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Background: The risk of life-threatening major cardiovascular outcomes among patients with bullous pemphigoid (BP) is inconsistent in the current literature., Objective: To evaluate the risk and prognostic outcomes of myocardial infarction (MI), cerebrovascular accident (CVA), peripheral vascular disease (PVD) and pulmonary embolism (PE) in patients with BP. We additionally aimed to explore the influence of different therapeutic approaches on the risk of these outcomes., Methods: A population-based retrospective cohort study was conducted to compare BP patients (n = 3924) with age-, gender- and ethnicity-matched control subjects (n = 19,280) with regard to incident cases of MI, CVA, PVD and PE. Adjusted hazard ratio (HR) and 95% confidence intervals (CI) were estimated by multivariate Cox regression analysis. Data were retrieved from Clalit Health Services' computerized database., Results: Relative to their matched controls, patients with BP were at an elevated risk of MI (fully-adjusted HR: 1.44; 95% CI: 1.14-1.81; p = 0.002), CVA (fully-adjusted HR: 1.24; 95% CI: 1.06-1.45; p = 0.007), PVD (fully-adjusted HR: 1.60; 95% CI: 1.27-2.03; p = 0.003) and PE (fully-adjusted HR: 1.72; 95% CI: 1.28-2.32; p < 0.008). Patients with BP experienced heightened risk of all-cause mortality in the presence of comorbid MI (fully-adjusted HR: 1.61; 95% CI: 1.44-1.81; p < 0.001), CVA (fully-adjusted HR: 1.70; 95% CI: 1.52-1.89; p < 0.001), PVD (fully-adjusted HR: 1.38; 95% CI: 1.20-1.58; p < 0.001) and PE (fully-adjusted HR: 1.44; 95% CI: 1.10-1.88; p = 0.007). The therapeutic approach utilized to manage BP did not significantly influence the risk of cardiovascular outcomes., Conclusions: BP is associated with an elevated risk of MI, CVA, PVD, PE and cardiovascular mortality. Primary, secondary and tertiary cardiovascular prevention measures should be implemented among patients with BP., (© 2024 The Author(s). Australasian Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Australasian College of Dermatologists.)

Background: According to current guidelines, systemic or topical corticosteroids are both recommended as first-line treatments for bullous pemphigoid (BP). There is evidence to suggest that topical application may be associated with a lower risk of mortality. However, there is a lack of comprehensive large-scale data comparing mortality rates, as well as the risk of major adverse cardiac events (MACE), infections and relapse, between systemic and topical corticosteroid treatments., Objectives: To evaluate the risk of death, MACE, infections and relapse in patients with BP treated with systemic or topical corticosteroids., Methods: A population-based retrospective cohort study was performed using the TriNetX US Collaborative Network. As a measure against bias, propensity score matching for age, sex, 10 diseases and 6 medications was done, and 3 sensitivity analyses were conducted., Results: All-time risk of death was increased in US patients with BP exposed to any dose of systemic corticosteroids (n = 2917) vs. patients treated with topical clobetasol propionate [n = 2932; hazard ratio (HR) 1.43, 95% confidence interval (CI) 1.28-1.58 (P < 0.001)]. This was consistent in time-stratified analysis (1- and 3-year mortality rates) and in analysis contrasting prednisone (equivalent) doses of 1-10 mg (low) or 30-100 mg (medium-high) systemic corticosteroid to topical treatment. The increased risk of death in US patients with BP exposed to any dose of systemic corticosteroids vs. topical treatment was accompanied by increased risks for MACE (HR 1.33, 95% CI 1.08-1.64; P = 0.008) and infections (HR 1.33, 95% CI 1.15-1.54; P < 0.001). The risk of continued disease or relapse was decreased in patients treated with systemic vs. topical corticosteroids (HR 0.85, 95% CI 0.77-0.94; P = 0.002). Results regarding mortality and continued disease or relapse persisted in three sensitivity analyses. Potential limitations included the retrospective data collection, bias for treatment selection and miscoding., Conclusions: Pending validation in prospective studies, where feasible - and despite the heightened risk of relapse - topical corticosteroid treatment may be advantageous over systemic corticosteroid treatment owing to its significantly lower risk of death., Competing Interests: Conflict of interest The authors declare no conflicts of interest., (© The Author(s) 2024. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering disorder in adults. Most individuals with BP are over the age of 60. Its worldwide incidence has been increasing owing to population aging. Observational studies published over the last 2 decades highlight the non-negligible, albeit variable overall mortality of BP patients, with reported 12-month mortality rates of 10.8% to 40.8%, and 24-month mortality rates of 20.1% to 51.0%. Data in the Canadian population are lacking., Objectives: We aimed to estimate the 12- and 24-month overall mortality rate of Canadian patients diagnosed with BP, and to identify independent risk factors adversely impacting overall survival., Methods: A retrospective cohort study of 166 patients with a diagnosis of BP between 2010 and 2020 was carried out at Centre hospitalier de l'Université de Montréal (CHUM), a tertiary referral center in Montréal, Québec, Canada. Cumulative mortality was calculated using the Kaplan-Meier estimator, and independent prognostic factors were identified using a Cox proportional hazards regression model., Results: Eighty-five patients (51.2%) in our study were female. The median age was 79.1 years old, and 80 patients (48.2%) were 80 years old or older. Mortality at 12 and 24 months in our study cohort was 16.2% (CI95% = 10.5 - 21.8) and 27.6% (CI95% = 20.5 - 34.7), respectively. In a multivariate analysis, patients who were male, 80 years old or older, and/or had a diagnosis of a major neurocognitive disorder had a poorer overall survival., Conclusions: The all-cause mortality of patients with BP in our study population compared favorably with international data reported in the literature.

Background: Eosinophils play an important role in bullous pemphigoid (BP) pathogenesis. Although tissue infiltration with eosinophils has been known for a long time, there is a lack of knowledge about the relationship between tissue eosinophil levels and disease severity and clinical characteristics of the patients., Methods: Fifty-nine patients diagnosed with BP between January 2008 and December 2018 were reviewed. Haematoxylin-Eosin (H&E)-stained preparations were re-evaluated in terms of tissue eosinophil levels. For disease severity, Bullous Pemphigoid Disease Area Index (BPDAI) was used. The relationship between tissue eosinophil levels and disease severity and clinical features were evaluated., Results: Erosion/blister and urticaria/erythema BPDAI scores were higher in the group with high tissue eosinophil level than the group with low tissue eosinophil level. Tissue and peripheral blood eosinophil count were correlated with total urticaria/erythema BPDAI scores. There was no correlation between blood and tissue eosinophil count. The mortality rate was 64.7% vs 44.0% in the high vs low tissue eosinophil groups. Tissue eosinophil levels were high in patients with BP accompanying neurological disease., Conclusions: Tissue eosinophil count and peripheral blood eosinophil count were correlated with disease severity in BP. Tissue eosinophil levels were also high in patients with BP accompanying neurological disease., (© 2021 The Australasian College of Dermatologists.)

