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Journal of General Internal Medicine May 2013
Topics: Aged, 80 and over; Humans; Leg Dermatoses; Male; Pemphigoid, Bullous
PubMed: 23229905
DOI: 10.1007/s11606-012-2250-y -
Indian Journal of Dermatology,... 2023Background Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary...
Background Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. Aims The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. Methods We conducted a retrospective cohort study on newly diagnosed bullous pemphigoid patients between July 2001 and November 2019 in a referral unit for autoimmune blistering skin diseases in Romania. Results One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). The median follow-up among survivors was 48 months (interquartile range: 11-150). Ninety (60.8%) patients died during the follow-up period; of them, 38 (42.2%) had active disease at the time of death. Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Limitations This study lacks a control cohort to validate the obtained results. It was conducted in a retrospective manner in a single centre. In addition, indirect immunofluorescence microscopy was not performed in all patients. Conclusion Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients.
Topics: Humans; Pemphigoid, Bullous; Retrospective Studies; Prognosis; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Autoimmune Diseases; Skin Diseases, Vesiculobullous; Glucocorticoids; Microscopy, Fluorescence
PubMed: 36331841
DOI: 10.25259/IJDVL_705_2021 -
Medicina (Kaunas, Lithuania) Apr 2023Bullous pemphigoid (BP), the most common subepidermal autoimmune skin blistering disease (AIBD) has an estimated annual incidence of 2.4 to 42.8 new cases per million... (Review)
Review
Bullous pemphigoid (BP), the most common subepidermal autoimmune skin blistering disease (AIBD) has an estimated annual incidence of 2.4 to 42.8 new cases per million in different populations, designating it an orphan disease. Characterized by disruption of the skin barrier combined with therapy-induced immunosuppression, BP could pose a risk for skin and soft tissue infections (SSTI). Necrotizing fasciitis (NF) is a rare necrotizing skin and soft tissue infection, with a prevalence of 0.40 cases per 100,000 to 15.5 cases per 100,000 population, often associated with immunosuppression. Low incidences of NF and BP classify them both as rare diseases, possibly contributing to the false inability of making a significant correlation between the two. Here, we present a systematic review of the existing literature related to the ways these two diseases correlate. This systematic review was conducted according to the PRISMA guidelines. The literature review was conducted using PubMed (MEDLINE), Google Scholar, and SCOPUS databases. The primary outcome was prevalence of NF in BP patients, while the secondary outcome was prevalence and mortality of SSTI in BP patients. Due to the scarcity of data, case reports were also included. A total of 13 studies were included, six case reports of BP complicated by NF with six retrospective studies and one randomized multicenter trial of SSTIs in BP patients. Loss of skin integrity, immunosuppressive therapy, and comorbidities commonly related to BP patients are risk factors for necrotizing fasciitis. Evidence of their significant correlation is emerging, and further studies are deemed necessary for the development of BP-specific diagnostic and treatment protocols.
Topics: Humans; Fasciitis, Necrotizing; Pemphigoid, Bullous; Retrospective Studies; Skin; Soft Tissue Infections; Risk Factors; Randomized Controlled Trials as Topic; Multicenter Studies as Topic
PubMed: 37109703
DOI: 10.3390/medicina59040745 -
Experimental Dermatology Dec 2017Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering skin disease and is characterized by the presence of autoantibodies directed against the... (Review)
Review
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering skin disease and is characterized by the presence of autoantibodies directed against the hemidesmosomal proteins BP180 and BP230 that can be detected in the skin and serum of BP patients. Histologically, the dermal infiltration of eosinophils is obvious. The objective of this review was to present evidence that eosinophils play a key role in the pathogenesis of BP. Eosinophils, together with cytokines and chemokines regulating their production, recruitment and activation, are abundantly present in lesional skin, in blisters and in peripheral blood of patients with BP. Recently, using a cryosection model, eosinophils were demonstrated to induce dermal-epidermal separation in the presence of BP antibodies. Thus, eosinophils and their products, as well as mediators regulating their function, present promising targets for the treatment of BP.
Topics: Animals; Eosinophils; Humans; Immunoglobulin E; Molecular Targeted Therapy; Pemphigoid, Bullous
PubMed: 28833620
DOI: 10.1111/exd.13416 -
The Cochrane Database of Systematic... Oct 2010Bullous pemphigoid (BP) is the most common autoimmune blistering disease in the West. Oral steroids are the standard treatment.This is an update of the review published... (Review)
Review
BACKGROUND
Bullous pemphigoid (BP) is the most common autoimmune blistering disease in the West. Oral steroids are the standard treatment.This is an update of the review published in 2005.
