Bullous Disorders Research Articles

S3562 A Rare Case of Esophageal Dissecans Superficialis Occurring With Barrett's Esophagus

Introduction: Esophageal dissecans superficialis (EDS) is a rare desquamative disorder of the esophagus characterized by sloughing of the superficial mucosa. Less than 200 cases of EDS are reported worldwide. The pathogenesis is poorly understood. We present a case of EDS in the setting of Barrett’s esophagus (BE). The association of EDS with BE has not been previously reported. Case Description/Methods: The patient is a 66-year-old male with a history of hypertension, chronic obstructive pulmonary disease, and atrial fibrillation who was referred to an outpatient gastroenterology clinic for surveillance esophagogastroduodenoscopy (EGD) for BE. On medication review, he was taking amlodipine, carvedilol, albuterol, and aspirin. He smoked half a pack per day but denied significant alcohol or substance use. He was asymptomatic. On EGD, linear, peeling fragments of the superficial mucosa were found in the distal esophagus consistent with the classic features of EDS (Figure 1). A plaque of salmon-colored mucosa was also identified in the distal esophagus. Biopsies demonstrated BE and active inflammation. Outpatient treatment with a twice-daily oral proton pump inhibitor (PPI) was initiated. Three months later, repeat EGD revealed a short segment of BE and complete resolution of EDS. Discussion: EDS is a rare endoscopic finding. Clinical manifestations vary from an incidental finding to dysphagia, odynophagia, vomiting, heartburn, and abdominal pain. Although its pathogenesis is unclear, it is thought that EDS stems from conditions that cause esophageal damage, including heavy smoking, psychoactive medications, bisphosphonates, non-steroidal anti-inflammatory drugs, and cutaneous bullous disorders. We describe the first encounter of EDS in a patient with BE. In our case, it is speculated that chronic inflammation from gastroesophageal reflux disease and tobacco use caused mucosal injury, leading to the development of not only metaplastic changes but also sloughing esophagitis. This case highlights the importance of clinical recognition of EDS as its prognosis is generally favorable if treated promptly. Mucosal healing can be achieved with discontinuation of the culprit agent and PPI therapy, reducing unnecessary investigation and treatment.Figure 1.: Endoscopy showing multiple white sheets of peeling superficial mucosa with normal basal mucosa in the distal one-third of the esophagus, consistent with EDS.

Increased Fungal Infections while using Emerging Therapies (Biologics and Small-molecule Inhibitors) for Treating Skin Diseases: A Review

Biologics, such as tumor necrosis factor-α and interleukin inhibitors, are commonly used for treating immunological skin diseases, including psoriasis, psoriatic arthritis, and atopic dermatitis. The cluster of differentiation inhibitors and immune checkpoint inhibitors has also been used for treating bullous disorders and melanoma, respectively. Other small-molecule inhibitors, such as JAK inhibitors, have been introduced for treating atopic dermatitis and alopecia areata. Hence, given the importance of cytokines and small molecules in antifungal immunity, using these new treatments are proposed to increase the risk of fungal infections. Thus, this review presents an overview of the reported incidences and possible mechanisms of fungal infections related to the use of biologics, including small-molecule inhibitors used for dermatological treatments.

Role of nutritional supplements in selected dermatological disorders: A review.