Importance: The 1-year standardized mortality ratio (SMR) of bullous pemphigoid (BP) has been reported as 2.15 to 7.56 and lower in the US than in Europe., Objective: To estimate the worldwide 1-year SMR of BP., Data Sources: PubMed, Embase, Cochrane Library, Google Scholar, Lissa, and gray literature (eg, medRxiv) were screened for studies of BP published from inception to June 10, 2020, with review of reference lists., Study Selection: Retrospective and prospective studies reporting 1-year all-cause mortality rate in patients with BP and providing age statistics (eg, mean [SD])., Data Extraction and Synthesis: Two reviewers independently extracted the data. The 1-year SMR was computed in studies reporting 1-year mortality by combining information on age obtained from studies with aggregate data and individual data. Risk of representativity, misclassification, and attrition bias were assessed by a custom tool., Main Outcomes and Measures: The primary end point was the worldwide 1-year SMR. Secondary analysis included comparison of 1-year SMRs between continents in a meta-regression., Results: Three studies were performed in the US (n = 260), 1 in South America (n = 45), 16 in Asia (n = 1903), and 36 in Europe (n = 10 132) for a total of 56 unique studies and 12 340 unique patients included in the meta-analysis (mean [SD] age, 77.3 [12.7] years; 55.9% women). The mean (SD) patient age in the United States was 75.6 (13.7) years; in Asia, 73.8 (13.6) years; and in Europe, 78.1 (12.3) years. The worldwide 1-year SMR was estimated at 2.93 (95% CI, 2.59-3.28; I2 = 85.6%) for all 56 studies. The 1-year SMR in the US was 2.40 (95% CI, 0.89-3.90; I2 = 86.3%) for 3 studies; in Asia, 3.53 (95% CI, 2.85-4.20; I2 = 86.3%) for 16 studies; and in Europe, 2.77 (95% CI, 2.35-3.19; I2 = 86.3%) for 36 studies. After adjustment on the expected 1-year mortality rate, the European 1-year SMR did not differ significantly from the 1-year SMR in the United States (-0.48 vs Europe; 95% CI, -2.09 to 1.14; P = .56) and Asia (0.51 vs Europe; 95% CI, -0.56 to 1.58; P = .35). Risk of attrition bias was high (>10% censorship) in 16 studies (28.6%), low in 16 (28.6%), and unclear in 24 (42.9%). Only 4 studies (7.1%) had a sampling method guaranteeing the representativity of BP cases in a population., Conclusions and Relevance: Although heterogeneity was high and overall quality of follow-up was poor, this meta-analysis confirms the high mortality rate among patients with BP.

Bullous pemphigoid is an uncommon, autoimmune, blistering disease. Clinical features, associated conditions, and outcomes differ according to country. We aimed to determine the mortality rate and clinical characteristics of Thai patients and to evaluate the risk factors associated with survival.A retrospective analysis was conducted on 119 patients, over a ten-year period, at Songklanagarind Hospital, the largest tertiary university hospital in Southern Thailand.The median age of onset was 82 years [interquartile range 72, 90], and 60 (50.4%) patients were men. The underlying diseases were hypertension (53.8%), neurological disease (42.8%), and diabetes mellitus (31.9%). Fifty-eight patients (48.7%) experienced pruritus, and 61.3% of patients had mild cutaneous lesions (less than 10% of the body surface area) on the day of diagnosis. Nine percent of patients presented with mucosal involvement. Complete blood counts showed anemia (32.8%), neutrophilia (30.3%), and eosinophilia (42.9%). The 1-, 3- and 5-year overall mortality rates were 28.1% [95% confidence interval (CI), 7.8-36.6], 55.7% (95% CI, 44.4-64.7) and 71.9% (95% CI 59.9-80.2), respectively. On multivariate analysis, high neutrophil/lymphocyte ratio [odds ratio (OR) 5.55, P < .001] and anemia (OR 2.93, P = .025) were found to be independently associated with mortality rate, whereas disease remission (OR 0.25, P = .003) was demonstrated to be a good prognostic factor.This is the first study to analyze the mortality rate of Bullous pemphigoid in Thailand. Mortality was associated with high neutrophil/lymphocyte ratio and anemia.

There are differences concerning reported mortality rates and prognostic factors of bullous pemphigoid (BP) patients in different studies. Our objectives were to evaluate the mortality rates and prognostic factors among Israeli BP patients compared to matched control subjects. Three age- and sex-matched patients without BP (n = 261) who were treated in our clinic were selected and compared to BP patients (n = 87). Mean survival period of the BP group was 4.1 years (95% CI: 3.3-4.8 years) and 5.9 years among the non-BP group (95% CI: 5.6-6.3 years). The 1-year mortality rate was 24.1% for the BP group and 6.5% for the control group. In multivariate analysis, age above 80 was a significant risk factor for mortality [HR 3.22 (95% CI, 1.15-8.96), p = 0.03], while statins intake had a protective role [HR 0.36 (95% CI, 0.15-0.88), p = 0.03]. In univariant analysis, dementia [HR 2.44 (95% CI, 1.02-5.99), p = 0.04] was a risk factor. In conclusion, BP patients' mortality is correlated to increasing age at diagnosis, dementia, and statins use. Statins' protective role is newly discussed in the literature.

There is little consensus regarding mortality data in bullous pemphigoid (BP). The aim of this study was to evaluate mortality among a relatively large cohort of Israeli patients with BP and to perform a meta-analysis synthesizing existing data on 1-year mortality rates of patients with BP. This retrospective cohort study of 287 patients diagnosed with BP between 2000 and 2015 compared the mortality of patients with BP with age- and sex-matched control subjects in the general population. The results showed 1-, 5- and 10-year mortality rates of 26.9%, 56.9% and 69.5%, respectively, and a 3.4-fold higher risk of death. A systematic review and meta-analysis were then performed using a random effects model. Including the current study, 25 studies comprising 4,594 patients met the eligibility criteria. The pooled estimate of 1-year mortality rate was 23.5% (95% confidence interval 20.2-26.8; I2=81%; p < 0.001). The pooled 1-year mortality rate of European cohorts was prominently higher relative to the pooled rates of cohorts from the USA and Asia.

