Autoimmune Bullous Diseases Patients Research Articles

Characteristics and Risk Factors of Infections among Patients with Autoimmune Bullous Diseases: A Retrospective Single-Center Study in China

Abstract Background: Since infections can lead to adverse outcomes in autoimmune bullous diseases (AIBD), this study aimed to analyze infection characteristics in AIBD, predict infection risk factors and provide probable suggestions to infection prevention, which will spot a light on early discovery and prevention of infections in AIBD patients. Methods: This is a retrospective study of the medical records of inpatients diagnosed with AIBD at the Department of Infectious Diseases and Dermatology, Huashan Hospital from January 2017 to December 2021. We collected patients’ clinical manifestations and laboratory examination results. Risk factors for infections were evaluated using multivariate logistic regression. Results: A total of 263 AIBD patients were included in the study. The overall incidence of infection was 42.2% (111/263). Pemphigus had a higher infection rate (74/142, 52.1%) than bullous pemphigoid (37/121, 30.6%). Among the patients with infections, 49.5% (55/111) had bacterial infections, 13.5% (15/111) had fungal infections, 2.7% (3/111) had virus infections, and 34.2% (38/111) had mixed infections. Staphylococcus aureus and Candida were the most common pathogens in localized infections, while Cryptococcus and Candida were predominant in systemic infections. In the multivariate logistic model, pemphigus (odds ratio [OR] = 2.56, 95% confidence interval [CI] 1.41–4.63), hypoalbuminemia (OR = 3.78, 95% CI 1.68–8.50), and systemic glucocorticoid treatment (OR = 4.67, 95% CI 2.51–8.68) were independent risk factors for infection. Bullous pemphigoid (OR = 3.99, 95% CI 1.03–15.45), high-dose (average > 24 mg/day) compared to low-dose (average ≤ 6 mg/day) and medium-dose (average 6–24 mg/day) systemic glucocorticoid treatment (OR = 19.31, 95% CI 3.32–112.28, and OR = 5.71, 95% CI 1.37–23.79) were associated with increased risk factors of systemic infection. A methylprednisolone dose averaging 14 mg/day over the past three months has a 75.0% positive predictive value (PPV) for infections in AIBD patients. Conclusions: The incidence of infection in AIBD patients is high, with a broad spectrum of pathogens and infection locations. AIBD patients undergoing systemic glucocorticoid treatment, especially those receiving high doses in the previous three months, should be cautious about infection.

The Impact of the Initial Phase of the COVID-19 Pandemic on Patients With Autoimmune Bullous Diseases.

Introduction The follow-up of patients with autoimmune bullous diseases (AIBDs) was temporarily interrupted during the initial phase of the COVID-19 pandemic due to restrictions in healthcare services, given the high contagiousness and rapid spread of SARS-CoV-2. Our objective was to assess the impact of the initial phase of the COVID-19 pandemic on the treatments and disease activity of AIBD patients. Methods We conducted a telephone survey of patients with AIBDs who had been regularly followed up in our hospital prior to the onset of the pandemic. A structured questionnaire that we designed was used. This questionnaire comprised questions examining the following issues between March and June of 2020: patients' follow-up, treatment, COVID-19 infection status, and changes in disease activity. Results Thirty-nine patients were included in the study. Among those, 26 (66.7%) were immunosuppressed. The frequency of follow-up for 37 patients (94.9%) changed significantly (p<0.001): 28 patients (71.8%) did not visit the hospital, and 26 of them (92.9%) did not communicate at all. The treatment for 10 patients (25.6%) was altered, either by their physician or by themselves. Disease activity reactivated in patients who altered their own treatments. There was only one patient (2.6%) who contracted COVID-19. Conclusions Documenting this period revealed that some patients were negatively impacted by the pandemic initially. The most significant contributing factor was the interruption of patient-physician communication.

Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center.