While a plethora of literature continues to be published on the role of nutritional agents both in lay press and indexed journals, the data is not on a firm footing and leaves the dermatologist in a quandry and the patient confused. The various agents include vitamins, minerals, amino acids, antioxidants, diets & gluten. A proper knowledge of the role of nutritional supplements in dermatological diseases can be a useful tool in advising the patients and in certain cases ameliorating the disorder. Literature review of last 15 years was made using the terms "diet in dermatology," "nutrition and skin," "nutritional supplements in dermatology," "nutritional agents and acne," "nutritional agents and alopecia," and "nutritional agents and psoriasis." While there are multiple publications on the use of nutritional supplements for amelioration of skin diseases, most of them are based on either associations or in vitro studies, but very few transcend the rigors of a clinical trial or the holey grail of a double-blinded randomized controlled trial. There seem to be some evidence in acne, psoriasis, telogen effluvium, urticaria & vitiligo. Coeliac disease and dermatitis herpetiformis have a strong link with diet. Rosacea has a strong link with certain foods, but the other disorders like melasma, aphthous stomatitis do not have any scientifically validated association with diet. Our updated review examines the role of nutritional supplements and antioxidants in various dermatological disorders. We have found that there are varying levels of evidence with notable associations of low glycemic diet & acne, fish oil & weight loss with psoriasis, fish oils & probiotics with atopic dermatitis & vitamins & botanical extracts with vitiligo. The evidence for diet and nutrition in bullous disorders and photoageing is scarce. The role of low histamine diet in urticaria is useful in select cases of episodic urticaria. Rosacea is triggered by hot and spicy food . Apart from gluten and Dermatitis Herpetiformis, no diet can be considered disease modifying in our reveiw. The lack of comparison of nutritional or dietary modiffication with conventional validated agents, makes the data difficult to translate in real world patient management.

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.

Human Desmocollin 3‒Specific IgG Antibodies Are Pathogenic in a Humanized HLA Class II Transgenic Mouse Model of Pemphigus

Pemphigus is a potentially lethal autoimmune bullous skin disorder, which is associated with IgG autoantibodies against desmoglein (DSG) 3 and DSG1. Notably, a subset of patients with pemphigus presents with a similar clinical phenotype in the absence of anti-DSG IgG, suggesting the presence of serum IgG reactive with desmosomal components other than DSG1 or DSG3. We and others have previously shown that such patients have serum IgG autoantibodies against desmocollin 3 (DSC3), a component of desmosomes, which induce loss of keratinocyte adhesion exvivo. Moreover, DSC3 hypomorphic mice show a severe blistering phenotype of the mucous membrane, which is highly characteristic of pemphigus. These findings prompted us to study the induction and regulation of anti-human DSC3 IgG in humanized mice transgenic for HLA-DRB1∗04:02, which is a highly prevalent haplotype in pemphigus. We show that IgG from sera of immunized mice induces acantholysis in a dispase-based keratinocyte dissociation assay through the activation of p38 MAPKs and EGFR. Passive IgG transfer from mice immunized with recombinant human DSC3 into neonates did not induce intraepidermal loss of adhesion presumably owing to the lack of homology between human and mouse DSC3. Exvivo stimulation of splenocytes from DSC3-immunized mice with human DSC3 leads to a significant proliferative IFN-γ and IL-4 T-cell response, which is restricted by HLA-DR/HLA-DQ. These findings suggest that the induction of pathogenic anti-DSC3 IgG is associated with DSC3-specific T cells that recognize DSC3 in association with HLA-DRB1∗04:02.

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.

BI14: Multiple autoimmune bullous disorders arising secondary to immune checkpoint blockade

BI14: Multiple autoimmune bullous disorders arising secondary to immune checkpoint blockade

Histomorphological spectrum of noninfectious vesiculobullous lesions of skin: A rural area based study

Background: Vesiculobullous lesions are the major contribution of various skin disorders. They are primary morphological representation of various pathological stimuli. Histopathology plays major role in the initial phase of diagnosis helps in further evaluation to identify the underlying etiology. Aim and Objective: To study the prevalence of various morphological types of vesiculobullous skin lesions in different age groups in a rural area. Methodology: A Retrospective study conducted from May 2018 to May 2021, at Katuri Medical College & Hospital, Guntur. A study of vesiculobullous lesions of the skin biopsies of 90 cases was received to the department of pathology, at Katuri Medical College & Hospital, for 3 years. Complete clinical and relevant history was recorded. The specimens were routinely processed and H&E-stained slides were studied. Results: Out of 454 skin biopsies, of which 90 cases (19.8%) were diagnosed as vesiculobullous lesions by histopathologically. Pemphigous vulgaris comprised 38 cases (42.23%) followed by Bullous pemphigoid 24 cases (26.67%), in clinically suspected Autoimmune Bullous Disorders. Conclusion: Clinical examination has greater significance in diagnosis still histopathological examination plays vital role, helps to arrive at a definitive diagnosis in the majority of vesiculobullous disorders. Skin biopsy is inexpensive, carried out as OPD procedure with minimal discomfort to the patient.

