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Bullous Pemphigoid

Asta Literature Retrieval: Pathophysiology and clinical mechanisms of Bullous Pemphigoid. Core disease mechanisms, molecular and cellular pathwa...

2026-07-31
Asta MONDO:0019082 Model: Asta Scientific Corpus Retrieval 19 citations

Asta Literature Retrieval: Pathophysiology and clinical mechanisms of Bullous Pemphigoid. Core disease mechanisms, molecular and cellular pathwa...

This report is retrieval-only and is generated directly from Asta results.

  • Papers retrieved: 19
  • Snippets retrieved: 20

Relevant Papers

[1] Pruritogens in pemphigoid diseases: Possible therapeutic targets for a burdensome symptom

  • Authors: S. Hiroyasu, J. Barit, Aoi Hiroyasu, D. Tsuruta
  • Year: 2022
  • Venue: The Journal of Dermatology
  • URL: https://www.semanticscholar.org/paper/439345d0ec9a68d501e7f33442df621509b7f3d0
  • DOI: 10.1111/1346-8138.16652
  • PMID: 36477831
  • PMCID: 10108135
  • Citations: 2
  • Summary: The present understanding of pruritus specific to pemphigoid diseases, especially the pruritogens that induce it, and the therapeutic options that have been explored so far are summarized.
  • Evidence snippets:
  • Snippet 1 (score: 0.536) > Pruritus is a hallmark feature in pemphigoid diseases, where it can be severe and greatly impact the quality of life of affected patients. Despite being a key symptom, the exact pathophysiological mechanisms involved in pruritus in pemphigoid are yet to be fully elucidated and effective therapies addressing them are limited. This review summarizes the present understanding of pruritus specific to pemphigoid diseases, especially the pruritogens that induce it, and the therapeutic options that have been explored so far. The majority of the available evidence is on bullous pemphigoid and epidermolysis bullosa acquisita. Histamine derived from basophils correlates with pruritus severity, with omalizumab demonstrating promising efficacy in pruritus for bullous pemphigoid. IL‐4/−13 contribute to itch in bullous pemphigoid with dupilumab being evaluated in clinical trials. Other pruritogens of interest include substance P, tryptase, and thymic stromal lymphopoetin, with therapies targeting them requiring further investigation. Scratching behaviors contribute directly to blister formation through various mechanisms, such as pathological autoantibody recruitment, T helper cell type 1 polarization, and exposure of intracellular autoantigens. Treatments addressing these pathways may contribute to decreasing disease severity. Additional studies are needed to fully characterize how pruritus is regulated in pemphigoid diseases, to help pave the way to develop novel and effective therapeutics that will not only address pruritic symptoms but also decrease disease severity.

[2] A Scoping Review of the Role of Metalloproteinases in the Pathogenesis of Autoimmune Pemphigus and Pemphigoid

  • Authors: N. Cirillo, S. Prime
  • Year: 2021
  • Venue: Biomolecules
  • URL: https://www.semanticscholar.org/paper/6969c4ad3fe0903815379325160d3881f6c93683
  • DOI: 10.3390/biom11101506
  • PMID: 34680139
  • PMCID: 8533820
  • Citations: 29
  • Summary: The results demonstrated that ADAM10 and MMP-9 activity is necessary for blister formation in experimental models of pemphigus vulgaris (PV) and BP, respectively.
  • Evidence snippets:
  • Snippet 1 (score: 0.514) > Pemphigus and pemphigoid are members of a heterogeneous group of chronic, potentially fatal autoimmune diseases associated with blistering of the skin and mucosae. Once considered part of the same disease spectrum, these bullous dermatoses were first classified as distinct entities by Lever in 1953 [1]. Histologically, pemphigus presents as an intraepithelial split (acantholysis) whereas in pemphigoid, the blisters develop at the dermo-epidermal junction resulting in a subepithelial split. > The two major pemphigus variants, pemphigus vulgaris and pemphigus foliaceus, account for 90-95% of diagnoses [2,3]. Pemphigus diseases are characterized by flaccid blisters and erosions and are treated with high dose corticosteroids and immunosuppressants. By contrast, pemphigoid presents with tense blisters and erosions, a negative Nikolsky sign and, commonly, is controlled using topical steroids although in more severe cases, systemic steroids are an option [2]. > The development of molecular techniques has facilitated the further classification of these blistering diseases. The pemphigoid group is now known to include at least eight disorders for which the molecular target antigens have been identified [4], although controversy exists regarding the pathogenic mechanisms of blistering [5]. In pemphigus, distinct autoantibody profiles and, in particular, the presence/absence and type of antibodies to cadherins determines the acantholytic signaling pathways [6,7] and possibly the clinical phenotype [8,9]. Despite advances in understanding the pathophysiology of these disorders, however, the mainstay of treatment is the use of corticosteroids and immune suppressants and, significantly, no mechanism-based treatments have been successfully translated to patient care. > Pemphigus and pemphigoid result from a deficit in cellular adhesion.