Background and Objectives: Autoimmune bullous diseases (AIBDs) may be treated with intravenous immunoglobulin (IVIG) infusions. This study aimed to evaluate the benefits and safety profiles of high-dose IVIG therapy in AIBD patients, as determined by clinical remission, the glucocorticosteroid-sparing effect, and adverse events at 12 months follow-up in a Central European university dermatology department setting. Materials and Methods: Our case series included 10 patients: five patients with pemphigus vulgaris, one with pemphigus herpetiformis, one with pemphigus foliaceus, one with bullous pemphigoid, two with epidermolysis bullosa acquisita. They underwent 4-12 monthly cycles of IVIG therapy at a dose of 2 g/kg per cycle. Results: The prednisone dosage reduction after 2, 6, and 12 months following the final IVIG course was 65.45%, 70.91%, and 76.37%, respectively. During the 12-month observation period, disease relapse was observed in 20% of patients, while others achieved complete or partial remission without or with minimal therapy. Side effects were seen in 80% of patients; they were transient and did not necessitate discontinuation of IVIG. Conclusions: IVIG demonstrates effectiveness as a treatment with a favorable safety profile. Nevertheless, its high cost remains a significant drawback, particularly in low-income countries. IVIG should be considered, especially in patients opposed to standard therapies or with contraindications to their use.

Outcome of COVID-19 in patients with autoimmune bullous diseases.

Background Data on outcomes of Coronavirus disease 2019 (COVID-19) infection in autoimmune bullous diseases (AIBDs) patients is scarce. Materials and methods This single-centre survey-based-observational study included patients registered in the AIBD clinic of the Postgraduate Institute of Medical Education and Research, Chandigarh, India. All registered patients were contacted over telephone between June and October 2021. A survey was conducted after obtaining informed consent. Results Among 1389 registered patients, 409 completed the survey. Two hundred and twenty-two (55.3%) patients were females and 187 (45.7%) were males. The mean age was 48.52 ± 14.98 years. Active disease was reported by 34% patients. The frequency of COVID-19 infection in responders was 12.2% (50/409), with a case-fatality ratio of 18% (9/50). Rituximab infusion after the onset of pandemic significantly increased the risk of COVID-19 infection. Active AIBD and concomitant comorbidities were significantly associated with COVID-19 related death. Limitation Relative risk of COVID-19 infection and complications among AIBD patients could not be estimated due to lack of control group. The incidence of COVID-19 in AIBD could not be determined due to lack of denominator (source population) data. Other limitations include telephonic nature of the survey and lack of COVID-19 strain identification. Conclusion Use of rituximab is associated with higher probability of COVID-19 infection, while advanced age, active disease and presence of comorbidities may increase the risk of COVID-19 mortality in AIBD patients.

Autoimmune bullous diseases during COVID-19 pandemic: 2022 update on rituximab and vaccine.

Autoimmune bullous diseases (AIBDs) are a heterogeneous group of life-threatening disorders associated with subepidermal or intraepidermal blistering. Skin barrier alterations and prolonged immunosuppressive treatments increase the risk of infections in patients with AIBDs, who are considered fragile. COVID-19 pandemic had a heavy impact on these patients. Although advances have been made in terms of prevention and treatment of COVID-19, this topic remains significant as the pandemic and its waves could last several years and, so far, a relevant proportion of the population worldwide is not vaccinated. This review is a 2022 update that summarizes and discusses the pandemic's burden on AIBD patients mainly considering relevant studies in terms of: (i) sample dimension; (ii) quality of control populations; (iii) possible standardization by age, gender and country. The findings show that: (i) the risk of COVID-19 infection and its severe course were comparable in AIBD patients and in the general population, except for rituximab-treated patients that presented a higher risk of infection and severe disease; (ii) the mortality rate in COVID-19-infected bullous pemphigoid patients was higher than in the general population, (iii) 121 cases of AIBD onset and 185 cases of relapse or exacerbation occurred after COVID-19 vaccination and a causal relationship has not been demonstrated so far. Altogether, acquired knowledge on COVID-19 pandemic could also be important in possible, albeit undesirable, future pandemic scenarios.

Assessing quality of life in patients with autoimmune bullous diseases using the Persian version of Treatment of Autoimmune Bullous Disease Quality of Life questionnaire finds similar effects in women as men.

Background:In autoimmune bullous diseases (AIBDs), autoantibodies loosen molecular adhesions in the skin and/or mucosa and lead to blisters and erosions. Immunosuppressive drugs reduce mortality of the AIBD; therefore, patients will have to live longer with comorbidities.Objective:This study aims to determine the quality of life of AIBD patients undergoing systemic treatment while investigating the survey’s relationship with various factors.Methods:In this 2-step cross-sectional study, we initially included 53 consecutive pemphigus patients to investigate reliability and validity of the Persian version of Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaire. Then, we conducted the study on 119 AIBD patients, currently under treatment at an AIBD clinic in Iran.Results:The mean TABQOL score for our patients was 13.87 ± 7.51. The highest TABQOL was for epidermolysis bullosa acquisita (24 ± 8.485) followed by pemphigus foliaceus (20.5 ± 14.181) and the lowest for pemphigus vulgaris (13.24 ± 6.54). There was no significant difference between patients’ TABQOL scores and their gender, history of rituximab injection, and disease severity scores. We only found a positive correlation between TABQOL and prednisolone dose.Conclusion:Treatments of AIBD considerably impact the quality of life of patients and an impairment in quality of life is correlated to higher doses of prednisolone.