Clinical Role of Oral Vitamin C and E Therapy in Skin and Hair Disorders

Clinical Role of Oral Vitamin C and E Therapy in Skin and Hair Disorders

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.

Utility of Direct Immunofluorescence in Cutaneous Autoimmune Bullous Disorders.

BackgroundAutoimmune bullous disorders (AIBD) are a heterogeneous group of disorders with substantial clinical overlap associated with blistering of skin or mucosa.AimsThe present study aimed to study the histopathological spectrum and evaluate the utility of direct immunofluorescence (DIF) on snap-frozen and paraffin-embedded sections in resolving the differential diagnosis of AIBD and connective tissue disorders of the skin. We also compared the efficacy of DIF on paraffin versus the snap-frozen sections in diagnosing AIBD.MethodsThe present study was conducted for three years (2017-2019) and included 27 biopsies. We also included a retrospective analysis that included 25 biopsies collected over three years (2014-2017). Histopathological examination and DIF were conducted on all samples.ResultsPemphigus vulgaris was the most common autoimmune cutaneous disorder constituting 37% (n = 10) in prospective and 36% (n = 9) in the retrospective study. DIF showed a specificity of 81.25% in our prospective study. While on the paraffin-embedded sections, it showed a specificity of 66.6% in our retrospective study. In the prospective study, DIF on paraffin-embedded sections had a positivity rate of 43.75% as compared to 81.25% in DIF done on snap-frozen sections.ConclusionDIF is a sensitive tool for the diagnosis as well as distinguishing immune-mediated bullous disorders from other lesions primarily when performed on snap-frozen sections. The diagnostic yield is enhanced by DIF in cases that pose a diagnostic dilemma both clinically and histologically. The final diagnosis depends on all clinical, histopathological and immunofluorescence findings.

Immunohistochemical Expression of Galectin-3 in Pemphigus Vulgaris.

Pemphigus vulgaris (PV) is an autoimmune bullous disorder related to immunoglobulin-G autoantibodies against desmoglein-3. Galectin-3 is one of the main elements of the immunoglobulin-E group which is essential in the cell-cell or cell-matrix adhesion. Although the presence of immunoglobulin-E autoantibodies in PV has been observed, no studies have been performed to describe the role of galectin-3 in PV. We evaluated galectin-3 expression in PV as a first step in assessing its impact in the pathogenesis of this autoimmune blistering process. In a retrospective study, 56 specimens from 45 patients diagnosed with PV were stained with antibodies to galectin-3. The percentages of nuclear and cytoplasmic galectin-3 expression as well as staining intensity were evaluated around blisters and adjacent unaffected skin. We observed a significant decrease in galectin-3 cytoplasmic and nuclear expression as well as stain intensity around blisters compared with adjacent unaffected skin. Although autoantibodies against desmogleins trigger the blister formation in PV patients, loss of galectin-3 may play a role in the extension of blister formation by initiating cell-cell disassembly at the level of the intercellular keratinocyte desmosome. We demonstrated a lower expression of galectin-3 around the blisters in PV. The pathogenesis of the blister formation may be related to lower expression of galectin-3. Additional studies are necessary to clarify the result of this outcome and determine the accurate pathogenesis of blister formation in PV.

