Bullous Skin Disorder Intensity Score Research Articles

Scoring Criteria for Autoimmune Bullous Diseases: Utility, Merits, and Demerits

Abstract Background: Scoring systems play a crucial role in dermatology by providing objective measurements of disease severity, treatment efficacy, and outcome comparisons. In autoimmune blistering diseases (AIBDs), standardized scoring systems are essential for accurate evaluations; however, there is currently a lack of consensus on scoring methods. Objective: This literature review explores scoring systems in AIBDs by tracing their development, addressing challenges, and highlighting their role in defining endpoints, regulatory considerations, and clinical trials. Materials and Methods: Existing scoring systems for AIBDs, such as the Pemphigus Disease Area Index, Autoimmune Bullous Skin Disorder Intensity Score, Pemphigus Oral Lesions Intensity Score, Oral Disease Severity Score, and Pemphigus Vulgaris Activity Score, are examined for their validity, reliability, and responsiveness. The Bullous Pemphigoid Disease Area Index for bullous pemphigoid is also discussed. The concept of minimal clinically important differences is explored to determine clinically significant improvements in disease severity. Conclusion: This review provides a comprehensive understanding of the central role of scoring systems in dermatology and their implications for research and clinical practice in AIBDs.

Pemphigus oral lesions area index (POLAI): A new scoring scale for assessing oral pemphigus vulgaris

AbstractObjectivesCurrent scales for Pemphigus vulgaris (PV) do not adequately represent the clinical variability of oral lesions. This study aimed to develop an independent scale, the Pemphigus Oral Lesions Area Index (POLAI), for assessment of oral PV exclusively, and compare POLAI, Pemphigus Disease Area Index (PDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Oral Disease Severity Score (ODSS) regarding inter‐ and intra‐observer reliability and validity.Materials and MethodsRetrospective cohort included 209 sets of digital‐photographs. Additional clinical cohort included 32 PV patients. All visits were assessed by four clinicians using the PDAI, ABSIS, ODSS and POLAI, and were rated by three specialists using the Physician's Global Assessment (PGA).ResultsThe intraclass correlation coefficient showed the inter‐observer reliability with 0.89 and 0.86 for PDAI, 0.87 for ABSIS, 0.93 for ODSS, 0.96 for POLAI, and 0.97 and 0.96 for PGA. Intra‐observer agreements showed excellent reliability for all 4 scores. Highest correlation was observed between PGA and POLAI (correlation coefficients were 0.96). The mean time taken to complete each scale was within 1.5 min.ConclusionPOLAI is valid for the assessment of oral PV with superior inter‐ and intra‐observer reliability to PDAI, ABSIS and ODSS, and is feasible in clinic.

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.

003 Utility of plucked hair direct immunofluorescence as a possible replacement for conventional skin biopsy direct immunofluorescence in immunological activity monitoring and prediction of relapse in pemphigus

Abstract Pemphigus vulgaris (PV) is a potentially lethal chronically relapsing autoimmune bullous disorder with intraepithelial lesions affecting the skin and mucous membranes. The prediction of clinical relapse has been studied with conventional skin biopsy and plucked hair direct immunofluorescence (DIF; Daneshpazhooh M, Naraghi ZS, Ramezani A et al. Direct immunofluorescence of plucked hair for evaluation of immunologic remission in PV. J Am Acad Dermatol 2011; 65:173–7) which can be of use in timing treatment withdrawal and helping with better management. The aims of our study were to determine the association of plucked hair root DIF positivity status at baseline with clinical relapse within the study duration, and to determine the correlation between skin biopsy and plucked hair DIF. Seventy-eight consecutive patients diagnosed with PV in clinical remission on therapy, on minimal therapy or off therapy, were included after obtaining informed consent. Three-monthly follow-up was carried out for 12 months. Immunological monitoring with plucked hair DIF was done at baseline and repeated every 3 months for 1 year or a relapse was experienced, whichever occurred earlier. Conventional skin biopsy DIF was performed at baseline, at 3 months and at the time of relapse. In patients receiving adjuvant therapy during their present remission episode, discontinuation of therapy was done at inclusion, at 3 months and at 6 months, and occurrence of relapse in relation to the time point of discontinuation was assessed. Of 78 patients, 22 had disease relapse and 56 remained in complete remission. Clinical relapse during the study duration was significantly more frequent in patients who had higher mean Autoimmune Bullous Skin Disorder Intensity Score during the preceding active disease episode (P = 0.033), pruritus during the present clinical remission episode (P = 0.007) and early discontinuation (within 3 months of study inclusion) of concomitant adjuvant therapy (P = 0.007). Immunological parameters associated with clinical relapse were baseline IgG (P = 0.005) and C3 positivity (P = 0.047) in plucked hair, greater intensity of IgG and C3 positivity at baseline in plucked hair (IgG, P = 0.002; C3, P = 0.033) and skin biopsy specimens (IgG, P = 0.004; C3, P = 0.0.002), and positive hair DIF at the time of adjuvant therapy withdrawal (P = 0.017). Plucked hair DIF is a reliable technique to assess immunological remission in patients with PV who are in clinical remission and helps to guide treatment according to immunological remission status.

