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.
Isotretinoin-related risk of depression and suicidal behavior is a topic of inconclusiveness. A crucial knowledge gap exists in defining the association of isotretinoin with other psychiatric ...comorbidities.
To evaluate the risk of psychiatric outcomes among patients with acne treated with isotretinoin versus oral antibiotics.
A global population-based retrospective cohort study enrolled 2 groups of patients with acne managed by isotretinoin (n = 75,708) and oral antibiotics (n = 75,708). Patients were compared regarding the risk of 9 psychiatric outcomes.
Relative to those treated with oral antibiotics, patients prescribed isotretinoin experienced lower risk of depression (hazard ratio HR, 0.90; 95% confidence interval CI, 0.87-0.93; P < .001), but comparable risk of major depressive disorder (HR, 0.97; 95% CI, 0.92-1.03; P = .318). Risk of suicidal attempts was comparable between groups (HR, 0.97; 95% CI, 0.85-1.11; P = .663), despite the elevated risk of suicidal ideation in those under isotretinoin (HR, 1.41; 95% CI, 1.32-1.50; P < .001). Patients under isotretinoin had lower risk of post-traumatic stress disorder (HR, 0.75; 95% CI, 0.68-0.82; P < .001), anxiety (HR, 0.84; 95% CI, 0.82-0.87; P < .001), bipolar disorder (HR, 0.65; 95% CI, 0.59-0.72; P < .001), schizophrenia (HR, 0.60; 95% CI, 0.48-0.76; P < .001), and adjustment disorder (HR, 0.82; 95% CI, 0.77-0.87; P < .001).
Retrospective data collection.
Isotretinoin confers lower risk of 6 psychiatric comorbidities and comparable risk of suicidal attempts.
Autoimmune bullous dermatoses (AIBDs) are a group of rare chronic inflammatory skin diseases, which clinically manifest as blisters and erosions of the skin and/or mucosa. Immunologically, AIBDs are ...characterized and caused by autoantibodies targeting adhesion molecules in the skin and mucosa. According to the histological location of the blistering, AIBDs are classified into the following two main subtypes: pemphigus (intraepidermal blistering) and pemphigoid (subepidermal blistering). Most AIBDs were potentially life-threatening diseases before the advent of immunosuppressive drugs, especially systemic steroid therapies, which suppress pathogenic immunological activity. Although there have been recent advancements in the understanding of the pathogenesis of AIBDs, glucocorticosteroids and/or adjuvant immunosuppressive drugs are still needed to control disease activity. However, the long-term use of systemic immunosuppression is associated with major adverse events, including death. Based on the growing understanding of AIBD pathogenesis, novel treatment targets have emerged, some of which are currently being evaluated in clinical trials. Within this article, we review the current clinical trials involving pemphigus and pemphigoid and discuss the rationale that lead to these trials. Overall, we aim to foster insights into translational research in AIBDs to improve patient care.
The association of different therapeutic approaches with long-term cardiovascular and metabolic outcomes in patients with pemphigus remains to be precisely evaluated.
To assess the risk of long-term ...cardiovascular and metabolic outcomes and all-cause mortality in patients with pemphigus managed by rituximab compared with those receiving treatment with first-line corticosteroid-sparing agents (azathioprine and mycophenolate mofetil MMF).
A global population-based retrospective cohort study compared 961 patients with pemphigus that was managed with rituximab with those treated with azathioprine or MMF (n = 961) regarding the risk of several cardiovascular and metabolic outcomes. Propensity score matching was performed to optimize comparability. Patients were enrolled from the Global Collaborative Network of TriNetX platform.
Risk of myocardial infarction, stroke, peripheral vascular disease, pulmonary embolism, hypertension, hyperlipidemia, type 2 diabetes, obesity, osteoporosis, and avascular bone necrosis.
Of 1602 participants, 855 (53.4%) were women and 747 (46.6%) were men; the mean (SD) age was 54.8 (16.6) years for those treated with rituximab and 54.4 (18.2) years for those treated with azathioprine or MMF. Compared with those treated by azathioprine/MMF, patients treated with rituximab experienced a lower risk of myocardial infarction (relative risk RR, 0.45; 95% CI, 0.24-0.86; P = .01), stroke (RR, 0.42; 95% CI, 0.26-0.69; P < .001), peripheral vascular disease (RR, 0.47; 95% CI, 0.28-0.79; P = .003), hypertension (RR, 0.48; 95% CI, 0.38-0.63; P < .001), hyperlipidemia (RR, 0.45; 95% CI, 0.32-0.64; P < .001), type 2 diabetes (RR, 0.63; 95% CI, 0.51-0.77; P < .001), obesity (RR, 0.49; 95% CI, 0.34-0.72; P < .001), and osteoporosis (RR, 0.46; 95% CI, 0.30-0.71; P < .001). The all-cause mortality was comparable between patients in both groups (hazard ratio, 0.94; 95% CI, 0.62-1.43; log-rank P = .77).