Background: Bullous pemphigoid (BP) is a subepidermal blistering disease characterized by autoantibodies against the two hemidesmosomal proteins, BP180 (type XVII collagen) and BP230. The multicentre prospective BLISTER (Bullous Pemphigoid Steroids and Tetracyclines) trial randomized 253 patients with BP to compare the benefits and harms between initial treatment with doxycycline or prednisolone., Objectives: To analyse distinct autoantibody profiles for the prediction of the disease course in a well-characterized cohort of BP sera., Methods: One hundred and forty-three patients of the BLISTER trial consented to participate in this serological study. Sera taken at baseline were analysed by (i) indirect immunofluorescence, (ii) anti-BP180 NC16A (16th noncollagenous domain) and anti-BP230 enzyme-linked immunosorbent assay and (iii) immunoblotting with various substrates. Results were then linked with clinical parameters including age, Karnofsky score, number of blisters, related adverse events and mortality., Results: Disease activity correlated with immunoglobulin (Ig)G anti-BP180 levels but not with levels of anti-BP230 IgG and anti-BP180 IgE. High levels of both anti-BP180 IgG and anti-BP230 IgG were associated with a low Karnofsky score. The presence of anti-BP230 IgG was more frequent in older patients. Those with higher total IgE serum levels suffered from fewer adverse events. Higher IgG anti-BP180 levels were associated with an increased 1-year mortality rate., Conclusions: Analysis of the autoantibody profile is not only of diagnostic relevance but may also be helpful in predicting the course of the disease., (© 2018 British Association of Dermatologists.)

There are inconsistent data on mortality rates in patients with bullous pemphigoid (BP). Trends in mortality in BP throughout the years are yet to be established. The aim of the present study was to study the mortality in BP patients relative to the general population and to estimate trends in standardized mortality over the past 30 years. We performed a systematic review and meta-analysis of observational studies in Medline, Embase and Scopus (1823-2017). Reference lists of included studies were also searched for eligible studies. Quality of evidence was assessed using the Newcastle-Ottawa Scale (NOS). A meta-analysis was performed using random-effects models to estimate pooled standardized mortality ratios (SMR) with 95% confidence intervals (CI). Meta-regression models were used to investigate the secular trends in SMR. Ten studies were included covering the period 1960-2015 (1736 patients, 746 deaths). Pooled all-cause SMR was 3.6 (95% CI, 2.6-5.0). There was no trend in all-cause SMR across the last three decades (regression coefficient 0.02 [change in logSMR/year]; 95% CI, 0.04-0.08; P = 0.545). In conclusion, there is a 3.6-fold increased mortality among patients with BP as compared with the age-matched general population. The excess mortality in BP has not changed significantly over the past 30 years., (© 2018 Japanese Dermatological Association.)

Bullous pemphigoid (BP) is a chronic autoimmune blistering disorder that has a predilection for the elderly. It is characterized by a poor prognosis due to its high mortality rate and the tendency to relapse. The relapse rate of BP ranges from 27.87% to 53% after disease remission, while the majority of relapses occur early (within 6 months) during remission. Clinical interventions aimed to prevent early relapses could reduce potential complications from first-line treatment and make follow-up care easier for clinicians in practice, thus improving the prognosis of BP and the quality of patients' lives. In this article, we attempt to review previous studies from the medical literature concerning relapse and risk factors related to relapse in BP patients. Treatments found to be able to reduce or increase relapse rate were also discussed. We propose that clinicians be aware of these risk factors and manage proper treatment accordingly. Key Messages   • The relapse rate of bullous pemphigoid ranges from 27.87% to 53% after disease remission, while the majority of relapses occur early (within 6 months) during remission.   • Ageing, disease severity, neurological disorder, serum ECP, BAFF, IL-17, IL-23, CXCL10 and anti-BP180 antibody levels have been shown to be related to relapse, while confirmation studies are needed before using them for guidance for relapse prevention.   • Longer usage of corticosteroid with a low dose, combination of immunosuppressants and intravenous immunoglobulin are helpful in lowering relapse rate.

Bullous pemphigoid (BP) is associated with higher mortality and coexisting comorbidities, some of them affecting poor prognosis. The aim of the study was to identify prognostic factors causing greater mortality both in the 1st and 3rd year of follow-up and to determine the 1-, 2-, 3-year mortality rates, standardized mortality ratio (SMR) in Polish BP patients. All patients with BP (a cohort of 205 patients, mean age 76.2 years) diagnosed between 5 January 2000 and 10 December 2013 in a referral unit for autoimmune bullous diseases at the university hospital in Poland were included retrospectively. Mortality data were obtained from the Centre for Document Personalization at the Minister of Interior and Administration. Our original observation was that prednisone in moderate dose (0.5 mg kg -1 ) in monotherapy was an independent risk factor of fatal prognosis in the 1st year of follow-up, assessed using multivariate analysis. We confirmed the strong correlation between neurological diseases and greater mortality. Both in the 1st and 3rd year of follow-up, dementia and Parkinson disease resulted in increased mortality. We also found that arrhythmias significantly increased mortality in the 1st and 3rd year of follow-up. The prognostic factors in BP changed over time of follow-up. In the 3rd year of observation, the age above 77, longer hospitalization and BP severity were associated with greater mortality. We observed poorer prognosis in BP patients than age-matched general Polish population. The 1-, 2-, 3-year mortality rates were 22.4, 31.2, 39.5% and SMR was 3.8 (95% CI 3.4-3.7).

Studies suggest an association between neurological disorders and bullous pemphigoid. The goal of this systematic review was to characterize the occurrence of neurological disorders in patients with bullous pemphigoid. We performed a systematic review of the current English literature from 1984 to June 1 st , 2015 for documented cases of coexistent BP and neurological disorders. The literature search resulted in 53 articles meeting the inclusion criteria. Patients with bullous pemphigoid had an increased risk of stroke (OR: 4.43 [95% CI: 2.69-7.28]; p<0.001), dementia (OR: 5.48 [95% CI: 3.26-9.23]; p<0.001), Parkinson's (OR: 3.06 [95% CI: 1.97-4.77]; p< 0.001), and epilepsy/seizures (OR: 22.88 [95% CI: 2.64-198.21]; p = 0.0045). Neurological disorders preceded bullous pemphigoid in the majority of cases with a mean time interval of 6.7 years. The one-year mortality was increased in bullous pemphigoid patients who had concomitant stroke (OR: 2.87 [95% CI: 1.67-4.96]; p<0.001). Bullous pemphigoid patients have an increased association with neurological disorders which may increase mortality.

Bullous pemphigoid (BP) is a chronic debilitating autoimmune blistering disease that frequently occurs in the elderly population. Previous studies have suggested a high morbidity and mortality associated with BP. However, relatively few studies have investigated prognostic factors of BP mortality, and they showed considerably various results. This meta-analysis aimed to quantitatively assess the association between several potential prognostic factors and risk of mortality in bullous pemphigoid. A comprehensive search was performed using Pubmed, Embase, and Cochrane Library. Cohort studies that assessed prognostic factors of BP mortality were included. Random-effects model was utilized to calculate the pooled hazard ratio (HR). Publication bias was evaluated qualitatively by constructing a funnel plot and quantitatively by conducting Egger's test. 14 studies were included comprising 2499 patients. Combined HRs suggested that advanced age (HR 1.63, 95% CI 1.34-1.97), presence of circulating antibodies (HR 1.77, 95% CI 1.20-2.62), concomitant dementia (HR 2.01, 95% CI 1.22-3.33), and concomitant stroke (HR 1.86, 95% CI 1.29-2.67) have an unfavorable impact on patient survival. Gender, disease extent, mucosal involvement, and indirect immunofluorescence result were not shown to be linked to mortality by our analysis. This study indicated that BP patients with older age, circulating antibodies, dementia, and stroke are at greater risk of mortality. Clinicians should be aware of this association and utilize this information in patient education and treatment process.