Lymphocyte-predominant lesional inflammatory infiltrates of the skin are associated with mucosal-dominant phenotype in pemphigus.

Pemphigus is a potentially life-threatening autoimmune blistering disease. To date, studies assessing the association of histopathology with clinical phenotype are lacking. We sought to evaluate the main histopathologic findings and, also, the potential links between cutaneous inflammatory infiltrates and clinical characteristics in pemphigus. We conducted a retrospective cohort study in patients diagnosed with pemphigus vulgaris (PV) and pemphigus foliaceus (PF) in a referral center for autoimmune blistering diseases. A total of 124 patients were included in the study (97 had PV and 27 had PF). On biopsy specimens, PV was more frequently associated with the "row of tombstones" feature (36.1% vs. 11.1%, p=0.013), and PF was associated with acanthosis (44.4% vs. 23.7%, p=0.034). Acantholysis was found in the upper half of the epidermis in PF (96.3% vs. 5.15%, p < 0.001), as opposed to the lower half in PV (75.2% vs. 0%, p=0.002). Patients with lymphocyte-predominant inflammatory infiltrates in lesional skin specimens presented with a higher frequency of the mucosal-dominant phenotype (25.5% vs. 9.1%, p=0.014), higher-density cellular infiltrate (100% vs. 41.6%, p < 0.001), and more frequent acantholytic cells (42.6% vs. 23.4%, p=0.025). Neutrophil-predominant infiltrates in specimens from lesional skin were linked to a milder disease based on median Pemphigus Disease Area Index (38.9% vs. 13.2%, p=0.036) and Autoimmune Bullous Skin Disorder Intensity Score (20.2 vs. 36.3, p=0.019), while eosinophil-predominant inflammatory infiltrates were more often associated with eosinophilic spongiosis (100% vs. 23.1%, p=0.014). Lymphocyte-predominant infiltrates in lesional skin specimens of pemphigus patients predict a mucosal-dominant phenotype, while neutrophil-predominant infiltrates are associated with a milder disease.

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.

Downregulation of aquaporin 3 in patients with pemphigus vulgaris.

Pemphigus vulgaris (PV) is an autoimmune bullous skin disease. Aquaporin 3 (AQP3) is a glycerol/water channel involved in several physiological functions. Evaluation of the tissue expression and localization of AQP3 in the skin of PV patients. Twenty-seven PV patients and 30 controls were included. The patients were subjected to history taking, clinical evaluation, Autoimmune Bullous Skin Disorder Intensity Score and 4-mm punch biopsy. The biopsies were stained using anti-human AQP3 antibody and the immunofluorescence pattern and intensity were evaluated using a scoring system and ImageJ software analysis. AQP3 was expressed in the basal epidermis in 27 (100%) and in the suprabasal epidermis in 19 PV patients (70.4%). It was expressed in all controls in basal and suprabasal layers. Intensity of AQP3 immunofluorescence was strong in 2 (7.4%), moderate in 19 (70.4%) and weak in 6 patients (22.2%) while it was strong in 18 (60%) and moderate in 12 controls (40%). AQP3 expression was significantly lower in patients than controls in the suprabasal epidermis (p= 0.001). Patients with extensive disease had significantly weaker AQP3 intensity than those with marked disease (p= 0.005) Downregulation of AQP3 in patients with PV, especially in the suprabasal layers and in extensive clinical disease, suggests a potential role of AQP3 in the pathogenesis of PV.

