-
Indian Journal of Dermatology,... 2017Bullous pemphigoid is the most common subepidermal immunobullous disorder. Studies have reported the association between bullous pemphigoid and various neurological...
BACKGROUND
Bullous pemphigoid is the most common subepidermal immunobullous disorder. Studies have reported the association between bullous pemphigoid and various neurological diseases.
AIMS
The aim of this study was to evaluate whether bullous pemphigoid is associated with pre-existent neurological diseases and whether specific diseases exhibit this association.
METHODS
All dermatology inpatients from January 2010 to May 2015 were analyzed. Bullous pemphigoid cases were identified based on clinical features and consistent histopathologic and direct immunofluorescence findings. Patients with other autoimmune bullous skin disorders were excluded. An equal number of inpatients with other skin conditions were selected randomly as age- and sex- matched controls.
RESULTS
Out of 3015 inpatients, 103 cases of bullous pemphigoid and 103 age- and sex-matched controls were included. Seventy six patients with bullous pemphigoid had a history of at least one neurological disease. After adjusting for age, gender, race, functional status and neuro-psychiatric medications, patients with bullous pemphigoid were found to be approximately thrice as likely to have a history of at least one neurological disease than were controls (odds ratio: 2.88; 95% confidence interval: 1.32-6.26; P = 0.008). Amongst the pre-existing neurological diseases, only dementia was statistically more prevalent in bullous pemphigoid cases compared to controls (adjusted odds ratio: 2.61; 95% confidence interval: 1.19-5.75; P = 0.017). Parkinson disease and psychiatric disorders demonstrated a higher adjusted risk among bullous pemphigoid patients but the difference was not statistically significant.
LIMITATIONS
The limitations were potential referral and selection bias, as the patients were inpatients. There is a possible misclassification as the diagnosis of neurological diseases was performed using medical records. The duration from the diagnosis of neurological diseases to bullous pemphigoid could not be accurately determined as it was a retrospective review of records and most neurological diseases have a prolonged course.
CONCLUSIONS
Pre-existent neurological disease, specifically dementia, was found to be associated with bullous pemphigoid.
Topics: Aged; Aged, 80 and over; Case-Control Studies; Dementia; Female; Humans; Male; Nervous System Diseases; Pemphigoid, Bullous; Retrospective Studies
PubMed: 28584223
DOI: 10.4103/0378-6323.198451 -
F1000Research 2018Pemphigus and pemphigoid are characterized as autoimmune blistering diseases in which immunoglobulin G autoantibodies cause blisters and erosions of the skin or mucosa... (Review)
Review
Pemphigus and pemphigoid are characterized as autoimmune blistering diseases in which immunoglobulin G autoantibodies cause blisters and erosions of the skin or mucosa or both. Recently, understanding of the pathophysiology of pemphigus and pemphigoid has been furthered by genetic analyses, characterization of autoantibodies and autoreactive B cells, and elucidation of cell-cell adhesion between keratinocytes. For the management of pemphigus and pemphigoid, the administration of systemic corticosteroids still represents the standard treatment strategy; however, evidence of the efficacy of therapies not involving corticosteroids, such as those employing anti-CD20 antibodies, is increasing. The goal should be to develop antigen-specific immune suppression-based treatments.
Topics: Antibodies, Monoclonal; Humans; Immunotherapy; Pemphigoid, Bullous; Pemphigus
PubMed: 30228865
DOI: 10.12688/f1000research.14474.1 -
BMJ (Clinical Research Ed.) Jul 2008Is increasing in elderly people in the UK
Is increasing in elderly people in the UK
Topics: Female; Humans; Incidence; Male; Pemphigoid, Bullous; Pemphigus; United Kingdom
PubMed: 18614512
DOI: 10.1136/bmj.a209 -
Frontiers in Immunology 2019Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by autoantibodies targeting cellular adhesion molecules. While IgE autoantibodies are... (Review)
Review
Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by autoantibodies targeting cellular adhesion molecules. While IgE autoantibodies are occasionally reported in other autoimmune blistering diseases, BP is unique in that most BP patients develop an IgE autoantibody response. It is not known why BP patients develop self-reactive IgE and the precise role of IgE in BP pathogenesis is not fully understood. However, clinical evidence suggests an association between elevated IgE antibodies and eosinophilia in BP patients. Since eosinophils are multipotent effector cells, capable cytotoxicity and immune modulation, the putative interaction between IgE and eosinophils is a primary focus in current studies aimed at understanding the key components of disease pathogenesis. In this review, we provide an overview of BP pathogenesis, highlighting clinical and experimental evidence supporting central roles for IgE and eosinophils as independent mediators of disease and via their interaction. Additionally, therapeutics targeting IgE, the Th2 axis, or eosinophils are also discussed.