[3] Review on Bullous Pemphigoid: Fixed Drug Eruption or Autoimmune Disorder

  • Authors: Adarsh Keshari, K. Jain, Roshan Pandey, Ayush Mishra, Sarita Jangra et al.
  • Year: 2024
  • Venue: Journal of Pharmaceutical Technology, Research and Management
  • URL: https://www.semanticscholar.org/paper/e1dea81c672a1cb5aee440e0961d510a9d9f0890
  • DOI: 10.15415/jptrm.2024.122002
  • Summary: Bullous pemphigoid is a distinct autoimmune disease with unique immunopathological mechanisms compared to fixed drug eruption, and understanding its pathogenesis, drug interactions, and diagnostic methods enhances accurate diagnosis and management of both spontaneous and drug-induced bullous pemphigoid.
  • Evidence snippets:
  • Snippet 1 (score: 0.509) > Background: Bullous pemphigoid is a blistering disease of autoimmune nature predominantly affecting the geriatric population. It is characterized by blister formation at the subepidermal level, due to autoantibodies at the dermo-epidermal junction targeting proteins BP180XV11 and BP230. Mainly an autoimmune condition, diagnosis and treatment get complicated as it overlaps with drug-induced hypersensitivity reactions, including fixed drug eruption. Unlike Bullous Pemphigoid, it is a condition of localized hypersensitivity mediated by T cells. > Purpose: The review tries to establish Bullous Pemphigoid as an autoimmune condition separate from fixed drug eruption. It is centered on the causative role of medications, which include diuretics, antibiotics, and dipeptidyl peptidase-4 inhibitors, in drug-induced bullous pemphigoid. Besides, it examines genetic, immunological, and environmental etiologies of the disease and delineates clinical and diagnostic characteristics of Bullous Pemphigoid and fixed drug eruptions. > Method: A systematic analysis of current literature was performed, focusing on the pathophysiology, immunological mechanisms, and histopathological differences between Bullous Pemphigoid and fixed drug eruptions. The review also examines the role of medications, genetic predispositions such as specific human leukocyte antigen haplotypes, and the diagnostic utility of histopathological and immunological methods like direct immunofluorescence. > Results: Autoantibodies against BP180 and BP230 in bullous pemphigoid initiate inflammatory cascades, causing subepidermal blistering and eosinophilic infiltration. Fixed drug eruption involves basal cell necrosis and localized lymphocytic infiltration. Drugs like dipeptidyl peptidase-4 inhibitors exacerbate bullous pemphigoid through immune modulation and oxidative stress. Genetic susceptibility plays a significant role, and immunological tests such as direct immunofluorescence help distinguish the two conditions. > Conclusion: Bullous pemphigoid is a distinct autoimmune disease with unique

[4] Role of Regulatory Immune Cells and Molecules in Autoimmune Bullous Dermatoses

  • Authors: T. Cao, S. Shao, H. Fang, Bing Li, Gang Wang
  • Year: 2019
  • Venue: Frontiers in Immunology
  • URL: https://www.semanticscholar.org/paper/c8b752685c67600e6005dd7262f2c71d7cebe877
  • DOI: 10.3389/fimmu.2019.01746
  • PMID: 31428090
  • PMCID: 6688483
  • Citations: 17
  • Summary: The role of regulatory immune cells and molecules in the pathogenesis of pemphigus vulgaris and bullous pemPHigoid, the two most representative forms of AIBD, are highlighted, and issues that should be addressed in future investigations are indicated.
  • Evidence snippets:
  • Snippet 1 (score: 0.500) > In contrast, pemphigoid diseases are characterized by autoantibodies that directly attack structural proteins within the dermal-epidermal junction, leading to urticarial lesions, and tense blisters on the skin (8). This group includes bullous pemphigoid (BP), linear IgA bullous dermatosis, dermatitis herpetiformis, mucous membrane pemphigoids, herpes gestationis, and epidermolysis bullosa acquisita (7). > Recent studies have shown several regulatory immune cells and molecules to be involved in the progression of AIBD. In this review, we aim to elucidate the different mechanisms and roles of regulatory immune cells and molecules in AIBD by summarizing and discussing the findings of various clinical and experimental studies, and outline some of the challenges that lie ahead.

[5] Editorial: Autoimmune blistering diseases: advances in the understanding of pathogenesis and new therapeutic horizons

  • Authors: G. Gasparini, K. Amber, E. Cozzani, Aurora Parodi
  • Year: 2023
  • Venue: Frontiers in Medicine
  • URL: https://www.semanticscholar.org/paper/115e121ccba4f8dca8a2feb68b98a3d416aee829
  • DOI: 10.3389/fmed.2023.1243878
  • PMID: 37502359
  • PMCID: 10369339
  • Summary: This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY).
  • Evidence snippets:
  • Snippet 1 (score: 0.491) > Developing in vitro and animal models of AIBDs is a one the fundamental approaches for investigating pathogenic mechanisms and also for initial testing of new drugs. Radine et al. evaluated in electron microscopy the ultrastructural desmosomal morphology in the human skin organ culture (HSOC) model injected with either anti-desmoglein (DSG) 1/3 single-chain variable fragment (scFv, termed Px4-3). The authors demonstrated that the HSOC pemphigus model is an attractive tool to unravel novel therapeutic targets (Radine et al.). > Currently, several clinical trials are ongoing for the two most common AIBDs, namely pemphigus and bullous pemphigoid (BP). On the other hand, for rarer AIBDs, such as epidermolysis bullosa acquisita, mucous membrane pemphigoid or paraneoplastic pemphigus, specifically designed interventional clinical trials are scant. This phenomenon is reflected also in the research trends on AIBDs. Huang et al. analyzed with a bibliometric study the trending topics in the field of pemphigoid diseases and highlighted that most publications focus on molecular mechanisms and disease management, but 70% of the studies focus on BP while only a minority investigated other AIBDs belonging to the pemphigoid group. > New drugs targeting various pathways are currently being studied for the treatment of BP. The main targeted pathways are: type 2 immune response (both innate and adaptive; e.g., IL4/IL13, IL5, and eosinophils), immunoglobulins production and catabolism [B cells, circulating IgEs, and neonatal Fc receptor (FcRn)], complement cascade and IL-17 axis. > Zeng and Murrell thoroughly describe in their review article, how type II immunity, in particular eosinophils, are at the core of BP pathogenesis, and how other pathways, including IL-17/23 axis and complement, converge on this mechanism, making it a promising therapeutic target in BP. Nonetheless, not all studied targeted therapies for eosinophils gave satisfactory results.