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.

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.

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.

Family impact of pemphigus disease in an Iranian population using the Family Dermatology Life Quality Index.

BackgroundPemphigus vulgaris (PV) is a rare but seriously disabling disorder of the skin and mucous membranes that can gravely impact the quality of life (QoL) of patients.ObjectiveThe aim of the present study was to identify how family members of patients with PV are affected by the disease.MethodsA total of 118 patients with confirmed PV and one of their family members (caregivers) were enrolled in the study. To calculate disease severity, the Autoimmune Bullous Skin Disorder Intensity Score was used. The Persian version of the Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate the QoL of patients and the Family Dermatology Life Quality Index (FDLQI) to evaluate the QoL of caregivers.ResultsThe mean age of patients was 43.14 ± 12.5 years. Ninety patients (76.3%) were female. Eighty-one patients (68.6%) had the mucocutaneous phenotype and 37 cases (31.4%) the mucosal phenotype. The DLQI score was 10.1 ± 7.1 for patients. The DLQI score was higher for patients with the mucocutaneous phenotype (11.8 ± 7.5) than those with the mucosal phenotype (6.4 ± 4.9; p < .001). QoL was significantly affected by disease severity. FDLQI score was 13 ± 7 for caregivers, and was significantly higher in older caregivers and married ones. There was a positive correlation between patients’ admission frequencies and FDLQI score. FDLQI score was also significantly affected by the Autoimmune Bullous Skin Disorder Intensity Score of patients’ disease severity. The QoL of patients and their caregivers showed a significant positive correlation.ConclusionThe QoL of patients and their families are impaired significantly, and is considerably prominent in the mucocutaneous phenotype of PV and more severe forms.LimitationPemphigus Disease Area Index (PDAI) and Autoimmune Bullous Disease QoL (ABQoL) were not used in this study.

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.

Clinical Relevance of Polymorphonuclear Myeloid-Derived Suppressor Cells in Autoimmune-Blistering Disorders Pemphigus Vulgaris and Bullous Pemphigoid

Clinical Relevance of Polymorphonuclear Myeloid-Derived Suppressor Cells in Autoimmune-Blistering Disorders Pemphigus Vulgaris and Bullous Pemphigoid

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.

Experiencia con rituximab en el tratamiento de las enfermedades ampollares autoinmunes

BackgroundPemphigus vulgaris and foliaceus are bullous autoimmune diseases caused by antibodies directed against desmoglein 1 and/or 3. The standard treatment is high-dose oral glucocorticoids and other immunosuppressants such as azathioprine or mycophenolate. Rituximab (RTX), an anti-CD20 monoclonal antibody, has been shown effective in the treatment of these entities. ObjectiveTo assess the degree of response to RTX (partial response/complete response) and the time to reach it; the time to relapse, the evolution of anti-desmoglein antibodies 1/3 and the possible adverse effects. Patients and methodsThis is a retrospective descriptive and single institution study including all patients diagnosed with pemphigus vulgaris or pemphigus foliaceus treated with RTX and with a minimum post-treatment follow-up of 6 months. ResultsSix patients were included (4 pemphigus vulgaris and 2 pemphigus foliaceus) with a mean follow-up of 84 months. The total number of RTX cycles administered was 15, reaching a complete response in 7 cycles, a partial response in 6 and no response in 1. An improvement in the Autoimmune Bullous Skin Disorder Intensity Score was observed both in the cutaneous involvement (range 0-150; mean pre/pos-RTX 3,19/1) as in the oral extension (range 0-11; mean pre/pos-RTX 9,21/1,33). The mean time to relapse was 20.75 months. Only two patients had mild immediate adverse effects during drug perfusion (headache and high blood pressure). DiscusionRTX is an effective and safe drug in the management of pemphigus. It could achieve long remission periods without therapy or with minimal concomitant one, permitting to avoid AE of chronic treatment with corticosteroids.