Background: Bullous pemphigoid (BP) is a disease of the elderly and may be associated with neurological and cardiovascular diseases and diabetes. Mortality rates strongly exceed those of the background population., Objectives: To investigate the frequency of comorbidities and their temporal relation to BP., Methods: A register-based matched-cohort study on all Danish patients with a hospital-based diagnosis of BP (n = 3281). The main outcomes were multiple sclerosis (MS), Parkinson disease (PD), Alzheimer disease (AD), stroke, diabetes types 1 and 2, malignancies, ischaemic heart disease (IHD), hypertension and eventually death., Results: At baseline, patients with BP had increased prevalences of MS [odds ratio (OR) 9·7, 95% confidence interval (CI) 6·0-15·6], PD (OR 4·2, 95% CI 3·1-5·8), AD (OR 2·6, 95% CI 1·8-3·5) and stroke (OR 2·7, 95% CI 2·4-2·9). Furthermore, malignancies, cardiovascular disease and diabetes were over-represented among patients with BP: type 1 diabetes (OR 3·1, 95% CI 2·5-3·8), type 2 diabetes (OR 2·3, 95% CI 2·0-2·6), malignancies (OR 1·3, 95% CI 1·1-1·4), IHD (OR 1·7, 95% CI 1·5-1·9) and hypertension (OR 2·0, 95% CI 1·8-2·2). During follow-up, the risk of MS was significantly higher among patients with BP [hazard ratio (HR) 9·4, 95% CI 4·9-18·0], even if events during the first year after diagnosis of BP were excluded (HR 5·1, 95% CI 2·3-11·3). Patients with BP had an average increased mortality rate of 2·04 (95% CI 1·96-2·13)., Conclusions: We discovered a significantly increased frequency of MS among patients with BP. At the time of diagnosis, patients with BP had an excessive number of comorbidities and an increased mortality rate over the following years., (© 2017 British Association of Dermatologists.)

Background: We hypothesize that autoantibodies are induced after the blood-brain barrier is damaged by stroke and the risk of bullous pemphigoid (BP) is increased after stroke. We assess the risk of BP after first-ever stroke in a nationwide population-based cohort of first-ever stroke patients., Methods: We extracted data from the Longitudinal Health Insurance Database 2005 and identified patients with first-ever stroke as well as control patients matched for age, gender, and year of enrollment. The risk of BP in first-ever stroke patients in comparison with that in control patients was analyzed using Cox regression., Results: Of 12,607 patients with first-ever stroke, 38 (0.3%) patients developed BP in a median of 3.5 years. In the control patients, 8 persons (0.06%) had BP in a median of 3.7 years. The crude hazard ratio (HR) of BP in first-ever stroke patients was 4.83 (95% CI 2.25-10.34, p < 0.001) compared to the control group. The adjusted HR was 4.20 (95% CI 1.94-9.08, p < 0.001) after adjustments for age, gender, hypertension, diabetes, dementia, epilepsy, Parkinson disease, furosemide, and neuroleptics for stroke patients., Conclusions: The risk of BP is increased in first-ever stroke patients in a nationwide population-based cohort and this association is independent of well-known confounders of BP., (© 2017 S. Karger AG, Basel.)

Background: Bullous pemphigoid (BP) is associated with significant disability and comorbid health disorders that may lead to or result from hospitalization. However, little is known about the inpatient burden and comorbidities of BP., Objectives: To obtain data on the inpatient burden and comorbidities of BP in the U.S.A., Methods: We analysed data from the 2002 to 2012 National Inpatient Sample, including a representative 20% sample of all hospitalizations in the U.S.A. (72 108 077 adults)., Results: The prevalence of hospitalization for BP increased from 25·84 to 32·60 cases per million inpatients from 2002 to 2012. In multivariate logistic regression models with stepwise selection, increasing age, nonwhite ethnicity, higher median household income, being insured with Medicare or Medicaid, and increasing number of chronic conditions were all associated with hospitalization for BP (P < 0·05 for all). The top three primary discharge diagnoses for patients with a secondary diagnosis of BP were septicaemia (prevalence 5·51%, 95% confidence interval 5·03-5·99), pneumonia (4·60%, 4·19-5·01) and urinary tract infection (3·52%, 3·15-3·89). Patients with BP also had numerous autoimmune, infectious, cardiovascular and other comorbidities. Interestingly, BP was associated with multiple neuropsychiatric disorders, including demyelinating disorders, dementias (presenile, senile, vascular and other), paralysis, neuropathy (diabetic, other polyneuropathy), Parkinson disease, epilepsy, psychoses and depression. The mean annual age- and sex-adjusted in-hospital mortality rate was significantly higher in patients with a secondary diagnosis of BP compared with no BP (2·9%, range 2·8-3·9% vs. 2·1%, range 1·9-2·2%). Significant predictors of mortality in patients with BP included increasing age, nonwhite ethnicity and insurance with Medicaid or other payment status (P < 0·05 for all)., Conclusions: Hospitalization for BP increased significantly between 2002 and 2012. Moreover, there were significant ethnic and healthcare disparities with respect to hospitalization and inpatient mortality from BP., (© 2016 British Association of Dermatologists.)

Bullous pemphigoid (BP) is an autoimmune skin disease of elderly people, which is associated with increased mortality. The aim of this study was to investigate the standardized mortality ratio (SMR) for BP in Finland, and concomitant comorbidities and medications. This was a retrospective database study of all cases of BP diagnosed at the Department of Dermatology, Oulu University Hospital, Finland, between 1985 and 2012. A total of 198 immunologically confirmed cases of BP were found. One-year mortality was 16.7%, and SMR 7.56 (95% confidence interval (CI) 4.98-10.14). The most common comorbidities were cardiovascular diseases (76.3%) and neurodegenerative diseases (40.9%). Malignancies (8.6%) were associated with increased mortality (hazard ratio = 2.4, 95% CI 1.1-5.5, p = 0.047). A novel finding was that polypharmacy was very common in patients with BP, and the higher the number of drugs, the greater the mortality. In conclusion, the mortality for BP in Finland is 7.6-fold that of a reference population, and malignancies and polypharmacy are associated with increased mortality.