[6] Researching trends in pemphigoid diseases: A bibliometric study of the top 100 most cited publications

  • Authors: Shih-Cheng Huang, Tsu-Man Chiu, Chien-Ying Lee, Hui-Chin Chang, Wen-Jun Wu et al.
  • Year: 2023
  • Venue: Frontiers in Medicine
  • URL: https://www.semanticscholar.org/paper/4af460839fcaf5dd1cbbe9f3b52a70f36211c4fa
  • DOI: 10.3389/fmed.2022.1088083
  • PMID: 36698818
  • PMCID: 9868262
  • Citations: 6
  • Summary: This comprehensive bibliographic study of pemphigoid diseases provided an overview of current research focuses in the field, and topics such as disease management, molecular mechanism of pathogenesis, and drug-inducing pemphygoid diseases were highly mentioned in the most-cited studies.
  • Evidence snippets:
  • Snippet 1 (score: 0.471) > Background In the field of autoimmune and inflammatory disorders, different approaches were applied to provide information regarding disease activity, comorbidities, epidemiological reports and risk factors. However, no previous studies had thoroughly analyzed the research trend in the field, and the bibliometric analysis focusing on pemphigoid diseases was available. The objective of the current study was to evaluate the current research trend in the field. Methods A search has been conducted for the Web of Science database based on various subcategories of pemphigoid diseases. Detailed information including articles’ publication types, Author information, citation, and publication information was attained for further analysis. Results Within the 6,995 studies, the top 100 most-cited articles were extracted for analysis. Among the top 100 studies, 70% of the studies focused on bullous pemphigoid. More than 60% of the top 100 studies were studies with original data. Furthermore, 30% of the studies were guidelines and narrative reviews. For the issues primarily focused on, most of the high-impact studies described the molecular mechanism of pemphigoid diseases (26%), managements (19%), risk factors of pemphigoid diseases (17%). Additionally, some other studies provided general review or discussed about the issue of epidemiology, diagnosis/definition, comorbidities and clinical characteristics of pemphigoid diseases. Conclusion This comprehensive bibliographic study of pemphigoid diseases provided an overview of current research focuses in the field. Topics such as disease management, molecular mechanism of pathogenesis, and drug-inducing pemphigoid diseases were highly mentioned in the most-cited studies. For researchers and clinicians, the researching trend and study focus in the top-100 cited studies could serve as a potential reference for future investigation and patient management.

[7] The pathological function of neutrophils in pemphigoid diseases

  • Authors: Daisuke Matsumoto, Beni Amatya, Daisuke Tsuruta, S. Hiroyasu
  • Year: 2024
  • Venue: Dermatologica Sinica
  • URL: https://www.semanticscholar.org/paper/79539c6a9740572c4073936d7ddfbb8b6f998494
  • DOI: 10.4103/ds.ds-d-24-00027
  • Citations: 1
  • Summary: The present review provides a comprehensive summary and critical evaluation of the current understanding regarding the role of neutrophils in PDs and discusses the potential of targeting neutrophil-associated pathways as a novel therapeutic approach for the diseases.
  • Evidence snippets:
  • Snippet 1 (score: 0.458) > Pemphigoid diseases (PDs) are a group of autoimmune blistering diseases, including bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid, linear immunoglobulin A disease, and other rare variants. These diseases are characterized by the presence of autoantibodies that target proteins at the dermal-epidermal junction, resulting in the formation of tense blisters and erosions on the skin and/or mucosa. The current therapeutic approaches, such as systemic corticosteroid, are associated with significant adverse effects, highlighting that safer and more effective treatment options are an urgent clinical need. To address this unmet need, a comprehensive understanding of the detailed mechanisms underlying PDs is essential. Based on their histopathological infiltration in pemphigoid lesions, neutrophils have long been implicated as major contributors to the initiation and progression of the diseases. Numerous in vivo and in vitro studies have investigated the role of neutrophils in the pemphigoid pathology, revealing various pathological mechanisms induced by these cells, including the release of neutrophil elastase and matrix metalloproteinase-9, as well as the formation of neutrophil extracellular traps. The present review provides a comprehensive summary and critical evaluation of the current understanding regarding the role of neutrophils in PDs. In addition, it discusses the potential of targeting neutrophil-associated pathways as a novel therapeutic approach for the diseases.