OBJECTIVES
To assess treatments for bullous pemphigoid.
SEARCH STRATEGY
In August 2010 we updated our searches of the Cochrane Skin Group Specialised Register, the Cochrane Central Register of Controlled Trials (Clinical Trials), MEDLINE, EMBASE, and the Ongoing Trials registers.
SELECTION CRITERIA
Randomised controlled trials of treatments for participants with immunofluorescence-confirmed bullous pemphigoid.
DATA COLLECTION AND ANALYSIS
At least two authors evaluated the studies for the inclusion criteria, and extracted data independently.
MAIN RESULTS
We included 10 randomised controlled trials (with a total of 1049 participants) of moderate to high risk of bias. All studies involved different comparisons, none had a placebo group. In 1 trial plasma exchange plus prednisone gave significantly better disease control at 1 month (0.3 mg/kg: RR 18.78, 95% CI 1.20 to 293.70) than prednisone alone (1.0 mg/kg: RR 1.79, 95% CI 1.11 to 2.90), while another trial showed no difference in disease control at 6 months.No differences in disease control were seen for different doses or formulations of prednisolone (one trial each), for azathioprine plus prednisone compared with prednisone alone (one trial), for prednisolone plus azathioprine compared with prednisolone plus plasma exchange (one trial), for prednisolone plus mycophenolate mofetil or plus azathioprine (one trial), for tetracycline plus nicotinamide compared with prednisolone (one trial). Chinese traditional medicine plus prednisone was not effective in one trial.There were no significant differences in healing in a comparison of a standard regimen of topical steroids (clobetasol) with a milder regimen (RR 1.00, 95% 0.97 to 1.03) in one trial. In another trial, clobetasol showed significantly more disease control than oral prednisolone in people with extensive and moderate disease (RR 1.09, 95% CI 1.02 to 1.17), with significantly reduced mortality and adverse events (RR 1.06, 95% CI 1.00 to 1.12).
AUTHORS' CONCLUSIONS
Very potent topical steroids are effective and safe treatments for BP, but their use in extensive disease may be limited by side-effects and practical factors. Milder regimens (using lower doses of steroids) are safe and effective in moderate BP. Starting doses of prednisolone greater than 0.75 mg/kg/day do not give additional benefit, lower doses may be adequate to control disease and reduce the incidence and severity of adverse reactions. The effectiveness of adding plasma exchange, azathioprine or mycophenolate mofetil to corticosteroids, and combination treatment with tetracycline and nicotinamide needs further investigation.
Topics: Azathioprine; Clobetasol; Combined Modality Therapy; Drug Therapy, Combination; Drugs, Chinese Herbal; Glucocorticoids; Humans; Immunosuppressive Agents; Niacinamide; Pemphigoid, Bullous; Plasma Exchange; Prednisolone; Prednisone; Randomized Controlled Trials as Topic; Tetracycline
PubMed: 20927731
DOI: 10.1002/14651858.CD002292.pub3 -
International Journal of Molecular... Nov 2022Dipeptidyl peptidase-4 inhibitor (DPP4i) is a widely used antidiabetic agent. Emerging cases of DPP4i-associated bullous pemphigoid (DBP), whose pathogenesis remains...
Dipeptidyl peptidase-4 inhibitor (DPP4i) is a widely used antidiabetic agent. Emerging cases of DPP4i-associated bullous pemphigoid (DBP), whose pathogenesis remains unclear, have been reported. Thus, a retrospective study was conducted from January 2016 to June 2021 to determine the clinical, laboratory, and histopathological features of DBP and idiopathic bullous pemphigoid (IBP). We set up in vitro experiments using vildagliptin-treated HaCaT keratinocytes to validate what we found by analyzing published RNA sequencing data about the genes related to the dermal-epidermal junction. We also observed IL-6 expression by HaCaT cells treated with vildagliptin. We enrolled 20 patients with DBP and 40 patients with IBP. The total Bullous Pemphigoid Disease Area Index (BPDAI) score was similar in both groups. However, the BPDAI score of erosions and blisters in DBP was significantly higher than that in IBP (24.6 vs. 16.68, = 0.0189), and the score for urticaria and erythema was lower in DBP (12 vs. 19.05, = 0.0183). The pathological features showed that the mean infiltrating eosinophil number per high-power field was significantly lower in DBP than in IBP (16.7 vs. 27.08, = 0.023). The expression of , , , , and decreased significantly in vildagliptin-treated human keratinocytes. On the other hand, IL-6, the hallmark cytokine of bullous pemphigoid (BP) severity, was found to be upregulated in HaCaT cells by vildagliptin. These experimental findings imply less of a requirement for eosinophil infiltration to drive the inflammatory cascades in DBP blistering. Both immunologic and non-immunologic pathways could be employed for the development of DBP. Our findings may help explain the higher incidence of non-inflammatory BP that was observed in DBP.