Background: Bullous pemphigoid is an autoimmune blistering skin disease that predominantly affects the elderly. Conventional therapy using high-dose systemic corticosteroids and immunosuppressive agents can be ineffective in some patients and produce adverse events and relapses. Hence, alternate therapies are required., Objective: The clinical outcomes of patients with extensive, recalcitrant bullous pemphigoid treated with a combination therapy of rituximab (RTX) and intravenous immunoglobulin were evaluated., Methods: In this retrospective study, 12 patients (mean age of 68.25 years) unresponsive to previous conventional immunosuppressive therapy, intravenous immunoglobulin, and/or RTX were treated with this combination therapy., Results: Complete clinical resolution occurred in a mean of 4.6 months and previous systemic therapy was discontinued in 6.2 months. Two patients had a recurrence posttherapy and responded to additional RTX infusions. The remaining 10 patients had no recurrences. Patients were followed up for a mean of 73.8 months after discontinuation of all systemic therapy. All have remained in remission without adverse events for 6 years., Limitations: This was a retrospective study with a small sample size., Conclusion: The combination of RTX and intravenous immunoglobulin produced a sustained clinical remission without adverse events, infections, and hospitalizations. This specific combination protocol offers a promising therapy for patients with recalcitrant bullous pemphigoid., (Copyright © 2015 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Bullous pemphigoid (BP) mainly affects elderly patients. It is often associated with neurological disorders, which constitute a major risk factor of the disease. The aim of our study was to determine whether neurological disorders, particularly dementia, influence outcome and mortality in BP patients., Patients and Methods: We conducted a retrospective study of all patients with BP seen in our dermatology department consecutively between 1997 and 2011. Clinical, immunological and therapeutic data, number of relapses and survival status were compared according to the presence at diagnosis of neurological disorders, particularly dementia., Results: Among the 178 patients included, an associated neurological disease was present in 84 (47.2%) and dementia in 43 (24.2%) at the time of diagnosis of BP. Patients with associated dementia were older and had a lower Karnofsky index. Sixty-four patients (37.8%) had had at least one clinical relapse of BP, chiefly within the first 18 months after starting therapy. Coexistent neurological disease was not associated with BP relapse (P=0.55) contrary to an extensive BP phenotype at diagnosis (P=0.008). Coexistent neurological disease and/or dementia were associated with higher mortality (P=0.03 and P<0.001, respectively), but did not modify the type or the total duration of BP treatment., Conclusion: A coexistent neurological disease or dementia at the time of diagnosis of BP significantly increase the risk of mortality and shortens the duration of clinical follow-up of patients with BP, thus limiting the analysis of their influence on the outcome of BP itself., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Activation of blood coagulation has been demonstrated in bullous pemphigoid (BP), a rare autoimmune blistering disease, potentially leading to a prothrombotic state. In order to evaluate the incidence of venous thromboembolism (VTE) in BP, a cohort study was carried out on 432 BP patients (59% females; median age 76 years, interquartile range [IQR]: 68-82). At diagnosis, autoimmune bullous skin disorder intensity score (ABSIS) was calculated. VTE incidence was standardised with rates of the general population. Multivariable Cox proportional hazard model was used to estimate the hazard ratio of VTE according to ABSIS and concomitant risk factors. During a median follow-up of 4.2 years, 31 objectively-diagnosed VTE events were recorded. The incidence rate of VTE (per 1000 patient-years) was 17.2 overall (95% confidence interval [CI]: 11.1-23.2), 56.7 (95%CI: 33.0-80.4) during acute phase (22 VTE) and 6.3 (95%CI: 2.8-11.3) during remission (9 VTE). The standardised incidence ratio was 4.06 (95%CI: 2.73-5.65), higher during the acute phase (14.86, 95%CI: 9.20-21.88) than during remission (1.48, 0.66-2.63). The adjusted hazard ratio of VTE was 2.74 (95%CI: 1.07-7.04) for ABSIS > 48 vs ABSIS < 28, and 2.56 (95%CI: 1.00-6.70) in patients with ≥ 2 concomitant risk factors. In conclusion, BP patients have a 15-fold increased VTE risk during acute phase, proportional to disease severity and heightened by concomitant risk factors.

Bullous pemphigoid (BP) is an inflammatory autoimmune bullous disease involving cytokines and proteases in the process of blister formation. Recently, IL-17 and IL-23 were evidenced in lesional skin and serum of BP patients at time of diagnosis, but their involvement in disease outcome has still not been investigated yet. We then analysed IL-17 and IL-23 serum levels during the first months of follow-up upon treatment. Compared with age- and sex- matched controls, high levels of IL-23 were observed at baseline in BP patients serum (P < 0.01), while IL-17 levels was not. However, some BP patients expressed high IL-17 serum level, independently of disease severity. In these patients, those with ongoing remission reduced IL-17 concentration upon treatment (P < 0.001), whereas IL-17 level remained elevated in patients who relapsed. Meanwhile, IL-23 serum levels increased during the first month of treatment in BP patients who later relapsed (P < 0.01) and MMP-9 serum level was not controlled. Accordingly, we found that both IL-17 and IL-23 increased MMP-9 secretion from leukocytes in-vitro. Then, we showed that elevated IL-17/IL-23 serum concentrations helped to discriminate BP patients who later relapsed. Such uncontrolled inflammatory response raises the question whether these molecules could become biological target for BP patients resistant to steroid treatment.

Background: Bullous pemphigoid is an autoimmune subepidermal blistering skin and mucous membrane disease that usually occurs in elderly people. This study was done to determine clinical features, paraclinical findings, incidence of mucosal lesions, prognosis, course of the disease, and mortality rate of patients with bullous pemphigoid., Method: Retrospective descriptive study that reviews the existing files of 122 patients with bullous pemphigoid referred to dermatology clinics of Ghaem and Imam Reza hospitals in Mashhad, Iran, from 1995 to 2010., Result: The majority of patients were aged between 60 and 79 years, and 53% of them were women. The major prodromal lesions were erythematous and urticarial (36.8%) and eczematous lesions (32.7%); 84.3% of patients complained of pruritus, and mucosal lesions were observed in 31.1% of patients (all in the oral mucosa). The most frequent concurrent diseases with pemphigoid were hypertension and diabetes mellitus., Conclusion: Clinical features, paraclinical findings, incidence of mucosal lesions, prognosis, course of the disease, and mortality rate of bullous pemphigoid in the Khorasan region were approximately similar to results from other regions., (© 2015 The International Society of Dermatology.)

Background: Mortality rates in patients with bullous pemphigoid (BP) are higher than those in age-matched counterparts. However, the specific causes of death in BP subjects have not been evaluated systematically., Objectives: We sought to characterize the causes of death in patients with BP as recorded by death certificate and to compare these with death data for age- and location-matched control subjects., Methods: This was a retrospective cohort analysis conducted in a large tertiary referral center. Twenty-seven participants who had a confirmed antemortem diagnosis of BP, were residents of Olmsted County, Minnesota, and had died between January 1, 1999, and January 1, 2009, were included in the study. Underlying cause of death and multiple causes of death data for the study population were compared with data sourced from the Centers for Disease Control and Prevention (CDC) for a control group matched by age and geographic location of origin by International Classification of Diseases, 10th Revision (ICD-10) block, and specific ICD-10 codes., Results: Comparisons of specific ICD-10 codes revealed increased rates of sepsis (P = 0.031), dementia (P = 0.049), and major depressive disorder (P = 0.005) in the study group. The collective incidence of ICD-10 codes for infections indicated that infections were more frequent contributors to death in the study group (P = 0.035)., Conclusions: Clinicians should be mindful of contributors to death in patients with BP and might consider screening for mental health issues, educating patients on the early symptoms of sepsis, and minimizing risk factors for infection., (© 2013 The International Society of Dermatology.)