The results of this cohort study suggest that rituximab was associated with protection against long-term cardiovascular and metabolic outcomes compared with conventional immunosuppressants. This agent might be particularly preferred in individuals with preexisting cardiovascular and metabolic risk factors.
Risk of inflammatory bowel disease under isotretinoin is a scope of a long-standing controversy. The burden of isotretinoin-related irritable bowel syndrome has not been investigated.
To evaluate the ...risk of Crohn’s disease, ulcerative colitis (UC), and irritable bowel syndrome in patients with acne starting isotretinoin vs oral antibiotics treatment.
A global population-based retrospective cohort study assigned 2 groups of patients with acne initiating isotretinoin (n = 77,005) and oral antibiotics (n = 77,005). Comprehensive propensity-score matching was conducted.
The lifetime risk of Crohn’s disease (hazard ratio HR, 1.05; 95% CI, 0.89-1.24; P = .583) and UC (HR, 1.13; 95% CI, 0.95-1.34; P = .162) was comparable between study groups, whereas the lifetime risk of irritable bowel syndrome was lower in isotretinoin-prescribed patients (HR, 0.82; 95% CI, 0.76-0.89; P < .001). In time-stratified analysis, isotretinoin-related risk of UC was significantly increased during the first 6 months following drug initiation (HR, 1.93; 95% CI, 1.29-2.88; P = .001), but decreased afterward to level the risk of the comparator group. The absolute risk difference within the first 6 months was clinically marginal (5.0 additional UC cases/10,000 patients starting isotretinoin; 95% CI, 2.5-7.7).
Retrospective data collection.
Isotretinoin does not confer an elevated risk of Crohn’s disease, whilst it might be associated with a slight and transient increase in UC risk.
Psoriasis is a chronic immune-mediated inflammatory disease. Beyond the physical dimensions, the disease has an extensive emotional and psychosocial effect on patients, influencing their quality of ...life, social life and interpersonal relationships. Thus patient-reported outcomes are a crucial instrument for the evaluation of disease burden. Navigating life in times of the COVID-19 pandemic is challenging, especially for persons suffering from chronic diseases. We here analyzed the impact of lockdown restrictions on psoriasis patients. To compare the Dermatology Life Quality Index (DLQI) before and during the COVID-19 pandemic of patients with psoriasis. Retrospective longitudinal analysis in adult patients with moderate to severe psoriasis undergoing biologic treatment between January 2020 and January 2021. DLQI, patient demographics, Psoriasis Area and Severity Index (PASI), and recent biologic treatment were recorded. 103 patients were identified, of whom 19 had additional psoriatic arthritis. Female (n = 29) and male (n = 74) patients were distributed 1 to 3. Median age of patients was 54 years (range 18-85). All patients received biologic systemic treatment: anti-IL-23 (n = 39), anti-IL-17A (n = 30), anti-IL-12/23 (n = 25), or anti-TNFalpha (n = 9). Comparing DLQI scores before the COVID-19 pandemic and under lockdown restriction showed improved DLQI scores over time. Further analysis displayed that patients mostly ticked "not relevant" on social activities during lockdown. Thus, the DLQI scores may be artificial improved and may not really reflect the actual disease burden. Psoriasis patients showed a contrary improvement of life quality despite harsh COVID-19 lockdown suggesting that DLQI should be modified when social life is restricted.
Pemphigoid diseases (PDs) are chronic and life‐threatening autoimmune diseases of the skin and mucous membranes. PDs are characterized and caused by autoantibodies targeting components of the ...basement membrane. In the PD epidermolysis bullosa acquisita (EBA), the target autoantigen is type VII collagen. Current treatment options of PD, especially EBA, are limited and are mostly based on systemic immunosuppression. Animal models of PD have greatly advanced our understanding of PD pathogenesis. This has led to the identification of several novel therapeutic targets, including signalling molecules. Herein, the contribution of signalling molecules in the pathogenesis of the PD EBA and the effects of pharmacological targeting of these pathways are reviewed in detail. The p38 MAPK, ERK1/2, AKT, PI3Kβ, Hsp90, RORα, PDE4, Src kinases and CARD9 have been demonstrated to be critically involved in EBA pathogenesis. With the advent of new signal transduction inhibitors, we expect that the so far poor prognosis of EBA and other PD will improve significantly in the future.