Topics: Humans; Dipeptidyl-Peptidase IV Inhibitors; Dipeptidyl-Peptidases and Tripeptidyl-Peptidases; Hypoglycemic Agents; Interleukin-6; Pemphigoid, Bullous; Retrospective Studies; Vildagliptin
PubMed: 36430582
DOI: 10.3390/ijms232214101 -
Dermatologic Therapy Jun 2022
Topics: Antibodies, Monoclonal, Humanized; Humans; Pemphigoid, Bullous
PubMed: 35320611
DOI: 10.1111/dth.15466 -
Acta Dermato-venereologica Jan 2021Bullous pemphigoid constitutes a rare dermatological immune-related adverse event of programmed cell death protein 1 (PD-1)/programmed cell death ligand 1 (PD-L1)... (Review)
Review
Bullous pemphigoid constitutes a rare dermatological immune-related adverse event of programmed cell death protein 1 (PD-1)/programmed cell death ligand 1 (PD-L1) inhibitors. Herein, we review all published cases of anti-PD-1/PD-L1 related bullous pemphigoid and discuss current knowledge on this condition. Clinical and diagnostic findings were found to resemble those of classic bullous pemphigoid. A delayed onset of bullous pemphigoid after commencement of immunotherapy as well as a frequent precendence of a refractory pruritic eruption prior to blister development was oberved, both posing diagnostic challenges. In addition to topical and systemic treatment, most patients required either discontinuation or permanent interruption of immunotherapy. Assessment of tumour outcome did not reveal improved survival in patients developing bullous pemphigoid during immunotherapy, as suggested for other types of skin toxicity, including vitiligo. Better understanding of the pathogenetic mechanism and prognostic implications of this increasingly-reported adverse event is essential in order to establish optimal diagnostic and therapeutic management of these patients.
Topics: Humans; Immunotherapy; Ligands; Neoplasms; Pemphigoid, Bullous; Programmed Cell Death 1 Receptor
PubMed: 33426566
DOI: 10.2340/00015555-3740 -
Acta Dermato-venereologica Mar 2022
Topics: Humans; Pemphigoid, Bullous; Pityriasis Rubra Pilaris
PubMed: 35170740
DOI: 10.2340/actadv.v102.1289 -
Acta Dermatovenerologica Alpina,... Mar 2022Comorbidities of bullous pemphigoid (BP) have not been thoroughly described in Morocco. This study investigates clinical features, comorbidities, and medications in a...
BACKGROUND
Comorbidities of bullous pemphigoid (BP) have not been thoroughly described in Morocco. This study investigates clinical features, comorbidities, and medications in a cohort of Moroccan patients with confirmed BP to help decrease morbidity and mortality.
MATERIAL AND METHODS
This cross-sectional study involved 81 cases of BP diagnosed in 2015-2018 and 162 age- and sex-matched controls with complete follow-up at the Department of Dermatology in a university hospital setting.
RESULTS
Eighty-one individuals were included in the study; the mean age at diagnosis was 71.3 years, and 53% were men. The most common comorbidities were hypertension (58%), type 2 diabetes (43%), and dyslipidemia (31%). Almost a quarter of the cohort (28%) had been diagnosed with at least one neurological disease before the onset of BP. BP was significantly associated with the presence of malignancies (14%; p = 0.017) and stroke (16%; p = 0.009) compared to an age-matched control group. The most common standard medications were beta-blockers, diuretics, and statins. In total, 86% of the patients with type 2 diabetes were taking antidiabetic drugs, especially metformin (82%) and gliptins (51%).
CONCLUSION
This study showed that BP is associated with stroke and the presence of malignancy compared to the age-matched general population. This study also calls for investigation into the specific role of some drugs as inducing factors for BP.
Topics: Case-Control Studies; Comorbidity; Cross-Sectional Studies; Diabetes Mellitus, Type 2; Humans; Male; Pemphigoid, Bullous
PubMed: 35339128
DOI: No ID Found