Frequency of Various Dermatoses Requiring Histopathological Evaluation For Definite Diagnosis; A Retrospective Analysis

Objective: To assess the frequency of various dermatological disorders those require histopathological analysis for definite diagnosis. Methods: We included 669 patients of either gender and all ages in this retrospective study, carried out in the Department of Dermatology, Jinnah Hospital, Lahore for a duration of 2 years. Their clinical data and histopathological reports were analyzed. The dermatoses were categorized into (a) papulosquamous disorders, (b) bullous disorders, (c) eczemas, (d) neoplasia, (e) granulomatous disorders, (f) connective tissue diseases, (g) drug reactions, (h) vasculitides, (i) chronic ulcers and (j) miscellaneous disorders. Results: The frequency of various dermatoses noted were as follows: papulosquamous disorders 28.25%, bullous disorders 11.5%, granulomatous disorders 11.5%, connective tissue diseases 10.61%, miscellaneous disorders 10%, eczemas 9.4%, chronic ulcers 7.17 neoplasia 6.8%, vasculitides 2.84% and drug reactions were 1.79% of total biopsies. Conclusion: The frequency of different diagnostic groups was unique in some respects and confirmed to other studies in others. The significantly high frequency of papulosquamous disorders highlighted the importance of these disorders. Key words: Skin biopsy, papulosquamous disorders, bullous disorders, eczemas, neoplasia, granulomatous disorders, connective tissue diseases, drug reactions, vasculitides, chronic ulcers.

A retrospective cohort study reporting rituximab treatment for 33 patients with immunobullous disease.

Autoimmune bullous disorders, encompassing pemphigus and pemphigoid diseases, are associated with significant morbidity and mortality. This is in part due to high cumulative doses of corticosteroids in combination with immunosuppressant agents used in traditional treatment regimes. Rituximab is an antiCD20 monoclonal antibody which can induce complete remission, but it is currently unlicensed in the UK and approved only after other treatments have failed. We report a retrospective cohort study of 33 patients with pemphigus and pemphigoid diseases treated with rituximab from a single tertiary centre from 2013 to 2019. "Complete remission off therapy" was achieved by 27.3% (n=9), and a further 27.3% (n=9) had complete remission on minimal therapy. Twenty-one per cent (n=7) had "partial remission on minimal therapy"; 9.1% (n=3) patients were in the "consolidation phase," and 12.1% (n=4) had a "relapse/flare." A steady reduction in prednisolone doses was observed post-Rituximab infusion. Pre-Rituximab the median dose of prednisolone was 20mg (range 10-35, IQR 25), 15mg (range 9.5-22.5, IQR 13) at 1month, 9mg (range 5-10, IQR 5) at 6months, 4mg (range 0-5mg, IQR 5) at 12months and 0 (0-4.35, IQR 4.25) at 18months. Twelve per cent (n=4) of patients had documented infusion reaction symptoms. Twelve per cent (n=4) had later infective complications. This real clinic data adds to the evidence that Rituximab is a safe and effective treatment for both pemphigus and pemphigoid autoimmune blistering conditions. Significantly, we were able to demonstrate a substantial reduction in corticosteroid dosage in our cohort of patients following rituximab 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.

Detecting Lesional Granulysin Levels for Rapid Diagnosis of Cytotoxic T lymphocyte–Mediated Bullous Skin Disorders