Incidence of pemphigoid diseases in Northern Germany in 2016 - first data from the Schleswig-Holstein Registry of Autoimmune Bullous Diseases.

Autoimmune bullous diseases (AIBD) are rare disorders characterized by autoantibody formation against components of adhesion molecules; in pemphigoid diseases (PD), these are proteins of hemidesmosomes and basement membrane, important for cell-matrix adhesion in skin and/or mucous membranes. Incidences of these diseases vary considerably between different populations. To establish a registry prospectively recruiting all AIBD patients in a geographically well-defined region in Northern Germany (Schleswig-Holstein). Only patients with verified disease (by clinical presentation, histology, direct and/or indirect immunofluorescence and /or ELISA) living in Schleswig-Holstein were included. Incidences of PD were estimated based on the total number of inhabitants in Schleswig-Holstein, stratified by birth year and sex. Of 67 patients with PD [35 male, 32 female, mean age 75 (standard deviation 14.3years)], 83% were patients with bullous pemphigoid [n=56, 28 male, 28 female, mean age 78 (SD 9.9)]. The resulting crude incidences were 23.4 patients/million/year for all pemphigoid patients, 19.6 patients/million/year for bullous pemphigoid (age-standardized 16.9 patients/million/year) with a strong increase in bullous pemphigoid patients in the age group of 85-90years with 262 patients/million/year. Incidences for bullous pemphigoid were higher in urban compared to rural areas. Other PD (mucous membrane pemphigoid, linear IgA disease, anti-p200 pemphigoid) were less frequent with crude incidences of 2.1, 1.0 and 0.7 patients/million/year, respectively. This study prospectively analyses the incidence of PD in a carefully defined geographical area. The highest incidence among PD patients was found for bullous pemphigoid. The incidence of bullous pemphigoid is considerably increased compared to previous reports and reveals regional differences. Further studies are needed in order to clarify these findings.

Characteristics and outcomes of COVID-19 in patients with autoimmune bullous diseases: A retrospective cohort study

Characteristics and outcomes of COVID-19 in patients with autoimmune bullous diseases: A retrospective cohort study

Spectrum of autoimmune bullous diseases among children in Kuwait.

Autoimmune bullous diseases (AIBD) are rare among children. The data describing the overall spectrum and prognosis of pediatric AIBD (pAIBD) are scarce, and there are no established treatment guidelines. The present study examined the spectrum, clinical characteristics, and long-term prognosis of pAIBD in a tertiary care pediatric dermatology unit. Retrospective records of all pAIBD cases (<18years) registered over a span of 28years were analyzed. Records of 23 cases of pAIBD, including 16 boys and 7 girls, were reviewed. They constituted 8.5% of total AIBD patients from all age groups. Ninety-one percent of patients were of Arab ethnicity. Linear IgA bullous dermatosis was the most prevalent AIBD followed by bullous pemphigoid, bullous lupus erythematosus, and pemphigus variants (pyostomatitis-pyodermatitis vegetans and neonatal pemphigus). The mean age of onset and diagnosis was 6.4 and 7.7years, respectively. Systemic treatments, including systemic corticosteroids and dapsone, were required in most cases. Intravenous immunoglobulin G (IVIG) was also utilized as second-line therapy. The mean follow-up period was 76months with 90% of the patients in complete remission. AIBD pose a great challenge among children both in diagnosis and treatment. This study highlights the ethnic variability and underscores the need for additional similar, international studies to achieve a better understanding of the burden related to pAIBD and help establish treatment guidelines.

