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Pemphigus forms 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. It is characterized by the production of pathogenic autoantibodies directed against different proteins of the desmosome, leading histologically to intraepidermal cleavage, and clinically to vesicles and erosions on the epithelium of the mucous membranes and/or the skin. The diagnosis of the subtype of pemphigus is based on clinical features, the level of histologic cleavage, and the identification of the antigens recognized by circulating autoantibodies by immunoserological analyses. The epidemiological features of pemphigus vary considerably in different regions of the world. Observational studies examining comorbidities and associations among patients with pemphigus are scarce and sometimes inconclusive. The prognosis, mortality, and clinical outcomes in pemphigus have undergone dramatic change throughout the years. This review provides a brief overview about the different subtypes of pemphigus: pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, pemphigus herpetiformis, and IgA pemphigus. In addition, it summarizes the most recent understanding of the epidemiology, mortality data, and comorbidities of this group of organ-specific autoimmune diseases.

Subepidermal autoimmune bullous diseases of the skin and mucosae comprise a large group of chronic diseases, including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. These diseases are characterized by an antibody response toward structural components of the basement membrane zone, resulting in subepidermal blistering. The epidemiological features of these diseases vary substantially in different regions of the world. Observational studies investigating comorbidities and associations among patients with these diseases are inconsistent and sometimes inconclusive. This review provides a brief overview regarding each one of the subepidermal autoimmune bullous diseases. In addition, it summarizes the most recent understanding of the epidemiological features and associations of this group of organ-specific autoimmune diseases.

Pemphigus vulgaris (PV) is a life-threatening disease belonging to the pemphigus group of autoimmune intra-epidermal bullous diseases of the skin and mucosae. The therapeutic management of PV remains challenging and, in some cases, conventional therapy is not adequate to induce clinical remission. The cornerstone of PV treatment remains systemic corticosteroids. Although very effective, long-term corticosteroid administration is characterized by substantial adverse effects. Corticosteroid-sparing adjuvant therapies have been employed in the treatment of PV, aiming to reduce the necessary cumulative dose of corticosteroids. Specifically, immunosuppressive agents such as azathioprine and mycophenolate mofetil are widely used in PV. More recently, high-dose intravenous immunoglobulins, immunoadsorption, and rituximab have been established as additional successful therapeutic options. This review covers both conventional and emerging therapies in PV. In addition, it sheds light on potential future treatment strategies for this disease.

Bullous pemphigoid (BP) is the most common type of subepidermal autoimmune bullous diseases. BP characteristically affects the elderly and is seen mainly in patients older than 70 years. While the annual incidence of BP has been estimated to be between 2.4 and 23 cases per million in the general population, it rises exponentially to 190-312 cases per million in individuals older than 80 years. In addition, a growing body of evidence reports a remarkable trend of increased incidence of BP, showing a 1.9- to 4.3-fold rise over the past two decades. This demonstrable increase warrants a higher awareness of the increased risk to develop BP. This review summarizes the current understanding of the epidemiological features of BP and sheds light on the putative explanations for its growing incidence.

Abstract Objective Individuals with schizophrenia may be at an increased risk for COVID-19 morbidity due to the disease characteristics. In this study, we aimed to explore the odds of significant COVID-19 morbidity and mortality among schizophrenia patients while controlling for potential sociodemographic and medical confounders. Methods Schizophrenia patients and age-and-sex matched controls (total n = 51 078) were assessed for frequency of COVID-19 positivity, hospitalizations, and mortality. The odds for COVID-19-associated hospitalization and mortality were calculated using logistic regression models, while controlling for age, sex, marital status, sector, socioeconomic status, diabetes, ischemic heart disease, hypertension, hyperlipidemia, obesity, smoking, and chronic obstructive pulmonary disease. Results Individuals with schizophrenia were less likely to test positive for COVID-19; however, they were twice as likely to be hospitalized for COVID-19 (OR 2.15 95% CI 1.63–2.82, P < .0001), even after controlling for sociodemographic and clinical risk factors (OR 1.88 95% CI 1.39–2.55, P < .0001). Furthermore, they were 3 times more likely to experience COVID-19 mortality (OR 3.27 95% CI 1.39–7.68, P < .0001), compared to controls. Conclusions We found evidence of associations between schizophrenia and increased COVID-19 morbidity and mortality compared to controls regardless of sociodemographic and medical factors. As these patients present with a combination of potential risk factors for mortality, efforts should be made to minimize the effects of the pandemic on this vulnerable population.

The many clinical aspects of anti-p200 pemphigoid are not well-characterized. We aimed to analyze and correlate known existing data on the epidemiological, clinical, histological, and immunological features of anti-p200 pemphigoid. We performed a review using Medline, Embase, and Web of Science databases (1900-2018). Case reports and series of patients were included. A total of 68 eligible studies that comprised 113 anti-p200 pemphigoid patients were included in the qualitative analysis, where there was a mean age of onset of 65.5 years. All patients presented with bullae/vesicles, and 54.3% had urticarial plaques. A similarity to bullous pemphigoid was reported in 66.1% of cases, but palmoplantar (51.4%), cephalic (40.3%), and mucosal (38.5%) involvement, besides frequent development of scars/milia (15.7%), were reported. Autoantibodies against recombinant laminin γ1 were detected in the sera of 73.1% of patients. Psoriasis was present in 28.3% of anti-p200 pemphigoid patients, particularly among Japanese patients (56.4%). The incidence of pustular psoriasis in this subgroup, was significantly greater than in the normal population. In conclusion, the diagnosis of anti-p200 pemphigoid may be suspected when a subepidermal autoimmune blistering disease develops in a younger age group, along with significant acral and cephalic distribution and mucosal involvement.