Background: The reported mortality rates and prognoses of bullous pemphigoid (BP) vary among different countries. However, the mortality rate of BP has not been investigated in Korea., Objective: We sought to evaluate the mortality rate of Korean patients with BP in comparison to that of an age-matched general population in Korea, and to identify prognostic factors affecting overall survival., Methods: We conducted a retrospective analysis of 168 patients diagnosed with BP between 1993 and 2013 at Gangnam Severance Hospital in Seoul, Korea., Results: The 1-, 2-, and 5-year mortality rates of BP patients were 19.46% (95% confidence interval [CI], 13.83-27.00), 29.13% (95% CI, 21.91-38.08), and 58.03% (95% CI, 47.04-69.45), respectively. The median age was 73 years (range, 6-99 years). The standardized mortality ratio ranged from 2.43 (95% CI, 0.12-11.96) to 9.56 (95% CI, 2.43-26.02), depending on the age group. In multivariate analysis, old age at the time of diagnosis, stroke, diabetes, and delayed diagnosis were associated with increased mortality., Limitations: This study was conducted as a retrospective analysis and was based at a single institution., Conclusion: The mortality rate of patients with BP is significantly higher than that for the general population., (Copyright © 2014 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Bullous pemphigoid (BP) is the most frequently encountered autoimmune blistering disorder, affecting mainly elderly population. The aim of the study was to assess the incidence of BP in Podlaskie Province (north-east Poland) in 1999-2012, socio-demographic characteristics and mortality of patients with BP., Methods: The number of newly diagnosed (in two regional hospitals) cases of BP per million inhabitants of the province in consecutive years was calculated and analysis of age, sex, and residency of patients performed., Results: A total of 122 cases of BP were diagnosed (in 52 males and 70 females). The average annual incidence was 7.38 ± 3.51 per million inhabitants: 5.70 ± 4.16 and 9.26 ± 4.40 in urban and rural areas, P < 0.05, respectively, and had an increasing trend over the period analyzed. Age of patients with BP was 74.18 ± 12.12. The incidence among men and women over 75 years of age was 86.67 ± 61.39 and 51.49 ± 41.44, P < 0.05, respectively. The 1-year mortality of patients was 32.35 and 18.42% (P < 0.05) in men and women, respectively. Overall mortality was 36.11%., Conclusions: The incidence of BP in Podlaskie Province is almost twice as high among residents of rural than urban areas, and in males than females in advanced age. The results suggest that the incidence of BP will be increasing in an aging society, as will its impact on the quality of life of the population., (© 2014 The International Society of Dermatology.)

Background: Bullous pemphigoid (BP) is an autoimmune blistering disease that is associated with increased mortality., Objective: We sought to determine the incidence and mortality of patients with BP., Methods: A total of 87 residents of Olmsted County, Minnesota, were identified who had their first lifetime diagnosis of BP from January 1960 through December 2009. Incidence and mortality were compared with age- and sex-matched control patients from the same geographic area., Results: The adjusted incidence of BP was 2.4 per 100,000 person-years (95% confidence interval, 1.9-2.9). Incidence of BP increased significantly with age (P < .001) and over time (P = .034). Trend tests indicate increased diagnosis of localized disease (P = .006) may be a contributing factor. Survival observed in the incident BP cohort was significantly poorer than expected (P < .001). Survival was not different among patients with multisite versus localized disease (P = .90)., Limitations: Retrospective study design and study population from a small geographic area are limitations., Conclusion: Incidence of BP in the United States is comparable with that found in Europe and Asia. The mortality of BP is lower in the United States than Europe, but higher than previous estimates., (Copyright © 2014 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Background: Bullous pemphigoid (BP) is the most common autoimmune-mediated subepidermal blistering skin disease and is associated with significant morbidity and mortality., Objectives: To determine the 3-year mortality rate, risk factors and causes of death in patients with BP in Singapore, compared with the general population., Methods: We conducted a retrospective cohort study of all newly diagnosed patients with BP seen at the National Skin Centre from 1 April 2004 to 31 December 2009. Demographic and clinical data on comorbidities and treatment were recorded. Mortality information was obtained from the National Registry of Diseases., Results: In total 359 patients were included in our study. The 1-, 2-, 3-year mortality rates were 26·7%, 38·4% and 45·7%, respectively. The 3-year standardized mortality risk for patients with BP was 2·74 (95% confidence interval 2·34-3·19) times higher than for the age- and sex-matched general population. Parkinson disease, heart failure and chronic renal disease were associated with increased mortality, while combination treatment with low-to-moderate-dose corticoste-roids and immunomodulatory agents such as doxycycline and/or nicotinamide was associated with lower mortality. Overall, infections were the most common cause of death (59·8%), with the main causes of death being pneumonia (42·7%), cardiovascular disease (14·6%) and stroke (11·6%)., Conclusions: This study confirms an increased 3-year mortality rate for patients with BP in Singapore. Risk factors for increased mortality include medical comorbidities, especially neurological, cardiac and renal diseases. Treatment with combination therapy, including the use of low-to-moderate-dose corticosteroid, appeared to decrease mortality risk in patients with BP., (© 2013 British Association of Dermatologists.)

Background: Prognosis of patients with bullous pemphigoid (BP) is controversial, with a 1-year mortality rate ranging from 6% to 48%., Objective: To determine the mortality rate of a large cohort of patients with BP and to identify prognostic factors associated with early mortality., Methods: Patients diagnosed with BP between January 1, 1990 and December 31, 2010 in a referral unit for blistering skin diseases at a university hospital in Spain were studied retrospectively. Outcome measures were mortality rate during the first year after diagnosis, standardized mortality rate and poor prognostic factors., Results: A total of 101 patients were included in the study. The mean patient age at diagnosis was 77.8 years, and 52 (51.5%) were men. Overall mortality during the first year was 12.9%. We found a standardized mortality ratio of 2.33 [CI95 = (1.25-4.03)]. Advanced age (patient group >80 years old) was the only risk factor for lethal outcome found, with a multivariate risk estimate of 1.09 [CI95 = (1.02-1.16)]. No significant association with mortality was detected for comorbidities, hospitalization history or treatment received for BP., Conclusions: We found an increased mortality of our BP patients compared with the general population. The mortality rate of BP patients was 2.3 times the expected rate. Observed mortality rate was lower than described in previous European studies. Advanced age impacts the prognosis of patients with BP. Specific treatment for BP appeared not to influence survival., (© 2012 The Authors. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology.)