Topics: Antibodies, Monoclonal, Humanized; Autoantibodies; Cytokines; Dystonin; Eosinophilia; Eosinophils; Humans; Immunoglobulin E; Immunoglobulin G; Immunoglobulins, Intravenous; Pemphigoid, Bullous; Receptors, IgE
PubMed: 31636640
DOI: 10.3389/fimmu.2019.02331 -
International Journal of Molecular... Nov 2023Bullous pemphigoid (BP), the most common autoimmune blistering disease, is characterized by the presence of autoantibodies targeting BP180 and BP230 in the basement... (Review)
Review
Bullous pemphigoid (BP), the most common autoimmune blistering disease, is characterized by the presence of autoantibodies targeting BP180 and BP230 in the basement membrane zone. This leads to the activation of complement-dependent and independent pathways, resulting in proteolytic cleavage at the dermoepidermal junction and an eosinophilic inflammatory response. While numerous drugs have been associated with BP in the literature, causality and pathogenic mechanisms remain elusive in most cases. Dipeptidyl peptidase 4 inhibitors (DPP4i), in particular, are the most frequently reported drugs related to BP and, therefore, have been extensively investigated. They can potentially trigger BP through the impaired proteolytic degradation of BP180, combined with immune dysregulation. DPP4i-associated BP can be categorized into true drug-induced BP and drug-triggered BP, with the latter resembling classic BP. Antineoplastic immunotherapy is increasingly associated with BP, with both B and T cells involved. Other drugs, including biologics, diuretics and cardiovascular and neuropsychiatric agents, present weaker evidence and poorly understood pathogenic mechanisms. Further research is needed due to the growing incidence of BP and the increasing identification of new potential triggers.
Topics: Humans; Pemphigoid, Bullous; Autoantigens; Dipeptidyl-Peptidase IV Inhibitors; Autoantibodies; Autoimmune Diseases
PubMed: 38069109
DOI: 10.3390/ijms242316786 -
Indian Journal of Dermatology,... 2018Increased risk of venous thromboembolism is observed in several autoimmune inflammatory disorders. However, data on bullous pemphigoid, one of the most common autoimmune... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND AND OBJECTIVES
Increased risk of venous thromboembolism is observed in several autoimmune inflammatory disorders. However, data on bullous pemphigoid, one of the most common autoimmune blistering disorders, is limited. This systematic review and meta-analysis was conducted to summarize all available evidence.
METHODS
Two investigators independently searched published studies indexed in MEDLINE and EMBASE from inception to July 2016 using the terms for bullous pemphigoid and venous thromboembolism. The inclusion criteria were as follows: (1) cohort or case-control study evaluated the association between bullous pemphigoid and risk of venous thromboembolism, (2) effect estimates were provided as odds ratios, relative risk, hazard ratio, standardized incidence ratio with 95% confidence intervals, and (3) subjects without bullous pemphigoid were used as comparators for cohort studies, while subjects without venous thromboembolism were used as comparators for case-control studies. Point estimates and 95% confidence intervals were extracted from each study and were pooled together using the random-effect model, generic inverse variance method of DerSimonian and Laird. Cochran's Q test and the I2 statistic were used to evaluate the statistical heterogeneity.
RESULTS
Two retrospective cohort studies, one prospective cohort study, and one case-control study met the eligibility criteria and were included in the meta-analysis. The pooled odds ratio was 2.69 (95% confidence interval, 1.79-4.05). Statistical heterogeneity was high with I2 of 77%.
LIMITATION
Limited accuracy of diagnosis of primary studies and high between-study heterogeneity.
CONCLUSION
This meta-analysis demonstrated that patients with bullous pemphigoid have a significantly increased risk of venous thromboembolism.
Topics: Case-Control Studies; Humans; Pemphigoid, Bullous; Prospective Studies; Retrospective Studies; Risk Factors; Venous Thromboembolism
PubMed: 29185440
DOI: 10.4103/ijdvl.IJDVL_827_16 -
Indian Journal of Dermatology,... 2017Neurological diseases are important co-morbidities found in association with bullous pemphigoid. Various neurological conditions (stroke, Parkinson's disease, dementia,...
INTRODUCTION
Neurological diseases are important co-morbidities found in association with bullous pemphigoid. Various neurological conditions (stroke, Parkinson's disease, dementia, epilepsy and multiple sclerosis) have been reported as associations of this bullous disease; whether these are significant has not been definitely proved. However, the presence of neurological conditions is a predictor of poorer prognosis.
OBJECTIVES
Our aim was to examine the association of bullous pemphigoid and neurological diseases in Iranian bullous pemphigoid patients.
METHODS
The medical records of one hundred and sixty consecutive bullous pemphigoid patients who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran, from 2006 to 2011 were examined for evidence of any neurological disease. The control group comprised of 317 age- and sex-matched subjects.