Randomized controlled trials (RCTs) are the gold standard for evaluating the efficacy and safety of both pharmacological and non-pharmacological interventions. However, while they are designed to control confounders and ensure internal validity, their usually stringent inclusion and exclusion criteria often limit the generalizability of findings to broader patient populations. Moreover, RCTs are resource-intensive, frequently underpowered to detect rare adverse events, and sometimes narrowly focused due to their highly controlled environments. In contrast, real-world data (RWD), typically derived from electronic health records (EHRs) and claims databases, offers a valuable counterpart for answering research questions that may be impractical to address through RCTs. Recognizing this, the US Food and Drug Administration (FDA) has increasingly relied on real-world evidence (RWE) from RWD to support regulatory decisions and post-market surveillance. Platforms like TriNetX, that leverage large-scale RWD, facilitate collaborations between academia, industry, and healthcare organizations, and constitute an in-depth tool for retrieval and analysis of RWD. TriNetX’s federated network architecture allows real-time, privacy-compliant data access, significantly enhancing the ability to conduct retrospective studies and refine clinical trial designs. With access to currently over 150 million EHRs, TriNetX has proven particularly effective in filling gaps left by RCTs, especially in the context of rare diseases, rare endpoints, and diverse patient populations. As the role of RWD in healthcare continues to expand, TriNetX stands out as a critical tool that complements traditional clinical trials, bridging the gap between controlled research environments and real-world practice. This review provides a comprehensive analysis of the methodologies and applications of the TriNetX platform, highlighting its potential contribution to advance patient care and outcomes.

[4] Following the aforementioned routine assays available in most immunopathological laboratories, the challenge is to differentiate between EBA, anti-p200 pemphigoid, and anti-laminin 332 MMP, because in all of these diseases, autoantibodies deposit on the floor of the artificial blister when IIF microscopy is performed on salt-split human skin. [7] demonstrated that type IV collagen was demonstrated in the epidermal roof of the blister whenever this immunohistochemical stain was reactive. Since the level of separation is identical in both EBA and DEB, it is conceivable that similar pattern will be observed in EBA. More sophisticated diagnostic approaches In those patients with inconclusive serration pattern and nonreactive type IV collagen immunostaining, who do not show serum reactivity against type VII collagen, the diagnosis of EBA may be made by fluorescence overlay antigen mapping technique or direct immunogold electron microscopy considered as the gold standard of diagnosis.

Although Alopecia areata (AA) has been found to be associated with psychological distress, the scope and nature of this association has not been fully delineated. The current study sought to examine the association of AA with anxiety, depression, schizophrenia, and bipolar disorder, utilizing a large-scale matched controlled cohort design. Patients suffering from AA ( n  = 41,055) were matched to control cases ( n  = 41,055) by age, sex, and socioeconomic status (SES). The prevalence of the four major mental disorders was assessed while stratifying the sample by age and sex, and after adjusting for marital status, smoking, BMI, hypertension, and diabetes. Data were accessed via the Clalit Health Services (CHS) database, a comprehensive health registry utilized by the largest managed healthcare company in Israel. Anxiety was independently and positively associated with AA (OR 1.22, 95% CI 1.13–1.31, p  < 0.001), across all age groups above 30, with similar rates in males and females. Depression was also independently and positively associated with AA (OR 1.09, 95% CI 1.01–1.17, p  < 0.005), particularly in the 30–49 age group, with a higher association among females. A negative association was found between AA and schizophrenia (OR 0. 71, 95% CI 0.61–0.83, p  < 0.001). No association was found between AA and bipolar disease. Patients with AA are at risk for anxiety and depression, with female patients, and patients in the 30–49 age group being particularly vulnerable to develop a co-occurring mental disorder. Medical treatment should therefore include psychiatric evaluation and appropriate care.

Lichen planus (LP) is a T cell-mediated disease affecting the stratified squamous epithelia of the skin and/or mucus membrane. Histologically, the disease is characterized by a lichenoid inflammatory infiltrate and vacuolar degeneration of the basal layer of the epidermis. LP has three major subtypes: Cutaneous, mucosal and appendageal LP. Rarely, it may affect the nails in the absence of skin and/or mucosal changes. LP may also be induced by several drugs, typically anti-hypertensive medication or be associated with infections, particularly viral hepatitis. The diagnosis is based on the clinical presentation and characteristic histological findings. Although the disease is often self-limiting, the intractable pruritus and painful mucosal erosions result in significant morbidity. The current first-line treatment are topical and/or systemic corticosteroids. In addition, immunosuppressants may be used as corticosteroid-sparing agents. These, however are often not sufficient to control disease. Janus kinase inhibitors and biologics (anti-IL-12/23, anti-IL17) have emerged as novel future treatment options. Thus, one may expect a dramatic change of the treatment landscape of LP in the near future.