Background: End-of-life care is becoming a daily concern of French dermatology departments. The aim of this single-centre retrospective study was to assess changes in mortality and end-of-life care in a university dermatology department over a 14-year period., Patients and Methods: The medical records of all patients dying in our dermatology department from 1996 to 2009 were studied retrospectively. A descriptive analysis was performed together with a comparison between the two periods demarcated by the institution of the French National Cancer Plan at the end of 2002., Results: The number of patients dying in the department rose by 33% from 108 for period 1 (1996-2002) to 144 for period 2 (2003-2009). The majority of patients presented metastatic melanoma, with doubling of numbers between the two periods. During period 2, 40 % of patients were managed in collaboration with the mobile palliative care team., Conclusion: This study shows an increase in the number of deaths in a French university hospital dermatology department over the 14-year period in question, ascribable mainly to an increase in the number of patients receiving end-of-life care for metastatic melanoma. Significant changes were noted in the management of these patients with an increase in palliative care procedures., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background: Bullous skin diseases are known to be associated with significant morbidity and mortality. There have been no studies on mortality from severe bullous skin diseases in Canada., Methods: We used mortality data from the Statistics Canada website from 2000 to 2007 for three major bullous skin diseases: bullous pemphigoid; pemphigus; and toxic epidermal necrolysis (TEN). Crude and age-standardized mortality rates were calculated and compared with the corresponding US mortality rates. Linear regression was used to assess time trend and effect of gender and age on mortality rates., Results: During the period of eight years, there were 115 deaths attributed to pemphigoid, 84 to pemphigus, and 44 to TEN. The crude annual mortality rate was the highest for pemphigoid (0.045 per 100,000), followed by pemphigus (0.033), and TEN (0.017). None of these conditions demonstrated significant time trends in mortality rates over the eight-year period, although a trend towards decreasing pemphigus mortality was observed (P = 0.07). No gender difference in mortality was observed, but advanced age was associated with mortality in all three conditions., Conclusion: Among bullous skin diseases, pemphigoid is the leading cause of mortality in Canada. This is in contrast to the USA, where TEN is the leading cause of mortality from bullous skin diseases. It is not clear whether differences in healthcare systems explain these findings., (© 2012 The International Society of Dermatology.)

A major increase in the incidence of BP has been recently reported in the United Kingdom. In addition, there are some controversies about the over-mortality of BP patients. The primary objective was to reevaluate the incidence of BP in France as compared with that we estimated 15 years ago. The secondary objective was to assess mortality of BP patients. BP incidence was retrospectively estimated from all BP cases diagnosed between January 2000 and December 2005 in three French regions with a total population of 3.858 million inhabitants. BP mortality was assessed from a prospective cohort accrued during the same time period. A total of 502 incident BP patients (mean age: 82.6±8.8 years) were identified. Overall estimated incidence was 21.7 cases per million persons per year (95%CI:19.8-23.7 cases per million persons per year), which is about 3-fold higher than the incidence that we estimated 15 years ago. In the population aged 70 years or above, BP incidence was 162 cases per million per year (95%CI:147-177 cases per million per year). The overall 1-year survival rate was 62% (95% CI: 56-67%). The risk of death for BP patients was more than six times greater than that for the general population (SMR:6.60; 95%CI:5.47-7.90). The incidence of BP in France has increased 3-fold in the last 15 years. BP is associated with high mortality.

Simple descriptive population data are potentially helpful in understanding how bullous pemphigoid (BP) originates and evolves over time. Before embarking with etiological correlations, artifacts and biases should be ruled out. Ideally, epidemiological data should be complemented by immunological and genetic analyses aimed at providing better insight into the causation and prognosis of BP.

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease and is associated with an increased mortality. The end points of our study were to evaluate the mortality rate in a retrospective cohort of BP patients followed up to 5 years after the diagnosis and to determine prognostics factors., Methods: All new cases of BP diagnosed between 1990 and 2003 in the University Hospital of Geneva were retrospectively collected. 60 patients were included, 47 (88.6%) of whom were treated with a combination of corticosteroids and chlorambucil., Results: The 1-year, 2-year and 5-year probabilities of death were 26.7, 37.1 and 60.8%, respectively. Old age, dementia and use of chlorambucil at initial doses of 6 mg/day, but not at lower doses, were associated with poor prognosis in multivariate analysis., Conclusion: Our study confirms that BP is associated with a high mortality. The observed mortality rates are however higher than those of previous studies, which is probably related to the inclusion of more debilitated patients., (Copyright © 2012 S. Karger AG, Basel.)

Background: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and has been associated with a significant morbidity and an increased mortality rate., Objectives: Our aims were: (i) to evaluate the mortality rate of BP in Swiss patients during the first 3 years after the diagnosis of BP, as the primary endpoint; and (ii) to compare this mortality rate with that of the general Swiss population and determine prognostics factors, as secondary endpoints., Methods: All new cases of BP diagnosed in Switzerland between 1 January 2001 and 31 December 2002 were collected prospectively by means of a standardized data collection form. After collection of the data, the treating physicians were asked to specify the course and evolution of the disease. In total, 115 patients were included in our study., Results: The 1-, 2- and 3-year probability of death were 20·9%, 28·0% and 38·8%, respectively. The mortality rate of patients with BP was three times higher than that of age- and sex-matched members of the general Swiss population. Besides age, the presence of neurological diseases at diagnosis was associated with increased mortality in multivariate analysis., Conclusion: This is the first prospective study analysing the mortality rate of BP in an entire country. The calculated mortalities are in the lower range of those reported in previous European studies. However, in line with the latter, our findings confirm a high case-fatality rate for BP, with an increased 1-year mortality rate compared with the expected mortality rate for age- and sex-adjusted general population., (© 2011 The Authors. BJD © 2011 British Association of Dermatologists 2011.)

Background: Bullous pemphigoid is associated with poorly understood dramatically increased early mortality rates., Objectives: To assess the incidence of acute events predisposing to early mortality., Methods: Computerized medical records from the Health Improvement Network, a large population-based U.K. general practice database, were used to conduct a cohort analysis. Outcome measures were incidence rates of myocardial infarction, pulmonary embolism, pneumonia and sepsis compared with a matched control population., Results: People with bullous pemphigoid were three times as likely to develop pneumonia, adjusted rate ratio 2.94 [95% confidence interval (CI) 2.01-4.31] or pulmonary embolism, adjusted rate ratio 3.12 (95% CI 1.37-7.12) compared with matched controls. No statistically significant increase was seen for myocardial infarction, adjusted rate ratio 1.24 (95% CI 0.66-2.33), or sepsis, adjusted rate ratio 2.02 (95% CI 0.78-5.21)., Conclusions: The risk of pulmonary embolism and pneumonia is increased following a diagnosis of bullous pemphigoid. It may be possible to reduce associated mortality through considering prophylaxis with either antithromboembolic measures or antibiotic therapy and vaccination.