Rituximab in childhood and juvenile autoimmune bullous diseases as first-line and second-line treatment: a case series of 13 patients

Background Little data are available concerning the outcome of rituximab (RTX) therapy in pediatric patients with autoimmune bullous diseases (AIBDs). Objective We sought to evaluate safety and efficacy of RTX administration in pediatric patients with AIBDs and to assess first-line RTX therapy in pemphigus patients. Methods AIBD patients consisting of 12 pemphigus patients and a patient with bullous pemphigoid who received RTX before the age of 18 were enrolled. Detailed information regarding patients’ outcome after the first RTX cycle was assessed. Results The mean age of the patients at RTX infusion was 15 ± 2 years. Six patients in the pemphigus group received RTX as first-line therapy. In pemphigus patients: complete remission (on minimal therapy) was achieved by seven patients, partial remission (on minimal therapy) and complete remission (off therapy) were achieved by three patients and one, respectively. Relapse occurred in nine patients, which were mostly mild. Likewise, the BP patient received RTX with a good clinical response. The observed adverse events were mostly mild infusion reactions and a case of sepsis. Conclusion Rituximab is safe and effective in childhood/juvenile patients with AIBDs. Furthermore, RTX can be used as first-line treatment in pediatric patients with pemphigus.

Modern diagnostics of autoimmune bullous diseases

Blisters and erosions of skin and mucous membranes are key features of the clinically heterogeneous group of autoimmune bullous diseases (AIBDs). These can be divided into pemphigoid diseases with autoantibodies against structural proteins of the dermal-epidermal junction, pemphigus diseases with autoantibodies against desmosomal proteins, and dermatitis herpetiformis with autoantibodies against transglutaminases1 and2. Adifferentiation based only on clinical features is often not sufficient. After researching the literature in PubMed, the current diagnostic tools for AIBDs are summarized.AIBD diagnostics are performed using histology, direct and indirect immunofluorescence, as well as ELISA and immunoblotting. For serological diagnosis, the conventional multistep approach or multivariant assays for the analysis of autoantibodies against several target antigens in parallel can be applied. These allow aprecise classification of AIBD and therefore atailored use of different therapeutic regimens, e.g., for bullous pemphigoid or pemphigus foliaceus/vulgaris, as well as identification of disease entities with aknown association with neoplasia.Direct immunofluorescence is still the diagnostic mainstay of AIBDs. However, novel serological assays, such as target-antigen-specific ELISA or indirect immunofluorescence systems using BIOCHIP™ mosaic technology, allow serologic diagnosis in most AIBD patients and the exact classification of the disease entity at the molecular level.

Management strategies of autoimmune bullous diseases during the outbreak of 2019 Novel Coronavirus disease (COVID-19)

Since late December 2019, an outbreak of the novel coronavirus disease, which is mainly characterized by pulmonary lesions, has spread quickly worldwide. With the spread of the novel coronavirus, the outpatient lines of hospitals have mostly shut down, which means that routine clinical diagnosis and treatment for autoimmune bullous diseases patients have been disturbed. Due to the serious condition of autoimmune bullous diseases patients, they are prone to immune suppression and circulatory failure, and are more susceptible to infection than healthy individuals. These patients should thus be a priority group for novel coronavirus disease prevention. In this context, the protection and control measures for autoimmune bullous diseases patients against the novel coronavirus are of serious concern. Clinicians should strengthen their communication with patients, pay attention to changes in patients’ conditions, and carefully adjust the treatment strategy, while protecting against the novel coronavirus infection, to ensure the continuity, safety, and effectiveness of autoimmune bullous diseases treatment.

Analysis of the utility of direct immunofluorescence in the diagnosis of common immune mediated dermatological conditions

Background: Accurate diagnosis of immune-mediated dermatological diseases requires an evaluation of clinical, histopathological, and immunofluorescence findings. Immunofluorescence detects antibodies bound to antigens in tissue and in circulating body fluids. It acts as a valuable adjunct to clinical and histopathological diagnosis in patients of autoimmune bullous diseases (AIBDs), connective tissue diseases (CTD), and vasculitis. Aim: This study aimed to analyze the utility of direct immunofluorescence (DIF) in the diagnosis of common immune-mediated dermatological diseases by its correlation with clinical features and histopathology. Methodology: This is an observational retrospective study. Medical records of 205 clinically suspected patients of AIBD, CTD, and vasculitis were retrieved for analysis where both histopathology and DIF had been carried out for patients treated at the dermatology department of a tertiary care hospital between November 2015 and November 2018. Data were analyzed using Statistical Package for the Social Sciences software version 20.0. Results: A total of 187 out of 205 patients were accurately diagnosed based on histopathology and DIF studies. Histopathology was positive in 177/187 (95.7%) patients and DIF was positive in 153 (81.8%) patients. Clinical, histopathological, and DIF discordance was seen in the remaining 18 out of 205 patients. The sensitivity of DIF in AIBD, lupus erythematosus (LE), and vasculitis was calculated to be 89%, 82.6%, and 60%, respectively. The overall sensitivity of DIF was 80.8%. The sensitivity of DIF was 100% in pemphigus foliaceous, acute cutaneous lupus erythematosus, and immunoglobulin A (IgA) vasculitis. Out of 118 clinically suspected AIBD patients, nine were diagnosed on the basis of DIF alone. Conclusion: DIF is an indispensable tool in the accurate diagnosis of autoimmune dermatological conditions, especially in cases where clinical and/or histopathological features are inconclusive. It is invaluable in confirming the diagnosis of IgA vasculitis.