RESULTS
Neurological diseases were seen in 42 (26.4%) patients with bullous pemphigoid and in 29 (9.1%) controls (odds ratio: 3.53 (2.1-5.9), P< 0.001). Comparing cases to controls, stroke was seen in 17.5% versus 4.1%, odds ratio 4.96 (2.49-9.88); dementia in 5.6% versus 1.9%, odds ratio 3.09 (1.08-8.84); Parkinson's disease in 2.5% versus 2.2%, odds ratio 1.14 (0.33-3.94); epilepsy in 2.5% versus 0.6%, odds ratio 4.04 (0.73-22.3); and multiple sclerosis in 0 versus 0.3% odds ratio 1.00 (0.98-1.01).
LIMITATIONS
The main limitations of our study were referral bias, retrospective design and a rather low sample size.
CONCLUSIONS
Neurological diseases in general, and stroke and dementia in particular, were significantly associated with bullous pemphigoid in our study.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Case-Control Studies; Female; Humans; Iran; Male; Middle Aged; Nervous System Diseases; Pemphigoid, Bullous; Retrospective Studies; Young Adult
PubMed: 27679408
DOI: 10.4103/0378-6323.191132 -
BMC Psychiatry Aug 2020Bullous pemphigoid (BP) is an autoimmune blistering skin disease that takes a profound physical and mental toll on those affected. The aim of the study was to...
BACKGROUND
Bullous pemphigoid (BP) is an autoimmune blistering skin disease that takes a profound physical and mental toll on those affected. The aim of the study was to investigate the bidirectional association between BP and all bullous disorders (ABD) with a broad array of psychiatric disorders, exploring the influence of prescribed medications.
METHODS
This nationwide, register-based cohort study encompassed 6,470,450 individuals born in Denmark and alive from 1994 to 2016. The hazard ratios (HRs) of a subsequent psychiatric disorder in patients with BP/ABD and the reverse exposure and outcome were evaluated.
RESULTS
Several psychiatric disorders were associated with increased risk of subsequent BP (4.18-fold for intellectual disorders, 2.32-fold for substance use disorders, 2.01-fold for schizophrenia and personality disorders, 1.92-1.85-1.49-fold increased risk for organic disorders, neurotic and mood disorders), independent of psychiatric medications. The association between BP and subsequent psychiatric disorders was not significant after adjusting for BP medications, except for organic disorders (HR 1.27, CI 1.04-1.54). Similar results emerged with ABD.
CONCLUSION
Psychiatric disorders increase the risk of a subsequent diagnosis of BP/ABD independent of medications, whereas medications used for the treatment of BP/ABD appear to account for the subsequent onset of psychiatric disorders. Clinically, an integrated approach attending to both dermatological and psychiatric symptoms is recommended, and dermatologists should remain vigilant for early symptoms of psychiatric disorders to decrease mental health comorbidity.
Topics: Cohort Studies; Comorbidity; Denmark; Humans; Pemphigoid, Bullous; Schizophrenia
PubMed: 32819315
DOI: 10.1186/s12888-020-02810-x -
Actas Dermo-sifiliograficas May 2022
Topics: Blister; Humans; Pemphigoid, Bullous
PubMed: 35697410
DOI: 10.1016/j.ad.2020.10.009 -
The American Journal of Case Reports Sep 2021BACKGROUND Bullous pemphigoid is a common pruritic skin lesion reported in elderly patients. It is caused by an immunologic reaction between autoantibodies and...
BACKGROUND Bullous pemphigoid is a common pruritic skin lesion reported in elderly patients. It is caused by an immunologic reaction between autoantibodies and hemidesmosome proteins of epithelial cells. The disease is characterized by a symmetrical blister distribution on the body. Diagnosis should be suspected in elderly patients presenting with a tense blister on normal-appearing skin or on an erythematous base. In the literature, several forms of typical bullous pemphigoid after treatment with linagliptin have been reported. However, this is the first reported case of atypical nonbullous pemphigoid after linagliptin intake. CASE REPORT A 77-year-old woman presented with multiple erythematous papules and nodules on the upper extremities and trunk. The patient was being treated with linagliptin for diabetes. Diagnosis was made with biopsy and histopathological studies, followed by direct immunofluorescence. The histopathological study showed a subepidermal blister with an underlying polymorphous infiltrate, mainly of an eosinophilic profile. Direct immunofluorescence showed linear IgG and C3 antibodies to hemidesmosomes at the lamina lucida of the basement membrane. Thus, the diagnosis of atypical nonbullous pemphigoid was made. CONCLUSIONS This report emphasizes the great variety of bullous pemphigoid presentation and the need for a greater level of awareness of the adverse effects of linagliptin. Thus, atypical nonbullous pemphigoid should be considered among the potential differential diagnoses in patients with multiple erythematous papules and nodules on the upper extremities and trunk.
Topics: Aged; Autoantibodies; Basement Membrane; Blister; Female; Humans; Linagliptin; Pemphigoid, Bullous
PubMed: 34584063
DOI: 10.12659/AJCR.932356