[8] Pathophysiology of Bullous Pemphigoid: Role of Type 2 Inflammation and Emerging Treatment Strategies (Narrative Review)

  • Authors: V. Werth, D. Murrell, P. Joly, Renata Heck, Jamie M. Orengo et al.
  • Year: 2024
  • Venue: Advances in Therapy
  • URL: https://www.semanticscholar.org/paper/5eeb52d8f8bf82cce9767679886c0779b09d573e
  • DOI: 10.1007/s12325-024-02992-w
  • PMID: 39425892
  • PMCID: 11550233
  • Citations: 12
  • Summary: This review presents what is known about BP pathophysiology, including the role of type 2 inflammation, and discusses how findings from studies of biologics targeting type 2 immune mediators have helped to clarify the biological mechanisms driving BP pathophysiology.
  • Evidence snippets:
  • Snippet 1 (score: 0.447) > Bullous pemphigoid (BP) is an autoimmune blistering disease that most often affects elderly individuals and has a significant negative impact on quality of life. The disease is characterized primarily by autoantibodies to hemidesmosomal proteins BP180 and/or BP230, and an inflammatory reaction with notable features of type 2 inflammation, including elevated serum IgE, increased numbers of eosinophils in lesions and peripheral blood, and elevated expression of type 2 cytokines and chemokines in skin lesions. In this review, we present what is known about BP pathophysiology, including the role of type 2 inflammation, and discuss how findings from studies of biologics targeting type 2 immune mediators have helped to clarify the biological mechanisms driving BP pathophysiology. Future studies of these targeted therapies and others in development will help to further elucidate the mechanisms underlying BP pathophysiology and potentially provide better treatment options for patients.

[9] Prognostic factors for mortality in bullous pemphigoid: A systematic review and meta-analysis

  • Authors: Xianxia Chen, Yaqiang Zhang, Zhicheng Luo, Yujuan Wu, Taoxiang Niu et al.
  • Year: 2022
  • Venue: PLoS ONE
  • URL: https://www.semanticscholar.org/paper/1ab716082d41a510faf6031d1dfd8c741b3afd07
  • DOI: 10.1371/journal.pone.0264705
  • PMID: 35427358
  • PMCID: 9012347
  • Citations: 13
  • Influential citations: 1
  • Summary: Positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes mellitus were the prognostic factors for mortality in bullouspemphIGoid.
  • Evidence snippets:
  • Snippet 1 (score: 0.445) > In the recent two years, the effect of statins as a new indicator has emerged and had been considered to reduce the mortality of patients with bullous pemphigoid [11]. The specific mechanism may be immunomodulatory by reduction of the metabolites of the L-mevalonate pathway and up-regulation of the immune response through inhibition of 3-hydroxy-3-methylglutaryl CoA reductase [39]. Attention should be paid to the possible protective role of statins for the clinical prevention and treatment of patients with bullous pemphigoid. Hospitalisation days have been considered to be associated with increased mortality in patients with bullous pemphigoid. Monshi [10] believed that longer hospitalization days reflected the degree of disease, the complications in the disease course, and the occurrence of adverse events during treatment. However, there had been no uniform standard for the length of hospitalization, and this index can be easily affected by many factors, such as medical conditions and treatment levels.

[10] A Case Report on Idiopathic Severe Bullous Pemphigoid

  • Authors: F. Mohammadi, S. Zoha, Mohammed Baleeqhuddin, S. Ali, A. Faizan
  • Year: 2020
  • Venue: Journal of Drug Delivery and Therapeutics
  • URL: https://www.semanticscholar.org/paper/aaf23b1a887294962eea662c3ac2397e88fd41c5
  • DOI: 10.22270/jddt.v10i4-s.4311
  • Citations: 1
  • Summary: The treatment for bullous pemphigoid is systemic corticosteroids, topical steroids in combination with nicotinamide plus tetracycline, minocycline or doxycycline have shown success in multiple cases.
  • Evidence snippets:
  • Snippet 1 (score: 0.442) > Bullous Pemphigoid (BP) is an autoimmune disorder which exploits the immune system and affecting sub-epidermal region of skin causing mild itching to infection and blistering of sub-epidermal region. Bullous Pemphigoid is a rare dermatological disorder which can be seen in all age groups but mainly affecting elderly patients. 1 iology:-Many cases of bullous pemphigoid are due to autoantibodies against proteins arranged at the dermal-epidermal junction. Drug-induced bullous pemphigoid occurs up to 3 months after medication initiation and is usually noted during a younger to older subset of patients. Drugs which trigger this reaction are diuretics such as furosemide and spironolactone, NSAIDs, amoxicillin, PD-1/PD-L1 inhibitors, gliptins, and TNF-alpha inhibitors. 2 Pathophysiology:-There are 2 main components to the pathophysiology of BP: immunologic and inflammatory. The immunologic elements comprise autoantibodies against 2 parts of the basal keratinocyte hemidesmosomal proteins BP antigen 230 (BPAG1) and BP antigen 180 (BPAG2 or type XVII collagen) 3 These antigens play an essential part in the adhesion complexes that promote epithelial-stromal adhesion. When autoantibodies bind to their target antigen, the inflammatory component follows which then activates complement and mast cells. This causes neutrophils and eosinophils to release a variety of inflammatory cells resulting in the release of proteolytic enzymes that damage the dermal-epidermal junction 4 Clinical Presentation: -In the prodromal phase, patients may experience moderateto-severe pruritus alone or associated with urticarial, papular lesions. 5 This later evolves to bullae in weeks to months and are typically present in the axillae, on the flexor surface of the forearms, medial thighs, trunk, and abdomen. Approximately 20% of patients will have neither bullae nor erosions at time of presentation 6 . > Constitutional symptoms are uncommon, except in widespread, severe disease.