Assessment of the quality of life of Egyptian and Tunisian autoimmune bullous diseases’ patients using an Arabic version of the autoimmune bullous disease quality of life and the treatment of autoimmune bullous disease quality of life questionnaires

AbstractBackground: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden.Objectives: We aimed to assess the ABQOL and TABQOL in the Arabic population.Methods: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again.Results: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach’s alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=−0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=−0.3, p value was 0.007.Study limitations: Small sample size of some AIBDs and patients with severe disease.Conclusion: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient’s distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.

Bacteremia in autoimmune bullous disease patients undergoing double-filtration plasmapheresis

Background: Plasmapheresis is one of the treatment options for autoimmune bullous disease (AIBD).Objective: To evaluate the incidence of adverse events occurring during a course of plasmapheresis.Methods: This study enrolled 42 courses of double-filtration plasmapheresis (DFPP) from 28 patients with AIBD treated in Kurume University Hospital between 2007 and 2016. We examined the frequency of adverse events during the course of DFPP and associated features.Results: The most frequent adverse event was bacteremia (13 of 42 courses, 31.0%), followed by subcutaneous hemorrhage (1 course, 2.4%), and an abscess at the catheterization sites (1 course, 2.4%). No adverse event-related death was recorded. In the analysis of bacteremia occurrence, the erosion at the catheterization sites, the use of central venous catheter (CVC), and the number of DFPP cycles per course were significantly more or greater in the bacteremia group than in the non-bacteremia group (p = .0474, .0005, and .0035, respectively).Conclusions: Although DFPP is a good treatment option for AIBD, attention needs to be paid for the development of possible risks during a DFPP course. We believe that our results can be applied to other plasmaphereses in AIBD management.

Autoimmune bullous diseases with gingival expression: A proposed non-iatrogenic gingival biopsy technique

Gingival expression of autoimmune bullous diseases (AIBD) may be inaugural, exclusive or dominant (mucous membrane pemphigoid, pemphigus vulgaris). Histology and direct immunofluorescence are essential to diagnosis. The location of the biopsy and the surgical technique determine the histological quality of the tissue sample. However, gingival tissue is often considered fragile and easily impaired during biopsy. We suggest an original biopsy protocol for the gingival papillae that is simple to perform, non-iatrogenic, and readily accessible to all practitioners who usually treat AIBD patients presenting isolated gingival expression (dermatologists, stomatologists, odontology specialists, ENT specialists). We conducted a retrospective study from 2012to 2017identifying all patients presenting AIBD with gingival expression for whom we performed papillary gingival biopsy for diagnostic ends. Our main objective was to determine the diagnostic efficacy and safety of this surgical technique. Over the study period, 34papillary gingival biopsies were taken from 19patients: 15for histopathological examination and 19for direct immunofluorescence. Of the 34biopsies, only one could not be properly analyzed due to lack of epithelium and a second tissue sample was therefore necessary. No short- or long-term complications occurredduring post-operative follow-up. Gingival papilla biopsy is perfectly suited to the histopathological and immunohistochemical examinations needed for diagnosis of AIBD with isolated gingival expression. This surgical technique shows great efficacy and very good safety. However, additional studies are necessary to confirm our preliminary results, in particular the absence of iatrogenic effects.

Patient-reported outcomes in clinical practice in autoimmune bullous disease patients

Patient-reported outcomes in clinical practice in autoimmune bullous disease patients

A distinct cutaneous microbiota profile in autoimmune bullous disease patients.