Bullous skin disorders are induced by different pathomechanisms and several are emergent, including Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). Rapid diagnostic methods for SJS/TEN or cytotoxic T-lymphocyte (CTL)-mediated bullous disorders are crucial for early treatment. Granulysin, primarily expressed by CTLs, is a specific cytotoxic protein responsible for SJS/TEN and similar skin reactions. To assess granulysin levels in blister fluids to differentiate SJS/TEN and similar CTL-mediated bullous reactions from other autoimmune bullous disorders. Using ELISA, we measured granulysin in blister fluids from patients with bullous skin disorders, including SJS/TEN, erythema multiforme major, bullous fixed-drug eruption, bullous lupus erythematosus, paraneoplastic pemphigus, pemphigus vulgaris, bullous pemphigoid, purpura fulminans-related bullae, and hand-foot syndrome/hand-foot-skin reactions. We compared serum and blister granulysin levels in patients with SJS/TEN presenting varying severity, monitoring serial granulysin levels from acute to late stages. Overall, 144 patients presenting with bullous skin disorders were enrolled. Blister granulysin levels (mean ± SD) in CTL-mediated disorders, including TEN (n= 28; 3938.7 ± 3475.7), SJS-TEN overlapping (n= 22; 1440.4 ± 1179.6), SJS (n= 14; 542.0 ± 503.2), erythema multiforme major (n= 7; 766.3 ± 1073.7), generalized bullous fixed-drug eruption (n= 10; 720.4 ± 858.3), and localized bullous fixed-drug eruption (n= 16; 69.0 ± 56.4), were significantly higher than in non-CTL-mediated bullous disorders (P < .0001), including bullous lupus erythematosus (n= 3; 22.7 ± 20.1), paraneoplastic pemphigus (n= 3; 20.3 ± 8.6), pemphigus vulgaris (n= 3; 4.4 ± 2.8), bullous pemphigoid (n= 18; 4.0 ± 2.7), purpura fulminans (n= 4; 5.9 ± 5.5), and hand-foot syndrome/hand-foot-skin reactions (n= 6; 4.6 ± 3.5). Blister granulysin levels correlated with clinical severity of SJS/TEN (P < .0001). Determination of blister granulysin levels is a noninvasive and useful tool for rapid differential diagnosis of SJS/TEN and other similar CTL-mediated bullous skin disorders for treatment selection.

A Case of Epidermolysis Bullosa in Bhuth: A Case Report

Epidermolysis Bullosa (EB) is a group of inherited Bullous disorders characterized by formation of blistering following minor trauma (Nikolksy Sign), healing with scarring. It is caused by one or more mutations in at least one of 20 different genes that synthesize structural proteins that are involve in adherence of epidermal to dermis. We observed and cared for a 2 day old female neonate with low birth weight, normal antenatal history who presented at birth with spontaneous bullous skin eruptions to parts of skin of the upper and lower limbs which ruptured with minor mechanical trauma. She had conservative care and even though there was lack of finances for genetic test to pinpoint the mutated gene the wounds healed with acceptable scar. We are glad to share this rare condition with the scientific world.

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.

Psychiatric comorbidity in individuals with bullous pemphigoid and all bullous disorders in the Danish national registers

BackgroundBullous pemphigoid (BP) is an autoimmune blistering skin disease that takes a profound physical and mental toll on those affected. The aim of the study was to investigate the bidirectional association between BP and all bullous disorders (ABD) with a broad array of psychiatric disorders, exploring the influence of prescribed medications.MethodsThis nationwide, register-based cohort study encompassed 6,470,450 individuals born in Denmark and alive from 1994 to 2016. The hazard ratios (HRs) of a subsequent psychiatric disorder in patients with BP/ABD and the reverse exposure and outcome were evaluated.ResultsSeveral psychiatric disorders were associated with increased risk of subsequent BP (4.18-fold for intellectual disorders, 2.32-fold for substance use disorders, 2.01-fold for schizophrenia and personality disorders, 1.92–1.85-1.49-fold increased risk for organic disorders, neurotic and mood disorders), independent of psychiatric medications. The association between BP and subsequent psychiatric disorders was not significant after adjusting for BP medications, except for organic disorders (HR 1.27, CI 1.04–1.54). Similar results emerged with ABD.ConclusionPsychiatric disorders increase the risk of a subsequent diagnosis of BP/ABD independent of medications, whereas medications used for the treatment of BP/ABD appear to account for the subsequent onset of psychiatric disorders. Clinically, an integrated approach attending to both dermatological and psychiatric symptoms is recommended, and dermatologists should remain vigilant for early symptoms of psychiatric disorders to decrease mental health comorbidity.