[11] Coexistence of mucous membrane pemphigoid and vitiligo

  • Authors: Sanath Aithal, Satyaki Ganguly, S. Kuruvila
  • Year: 2014
  • Venue: Indian Dermatology Online Journal
  • URL: https://www.semanticscholar.org/paper/26aef582489e608b542541748f9755d040252df4
  • DOI: 10.4103/2229-5178.142511
  • PMID: 25396136
  • PMCID: 4228648
  • Citations: 2
  • Summary: A very rare coexistence of MMP and vitiligo is reported from India, the first such report from India.
  • Evidence snippets:
  • Snippet 1 (score: 0.425) > MMP is described as a heterogeneous group of chronic, infl ammatory, mucous membrane-dominated, subepithelial blistering diseases that manifest a varying constellation of oral, ocular, skin, genital, nasopharyngeal, esophageal, and laryngeal lesions. Life-threatening airway obstruction and sight-threatening ocular scarring can occur in this condition, also known as cicatricial pemphigoid, benign MMP and incorrectly as ocular pemphigoid. [1] Various basement membrane zone components have been identifi ed as targets of autoantibodies in MMP. These include bullous pemphigoid antigen 1 (BPAg1, 230 kDa), bullous pemphigoid antigen 2 (BPAg2, 180kDa), laminin 5, laminin 6, α 6 -integrin subunit, β 4 -integrin subunit, collagen VII, and other proteins of unknown identity and/or function. Considerable variability exists in the clinical presentation of MMP. [2] arring is common at non-oral sites of involvement, contributing to disease-related morbidity. [2] A multidisciplinary approach is essential in the management of MMP. Early recognition of this disorder and treatment may reduce disease-related complications. The choice of agents for treatment of MMP is based upon the sites of involvement, clinical severity, and disease progression. > Cicatricial pemphigoid (MMP) has been rarely reported in Indian literature. [3] Nayar et al. found there is an association with autoimmune diseases, both organ and non-organ-specifi c in a group of 34 patients with cicatricial pemphigoid suggesting a possible genetic basis for the association. [4] toimmune mechanisms with an underlying genetic predisposition are the most likely causes of vitiligo, although neurohumoral and autocytotoxic hypotheses are alternative theories or contributing mechanisms. Vitiligo is associated with a number of disorders also considered to be autoimmune.

[12] Analysis of the therapeutic effect of comprehensive nursing combined with digital droplet PCR technology on bullous dermatoid diseases

  • Authors: Conghui Chen
  • Year: 2025
  • Venue: Molecular & Cellular Biomechanics
  • URL: https://www.semanticscholar.org/paper/5f527759dd205eee4ef131dfad11c0c9f5587f97
  • DOI: 10.62617/mcb1291
  • Summary: Bullous skin disease is an autoimmune disorder characterized by blistering of the skin or mucous membranes that can seriously affect quality of life and health. Similar to studies at the biomolecular (Protein-Protein Interactions Mechanics), cellular (including Cell Membrane Elasticity, Cell Adhesion Mechanics, Cytoskeleton Mechanics, Mechanical Stimuli Response, Cellular Deformation Mechanisms, Intracellular Force Transmission), and tissue levels, the goal of this study is to facilitate more...
  • Evidence snippets:
  • Snippet 1 (score: 0.421) > All cases of bullous dermatosis are diagnosed through methods such as Wood's lamp examination, direct microscopic examination of disease material, and fungal culture [23]. From the perspective of Cell Mechanics and Molecular Mechanics, when counting the incidence, pathogen type, onset season, age, gender, site of onset, types, and detection methods in detail, we can potentially gain insights into how these factors interact with Cellular Deformation Mechanisms and Protein-Protein Interactions Mechanies within the context of bullous skin diseases. This in-depth analysis helps to explore the pathogenesis and characteristics of bullous skin diseases. > Figure 1 illustrates a model for the treatment of dermatological diseases. In terms of Nucleic Acid Mechanics and Protein-Nucleic Acid Interactions Mechanies, this model aims to determine effective diagnosis and treatment methods. By considering Biomolecular Force Spectroscopy, it can screen out rapid and accurate diagnostic methods suitable for bullous skin diseases in the region. These methods may be related to the study of how mechanical forces at the biomolecular level affect the detection and understanding of the disease. Applying these methods to clinical treatment can improve diagnosis and treatment efficiency. Moreover, in the process, it also promotes biomolecular research. This research could potentially uncover more about the role of Molecular Motor Mechanics and Cytoskeleton Mechanics in the pathophysiology of bullous skin diseases, as well as how Mechanical Stimuli Response within the cells might be involved in the disease process and its diagnosis and treatment. Additionally, understanding the Cell Membrane Elasticity and Cell Adhesion Mechanics in the context of bullous skin diseases can contribute to developing more targeted diagnostic and treatment strategies. > A total of 60 patients participated in this study, including 32 males and 28 females; The age range ranged from 25 to 70 years old, with an average age of 48.5 years, and the participation period was during the 12 months from January 2022 to December 2022. Patients are recruited from XYZ Hospital, a tertiary care center specializing in dermatology. All patients included in the study had a diagnosis of herpetiform dermatoses, specifically pemphigus vulgaris and pemphigus foliaceus, based on clinical findings and laboratory findings.

