoL EQ-5D questionnaire to assess health-related quality of life (HRQoL) in patients with pemphigus. To evaluate the HRQoL of patients with pemphigus by the EQ-5D and to analyse the convergent and known-groups validity of the EQ-5D in this patient population. Between 2014 and 2017, a multicentre cross-sectional study was carried out. Outcome measures included the five-level EQ-5D (EQ-5D-5L), Dermatology Life Quality Index (DLQI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and an average pain intensity visual analogue scale (VAS) for the past 3 months. In total, 109 consecutive patients with pemphigus participated in the study (mean age 57 years; 64% women). Among the EQ-5D dimensions, the most problems were reported regarding pain/discomfort (50%), mobility (43%) and anxiety/depression (43%). No significant difference was found in mean EQ-5D index scores between patients with pemphigus vulgaris and those with pemphigus foliaceus (0·81 vs. 0·86, P = 0·14). The mean EQ-5D index scores of patients with limited, moderate, significant and extreme pemphigus were 0·88, 0·82, 0·72 and 0·67, respectively (P = 0·001). The number of comorbidities was associated with greater impairment in EQ-5D index scores (P < 0·001). DLQI (rs = -0·62, P < 0·001) and the average pain intensity VAS (rs = -0·59, P < 0·001) more strongly correlated with the EQ-5D index scores than did ABSIS (rs = -0·40, P < 0·001). This is the first study employing the EQ-5D questionnaire in pemphigus. The EQ-5D is a valid measure of HRQoL in patients with pemphigus that can be useful both in clinical practice and in economic evaluations to assess the health gains associated with new effective treatments.

Usefulness of miRNA-338-3p in the diagnosis of pemphigus and its correlation with disease severity.

BackgroundPemphigus is a common life-threatening, autoimmune bullous disease effecting both cutaneous and mucous membranes. Previous diagnosis of pemphigus is based on clinical presentations, histopathology, immunofluorescence and enzyme-linked immunosorbent assay. Furthermore, no laboratory parameters could be used to indicate disease severity. MicroRNAs are endogenous small RNAs, which could be used as diagnostic biomarkers for some autoimmune diseases. Previously, miR-338-3p has been proven significantly up-regulated in pemphigus patients.MethodsPemphigus patients (including pemphigus vulgaris and pemphigus foliaceus) with active lesions and with remission, patients diagnosed as bullous pemphigoid and healthy volunteers were recruited, and miR-338-3p expression level was measured using reverse transcriptase-quantitative polymerase chain reaction (RT-qPCR). Active pemphigus patients accepting treatment were followed up for at least 2 weeks to investigate the expression change of miR-338-3p during treatment period. Target genes of miR-338-3p were screened through computer-aided algorithm and verified by RT-qPCR, Western blot and Luciferase activity assay.ResultsMiR-338-3p was specifically increased in patients diagnosed as pemphigus with active lesions. The expression level of miR-338-3p gradually decreased after effective treatment. MiR-338-3p expression was independently correlated with disease severity defined by PDAI (Pemphigus Disease Area Index) or ABSIS (Autoimmune Bullous Skin Disorder Intensity Score) criteria. Up-regulation of miR-338-3p could significantly suppress RNF114 expression at mRNA and protein level in vitro.DiscussionMiR-338-3p could be used as a diagnostic biomarker of pemphigus in addition to other traditional methods. Up-regulation of MiR-338-3p was associated with more severe condition in pemphigus. RNF114 is the target gene of miR-338-3p, which probably participates in the regulation of disease activity of pemphigus.

Adjuvant high-dose intravenous immunoglobulins for recalcitrant erosive oral lichen planus: mixed clinical responses.

Erosive oral lichen planus (OLP) is, at times, extremely difficult to treat and has a major impact on patients' quality of life. There are only limited therapeutic options, such as topical and systemic glucocorticoids, retinoids, and immunosuppressants with considerable side effects and limited efficacy upon chronic use. In the present individualised clinical trial, we assessed the efficacy of adjuvant intravenous immunoglobulins (IVIG; 2g/kg/monthly cycle) in addition to the oral retinoid, acitretin, in three patients with refractory OLP over a period of two to six months. The efficacy of adjuvant IVIG treatment was evaluated using the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) which measures both extent of mucosal lesions and functional sequelae. The three OLP patients showed mixed responses to adjuvant IVIG treatment, ranging from therapeutic efficacy to a lack of response to IVIG. In light of the observed therapeutic responses and a lack of good therapeutic options, adjuvant IVIG, although costly, warrants further investigation as a treatment option for OLP.