Background: Since high doses of superpotent topical corticosteroids (CS) have been shown to improve the survival of bullous pemphigoid (BP) patients and to be more effective than oral CS, few studies have described the long-term course of the disease of these patients. We report the course of BP in the Limoges region of France based on patients diagnosed successively between 2002 and 2004., Patients and Methods: This was a retrospective, single-centre study of 54 BP patients diagnosed with BP, conducted by the Department of Dermatology of the Limoges University Hospital over a 3-year period. We investigated epidemiological data, initial symptoms, type of treatment received and outcome at six and 12 months. The two primary end-points were disease control at month 12 and the combined outcome of deaths and relapse over the one-year follow-up period. We calculated the number of deaths, patients lost to follow-up, relapses, and remission at months 6 and 12., Results: Between January 2002 and December 2004, 54 new BP patients were diagnosed in the Limoges Department of Dermatology: 18 male and 36 female, median age 84 years. Forty patients were treated with superpotent topical CS, 14 required adjuvant immunosuppressant treatment (oral CS, methotrexate, dapsone, mycophenolate mofetil, leflunomide). Over the first 6 months, 28 patients could not be further investigated due to death (n = 20) or loss to follow-up (n = 8). In the 26 patients evaluable for treatment efficacy at month 6, control of the disease was achieved in 17 patients, and nine relapses were seen. The one-year Kaplan-Meier survival rate was 56%. Among the 23 patients evaluable at month 12, control of disease was achieved in 13 patients (complete remission without treatment, n = 2; remission with immunosuppressant treatment, n = 8). Therapeutic failure was concluded for 16 patients, eight of whom presented two relapses during the first year of follow-up., Conclusion: Despite recent therapeutic recommendations for BP, the prognosis of this disease remains severe. The risk of relapse and mortality rates observed in this study should prompt the development of new therapeutic strategies to supplant superpotent topical CS and reduce the morbidity and mortality associated with the disease.

Background: There are large discrepancies in reported mortality for bullous pemphigoid (BP)., Objective: We sought to determine the mortality of a large cohort of patients with BP and compare this with age-matched control subjects., Methods: Data were collected on 223 patients with a new diagnosis of BP between 1998 and 2003 through our cutaneous immunofluorescence laboratory databases. The mortality of patients with BP was compared with that of age-matched control subjects in the general US population., Results: The 1-, 2-, and 5-year mortality was 0.23 (95% confidence interval=0.18, 0.29), 0.37 (95% confidence interval=0.31, 0.44), and 0.50 (95% confidence interval=0.42, 0.57), respectively. However, relative to age-matched control subjects, no difference in expected mortality was detected., Limitations: This was a retrospective cohort analysis., Conclusions: Mortality of patients with BP is more likely related to advanced age and associated medical conditions than to disease-specific factors.

Objective: To determine the incidence of and mortality from bullous pemphigoid and pemphigus vulgaris in the United Kingdom., Design: Retrospective historical cohort study., Setting: Computerised medical records from the health improvement network, a large population based UK general practice database., Participants: Patients with pemphigus vulgaris and bullous pemphigoid diagnostic codes and age, sex, and practice matched controls., Main Outcome Measures: Incidence and mortality compared with the control population by calendar period, age group, sex, geographical region, and degree of social deprivation., Results: 869 people with bullous pemphigoid and 138 people with pemphigus vulgaris were identified. The median age at presentation for bullous pemphigoid was 80 (range 23-102) years, and 534 (61%) patients were female. The median age at presentation for pemphigus vulgaris was 71 (21-102) years, and 91 (66%) patients were female. Incidences of bullous pemphigoid and pemphigus vulgaris were 4.3 (95% confidence interval 4.0 to 4.6) and 0.7 (0.6 to 0.8) per 100 000 person years. The incidence of bullous pemphigoid increased over time; the average yearly increase was 17% (incidence rate ratio=1.2, 95% confidence interval 1.1 to 1.2). An average yearly increase in incidence of pemphigus vulgaris of 11% (incidence rate ratio=1.1, 1.0 to 1.2) occurred. The risk of death for patients with bullous pemphigoid was twice as great as for controls (adjusted hazard ratio=2.3, 95% confidence interval 2.0 to 2.7). For pemphigus vulgaris, the risk of death was three times greater than for controls (adjusted hazard ratio=3.3, 2.2 to 5.2)., Conclusions: Incidences of bullous pemphigoid and pemphigus vulgaris are increasing. The reasons for the changes in incidence are not clearly understood but have implications for identifying causative factors. Both disorders are associated with a high risk of death. Previous estimates may have underestimated the risk of death associated with these diseases.

The pemphigoid group of autoimmune blistering diseases includes distinct entities (bullous pemphigoid, mucous membrane pemphigoid, pemphigoid gestationis, linear IgA dermatosis and lichen planus pemphigoides) that are characterized by relatively consistent clinical, histologic and immunopathologic findings. Patients with these disorders have antibasement membrane autoantibodies that often display pathogenic (blister-forming) activity following passive transfer to experimental animals. Interestingly, such autoantibodies target important structural proteins that promote adhesion of epidermis to epidermal basement membrane in human skin. Autoimmune blistering diseases are characterized by substantial morbidity (for example pruritus, pain, disfigurement) and in some instances mortality. Treatment with systemic immunosuppressives has reduced morbidity and mortality in patients with these diseases.

Background: The clinicoepidemiological characteristics and course of bullous pemphigoid (BP) have not been described in populations from the Arabian Gulf. Hypothesis. Ethnic and regional variations can influence the clinical behaviour and course of autoimmune diseases., Methods: In this study, 43 patients with BP, registered in our autoimmune bullous diseases (ABD) clinic over a span of 14 years, were studied to determine the clinicoepidemiological features and course of the disease in our region., Results: BP was observed to be the second commonest ABD in our clinic (27%), with a minimum estimated incidence of 2.6 cases/million/year among the referral population. The largest proportion (93%) of the patients was of Arab ethnicity and the female to male ratio was 5.1 :1. Mean +/- SD age at diagnosis was 65.20 +/- 18.80 years. Most of the patients (96%) had moderate to severe disease, and mucosal involvement was seen in 37% of the patients. Systemic steroids (prednisolone 20-60 mg daily) alone or in combination with azathioprine, intravenous immunoglobulins, tetracyclines, mycophenolate mofetil or dapsone were used to treat theses cases. At the last follow-up, 32% of patients were in complete remission and off treatment. The first-year mortality was 30%. Old age and poor general medical condition were the significant risk factors (P < 0.05) contributing to the mortality., Conclusion: The study highlights the regional variations of BP and thus a need to uncover the ethnic, genetic and geographical influences, if any, responsible for these variations.