[13] A Multicenter Analysis of Patients with Bullous Pemphigoid: Clinical Characteristics and Insights into Drug-Associated Disease

  • Authors: A. Małolepsza, Aleksandra Kośny, K. Juczyńska, J. Czerwińska, Magdalena Jałowska et al.
  • Year: 2026
  • Venue: International Journal of Molecular Sciences
  • URL: https://www.semanticscholar.org/paper/5f61e343822b9955eacc5b2502fd0e59d0892be5
  • DOI: 10.3390/ijms27125587
  • PMID: 42353304
  • PMCID: 13299889
  • Summary: The clinical complexity of BP is highlighted and the importance of medication review and direct immunofluorescence in diagnostic evaluation is highlighted, as well as the importance of medication review and direct immunofluorescence in diagnostic evaluation.
  • Evidence snippets:
  • Snippet 1 (score: 0.418) > Based on routine clinical practice, this category may have included tetracyclines, particularly doxycycline, which are used as an evidence-based treatment option in selected patients with bullous pemphigoid. However, because the original retrospective data collection form did not systematically capture specific therapies within the "other" category, this assumption could not be reliably confirmed [13]. Furthermore, treatment-related data were descriptive and did not include standardized information on treatment sequence, treatment response, and relapse rates. In our cohort, patients with more comorbidities tended to receive fewer treatment modalities, suggesting that multimorbidity may limit rather than intensify therapeutic options. This is clinically plausible, as frailty, cardiovascular and metabolic comorbidities, and the risk of adverse events may reduce the feasibility of systemic treatment escalation. Thus, comorbidity burden should be considered an important factor shaping real-world therapeutic decision-making in BP. > In recent years, increasing attention has been given to drugs capable of inducing bullous pemphigoid, raising the question of how such a wide range of structurally diverse molecules can trigger the development of a single disease entity. The pathophysiology is characterized by autoantibodies directed against basement membrane proteins BP180 and BP230, but the underlying mechanisms remain incompletely understood. Some authors even distinguish two distinct entities: drug-associated BP and drug-triggered BP [29]. In the latter, the clinical course closely resembles that of classical BP, with recurrent exacerbations and remissions persisting despite withdrawal of the suspected triggering agent. > Recently, particular emphasis has been placed on the role of dipeptidyl peptidase-4 inhibitors and immune checkpoint inhibitors in the development of drug-associated bullous pemphigoid. In the context of DPP-4 inhibitors, some studies suggest a distinct clinical phenotype with less inflammatory skin manifestations, whereas others have not confirmed these differences [14,[30][31][32][33][34]]. These differences have been observed in studies conducted in Japan and China, whereas European studies do not emphasize this distinction.

[14] Blister Fluid Induces MMP-9-Associated M2-Type Macrophages in Bullous Pemphigoid

  • Authors: M. Riani, C. Muller, C. Bour, P. Bernard, F. Antonicelli et al.
  • Year: 2019
  • Venue: Frontiers in Immunology
  • URL: https://www.semanticscholar.org/paper/df6625983c6e076b7543bca3bf24d92ed5a9dd9d
  • DOI: 10.3389/fimmu.2019.01858
  • PMID: 31440247
  • PMCID: 6692716
  • Citations: 15
  • Influential citations: 3
  • Summary: The influence of serum and blister fluid from patients with BP on the polarization status of macrophages with regards to the metalloproteinase-9 (MMP-9) expression strongly support the presence of M2-phenotype macrophage phenotype with pro-inflammatory properties susceptible to favor blister formation in BP.
  • Evidence snippets:
  • Snippet 1 (score: 0.416) > Bullous pemphigoid (BP) is the most common skin autoimmune subepidermal blistering disease (1,2). The disease typically presents in the elderly with a generalized pruriginous, erythematous and bullous eruption. Biologically, BP is characterized by the binding of autoantibodies directed against two components of the hemidesmosome, BP230 and BP180 that generates an inflammatory response critical for blister formation. A pathophysiological mechanism of blister formation was established, which typically involved a variety of cellular types including eosinophils, neutrophils, lymphocytes, mast cells, and macrophages (3,4). Mast cell activation upon immune complex binding play a major role in neutrophils recruitment, while macrophages were supposed to rather amplify the neutrophil infiltration in a mast cell-dependent fashion (5,6). However, the role of macrophage polarization has not been taken into consideration in the pathophysiological BP process, and especially regarding the production of MMP-9, a metalloproteinase widely involved in blister formation (7). > Macrophages display a remarkable plasticity and can change their physiology in response to environmental factors with at one extremity the inflammatory or classically activated macrophage M1 phenotype and at the other, the anti-inflammatory or alternatively activated macrophage M2 phenotype (8). The M1 phenotype is classically induced by microbial products or proinflammatory cytokines such as IFN-γ and TNFα, whereas molecules such as IL-4, IL-13, M-CSF, immune complexes, IL-10, and glucocorticoids favor the orientation toward the M2type macrophage phenotype.

[15] Bullous Pemphigoid and Other Pemphigoid Dermatoses

  • Authors: Valeryia Pratasava, V. Sahni, Aishwarya Suresh, Simo Huang, Abhi C. Are et al.
  • Year: 2021
  • Venue: Medicina
  • URL: https://www.semanticscholar.org/paper/b95124eac88691c8a63e2d1db3972ab0ef713201
  • DOI: 10.3390/medicina57101061
  • PMID: 34684098
  • PMCID: 8539012
  • Citations: 32
  • Influential citations: 2
  • Summary: Clinicians that encounter the pemphigoid disorders may benefit from an overview of their clinical presentation, diagnostic work-up, and therapeutic management, with an emphasis on the most frequently encountered pemPhigoid disease, bullous pemPHigoid.
  • Evidence snippets:
  • Snippet 1 (score: 0.413) > The pemphigoid family of dermatoses is characterized by autoimmune subepidermal blistering. The classic paradigm for pemphigoid, and the most common member, is bullous pemphigoid. Its variable clinical presentation, with or without frank bullae, is linked by significant pruritus afflicting the elderly. Mucous membrane pemphigoid is an umbrella term for a group of subepidermal blistering dermatoses that favor the mucosal membranes and can scar. Epidermolysis bullosa acquisita is a chronic blistering disorder characterized by skin fragility, sensitivity to trauma, and its treatment-refractory nature. Clinicians that encounter these pemphigoid disorders may benefit from an overview of their clinical presentation, diagnostic work-up, and therapeutic management, with an emphasis on the most frequently encountered pemphigoid disease, bullous pemphigoid.