Bullous pemphigoid (BP) is the most common autoimmune blistering disease in Europe. As both the incidence of the disease and the relative proportion of the elderly population continue to rise, it represents a significant medical burden. Whereas some progress has been achieved in defining genetic risk factors for autoimmune blistering diseases, no environmental agent has been conclusively identified. Emerging evidence suggests that host immunity may influence the skin microbiota, while the latter modulates cutaneous immunity. Nevertheless, the relationship between skin microbial communities and autoimmune bullous disease has yet to be studied in humans. Here, we aim to characterise and compare the skin microbiome of patients with BP and healthy, age-matched controls at numerous body sites. Similar to what has been shown in healthy controls, the composition of skin microbiota in patients with BP appears to be very divergent and site specific. Microbial phylum abundances differ between perilesional sites of patients with BP and the same anatomic locations of control patients. A distinct cutaneous microbiota profile, which correlates with BP, further strengthens the significance of commensal-host interaction on our immune system. Moreover, these results raise the possibility that the cutaneous microbiome may contribute to the pathogenesis of BP, with important implications for the treatment of this disease.

Endoscopic findings of laryngopharyngeal and esophageal involvement in autoimmune bullous disease.

Autoimmune bullous disease (ABD) is induced by autoantibodies against cell adhesion molecules, and blistering may occur on the mucous membranes of the eyes, nose, mouth, oral cavity, laryngopharynx, and esophagus. Endoscopic prevalence and features of ABD-associated esophageal lesions are not well known. We conducted the present study to assess the endoscopic prevalence of ABD-associated mucosal lesions. Endoscopic prevalence of mucosal lesions, particularly laryngopharyngeal and esophageal lesions, was used as the primary endpoint to assess the significance of upper gastrointestinal endoscopy, and clinical and endoscopic features were secondary endpoints. Of 123 ABD patients, 50.4% had apparent oral or laryngopharyngeal lesions and 30.8% had laryngopharyngeal lesions. Esophageal lesions were detected through normal observation in 16.8% of affected patients, whereas 40.6% exhibited epidermolysis or blood blisters by mechanical inducement, regardless of esophageal mucosal lesion detection by normal observation. Additionally, 56.0% exhibited the Nikolsky sign with mechanical inducement. Of the 123 patients, 29.2% did not have exposed skin lesions. Of these patients, 77.7% had oral cavity or laryngopharyngeal lesions, 36.1% had esophageal lesions, and 58.3% exhibited the Nikolsky sign on esophageal mucosa. It is important to determine the endoscopic characteristics and findings of ABD. ABD can be suspected from endoscopic findings.

Possible triggering factors and comorbidities in newly diagnosed autoimmune bullous diseases.

The distribution and the demographic, etiological, and clinical features of autoimmune bullous diseases (ABDs) vary according to geographic regions of the world. The limited number of reported studies in Turkey are mostly retrospective and generally include only pemphigus. The aim of our study was to evaluate the demographic data, clinical features, associated triggering factors, and comorbidities in patients newly diagnosed with ABDs. In this descriptive and prospective study, the demographic data, inhabited regions, smoking and drinking habits, presence of comorbidities, and specific and general potential triggering factors of ABDs considered by the patients were examined. Furthermore, the distribution of lesions and autoimmune bullous skin disorder intensity scores were recorded. Of the total 59 ABD patients, 26 (44.1%) were diagnosed with pemphigus, 25 (42.4%) were diagnosed with pemphigoid, and 8 (13.5%) were diagnosed with dermatitis herpetiformis. Although there were some differences between the subgroups of ABDs, the onset of the disease was associated with a triggering factor (stress, foods, physical trauma, chemical agents, ultraviolet rays, medications, infections, malignancies, and pregnancy, in a decreasing frequency) in 96.6% of the patients. Autoimmune diseases, neuropsychiatric disorders, diabetes mellitus, and hypertension were found to be statistically significantly higher in the pemphigoid group compared to the pemphigus and dermatitis herpetiformis groups. Evaluation of triggering factors and comorbidities before starting treatment may assist in controlling the disease more swiftly and reducing the side effects of treatment in the management of ABDs. However, to evaluate the frequency and significance of our findings in order to determine whether they are statistically significant or not, we suggest a multicenter prospective clinical trial, which would include control groups and a higher number of patients for each of the disease groups and clinical subtypes.