[16] Heat Shock Protein 90 (Hsp90) and Hsp70 as Potential Therapeutic Targets in Autoimmune Skin Diseases

  • Authors: S. Tukaj, K. Sitko
  • Year: 2022
  • Venue: Biomolecules
  • URL: https://www.semanticscholar.org/paper/95a3cb15dc2a25a86387bace586e3821ced8cb81
  • DOI: 10.3390/biom12081153
  • PMID: 36009046
  • PMCID: 9405624
  • Citations: 45
  • Influential citations: 1
  • Summary: Preclinical data on the involvement of Hsp90 and Hsp70 in modulating the immune response is presented, specifically in the context of the treatment of selected autoimmune skin diseases with emphasis on autoimmune bullous skin diseases and psoriasis.
  • Evidence snippets:
  • Snippet 1 (score: 0.413) > Autoimmune bullous diseases (AIBDs) belong to a relatively rare and potentially life-threatening organ-specific group of inflammatory skin diseases characterized by the presence of autoantibodies against various structural proteins of the skin present in desmosomes (e.g., pemphigus vulgaris-PV) and hemidesmosomes (e.g., bullous pemphigoid-BP and epidermolysis bullosa acquisita-EBA), or against epidermal/tissue transglutaminases present in Duhring disease (also known as dermatitis herpetiformis-DH). Despite understanding of the pathophysiology of AIBDs, in which both innate and adaptive mechanisms of the immune response are undoubtedly involved, treatment for this group of diseases remains a challenge, due to frequent relapses after the discontinuation of therapy, numerous side effects associated with using, e.g., corticosteroids, or due to the lack of a fully effective drug [67][68][69].

[17] BULLOUS PEMPHIGOID A RARE AUTOIMMUNE DISEASE: A CASE REPORT

  • Authors: S. Biradar, S. Dhanavidya, P. Kavya, T. Keerthi, N. Sunanda et al.
  • Year: 2019
  • Venue: International Journal of Medicine and Medical Research
  • URL: https://www.semanticscholar.org/paper/efce45b04f023311dcaefd694b1a6f40159a7bed
  • DOI: 10.11603/ijmmr.2413-6077.2018.2.9248
  • Citations: 3
  • Summary: In the present case study, the use of Prednisolone has proven its efficacy in the extensive disease state of BP and improved the patient’s quality of life.
  • Evidence snippets:
  • Snippet 1 (score: 0.411) > Bullous pemphigoid is a rare autoimmune blistering disease; it typically affects the elderly and is followed by significant morbidity and mortality [7]. The clinical symptoms of the disease are development of oral lesions in about one-third of the patients; lesions may occur on the trunk, extremities, and intertriginous areas. In most of the cases, no clear precipitating factors are identified; some precipitating factors are exposure to ultraviolet light, radiation therapy and exposed to certain drugs like furosemide, penicillin, sulfasalazine, and captopril [7]. The disease is characterized by the formation of IgG auto antibodies targeting dystonin (bullous pemphigoid antigen 1 (BPAG1), and /or type XVII collagen also called bullous pemphigoid antigen 2 (BPAG2), which is a component of hemides mosomes [2]. In the present case fluid-filled lesions were seen initially over the trunk and gradually progressed involving lower and upper extremities. > The recommended treatment for BP is as follows [8]: > Initial therapy. Initial therapy is determined by the extent and rate of progression of the lesions. The priority is to control lesions usually in a slowly progressive form of the disease; initial treatment includes intralesional injections of corticosteroids or topical applications of corticosteroids. > Maintenance therapy. Once most lesions are healed, the dose and type of medication are gradually reduced to limit the risk of side effects. Understanding the rate of dose reduction is determined by clinical response and overall disease activity. It is important to monitor this balance and limit use of unnecessary medication as many fatalities are related to complications associated with the therapy. > The available treatments work via different mechanisms. Some aim to suppress the inflammatory process e.g. corticosteroids, antibiotics, anti-inflammatory mediators. Immune modulating treatments include intravenous immunoglobulins. Intravenous immunoglobulin has been widely tried as an immunomodulatory agent in various auto-antibody mediated blistering diseases. Systemic corticosteroids are most commonly used: dexamethasone and prednisolone.

[18] Upadacitinib for the management of bullous pemphigoid coexisting with psoriasis vulgaris: a case report and literature review

  • Authors: Fang Su, Tai Wang, Qunshi Qin, Zhi Xie
  • Year: 2024
  • Venue: Journal of Dermatological Treatment
  • URL: https://www.semanticscholar.org/paper/7da301fc8c62b90a0f42d61eac32645825001789
  • DOI: 10.1080/09546634.2024.2302394
  • PMID: 38263708
  • Citations: 18
  • Summary: Based on clinical observations, upadacitinib appears to be a promising and well-tolerated therapeutic option for patients with concurrent BP and psoriasis, offering valuable insights for developing appropriate treatment strategies in clinical practice.
  • Evidence snippets:
  • Snippet 1 (score: 0.403) > Bullous pemphigoid (Bp) is an autoimmune blistering skin disease, characterized by severe pruritus and the formation of blisters beneath the epidermis. Its pathogenesis involves an imbalance in th1/th2 subsets, with a predominance of th2 cytokines (1). psoriasis is an immune-mediated chronic, recurrent, inflammatory, and systemic disease, influenced by both genetic and environmental factors. the typical clinical manifestation includes localized or widespread scaly erythema or plaques, wherein dysfunctional helper t cells (th1, th17, etc.) play a crucial role in its pathogenesis (2). Both bullous pemphigoid and psoriasis are immune-related dermatological conditions that have garnered increasing attention in recent years. the precise mechanisms underlying their pathogenesis remain unclear; however, it is speculated that they may involve immune mechanisms, inflammatory cells, cytokines among others. Given the conflicting treatment approaches for Bp and psoriasis management strategies available currently, there exists significant importance in identifying effective therapeutic interventions. > the JaK/stat signaling pathway is a widely expressed signal transduction pathway involved in various biological processes, including cell proliferation, apoptosis, and immune regulation. It plays a crucial role in the development of inflammatory diseases (3,4), autoimmune disorders, and malignant tumors by activating JaKs and stats. Due to its potent regulatory effect, small molecule targeted drugs known as JaK inhibitors (JaKi) have emerged as a promising research focus for the treatment of immune-related skin diseases. JaKi can efficiently hinder JaK activation and obstruct the stat pathway by attaching to the adenosine triphosphate (atp) binding site on proteins, thus accomplishing therapeutic objectives (5). Notably, first-generation JaK inhibitors such as baricitinib or tofacitinib have shown efficacy in treating mucosal pemphigoid (6,7), while different targets alone or in combination inhibition by JaK inhibitors has demonstrated significant therapeutic effects in psoriasis (8).
  • Snippet 2 (score: 0.402) > Abstract Both bullous pemphigoid (BP) and psoriasis are common immune-related dermatological conditions in clinical practice, but the co-occurrence of these two diseases is rare. Currently, there is no consensus on the long-term safe and effective treatment for patients with both BP and psoriasis. JAK inhibitors are emerging as targeted therapeutic drugs that act by inhibiting Janus kinase activity, regulating the JAK/STAT pathway, blocking the transduction pathway of key proinflammatory cytokines, and influencing T-cell differentiation. These cytokines upstream of the JAK/STAT pathway play a pivotal role in the pathogenesis of numerous inflammatory and autoimmune disorders. Upadacitinib, a second-generation JAK inhibitor with high selectivity, demonstrates promising potential. This case report aims to provide a description of the successful treatment of bullous pemphigoid (BP) and psoriasis vulgaris by using upadacitinib, highlighting significant clinical outcomes. Additionally, we aim to analyze the underlying mechanism of upadacitinib in treating these two comorbidities by reviewing relevant literature from both domestic and international sources. Based on our clinical observations, upadacitinib appears to be a promising and well-tolerated therapeutic option for patients with concurrent BP and psoriasis, offering valuable insights for developing appropriate treatment strategies in clinical practice.

[19] Advances in Pemphigus and Pemphigoid: A Report of the International Meeting of the Pemphigus and Pemphigoid Foundation in Thessaloniki, Greece

  • Authors: Meropi Karakioulaki, Patrick Dunn, D. Murrell, Aimee S. Payne, Enno Schmidt et al.
  • Year: 2024
  • Venue: JID Innovations
  • URL: https://www.semanticscholar.org/paper/fe389e1a6cc07426f4a9357f3a2095cee6d5bb9b
  • DOI: 10.1016/j.xjidi.2024.100339
  • PMID: 39877685
  • PMCID: 11773198
  • Citations: 3
  • Summary: and
  • Evidence snippets:
  • Snippet 1 (score: 0.403) > The recent international meeting of the Pemphigus and Pemphigoid Foundation in Thessaloniki, Greece, marked a significant step forward in the understanding and management of these complex autoimmune diseases. > One of the primary areas of progress highlighted was the deepening understanding of molecular pathways involved in the pathogenesis of pemphigus and pemphigoid. This expanding knowledge offers new insights into how these diseases develop and, more importantly, opens up potential avenues for targeted therapies that could more precisely address disease mechanisms. > Nonetheless, the meeting also underscored ongoing unmet needs in treatment approaches, particularly the goal of achieving early control and sustained remission with reduced or no dependence on steroids. Developing therapies that minimize steroid use remains critical for patient QOL and safety because long-term steroid exposure can lead to serious side effects. The need for such therapies emphasizes the importance of innovative drug development and continued research into disease-modifying agents. > Looking to the future, participants identified several challenges that must be addressed to advance care further. Improving clinical trial design to better evaluate treatment outcomes and achieving consensus with regulatory agencies on metrics for success will be crucial for moving promising therapies forward. In addition, further characterization of disease pathogenesis, with a focus on individualized genetic and molecular profiles, is essential to enable more personalized, effective treatment plans for patients. > The meeting concluded with a collective expression of gratitude to all organizers and participants and a commitment to ongoing collaboration. Regular meetings such as this one will be vital for sustaining progress and fostering the innovations needed to transform patient care in pemphigus and pemphigoid.

Notes

  • This provider combines search_papers_by_relevance with snippet_search.
  • No synthesis or second-stage model call is performed.