Desmosomal hyper-adhesion affects direct inhibition of desmoglein interactions in pemphigus
During differentiation, keratinocytes acquire a strong, hyper-adhesive state, where desmosomal cadherins interact Ca2+-independently. Previous data indicate that hyper-adhesion protects keratinocytes from pemphigus vulgaris autoantibody (PV-IgG)-induced loss of intercellular adhesion although the underlying mechanism remains to be elucidated. Thus, we here investigated the effect of hyper-adhesion on PV-IgG-induced direct inhibition of desmoglein (Dsg) 3 interactions by atomic force microscopy.Hyper-adhesion abolished loss of intercellular adhesion and corresponding morphological changes of all pathogenic antibodies used. ...
Source: Journal of Investigative Dermatology - April 25, 2024 Category: Dermatology Authors: Letyfee Steinert, Michael Fuchs, Anna M. Sigmund, Dario Didona, Christoph Hudemann, Christian M öbs, Michael Hertl, Takashi Hashimoto, Jens Waschke, Franziska Vielmuth Tags: Original Article Source Type: research
EGFR Inhibition by Erlotinib Rescues Desmosome Ultrastructure and Keratin Anchorage and Protects Against Pemphigus Vulgaris IgG-Induced Acantholysis in Human Epidermis.
Pemphigus is a severe blistering disease caused by autoantibodies primarily against the desmosomal cadherins desmoglein (DSG)1 and DSG3 which impair desmosome integrity. Especially for the acute phase, additional treatment options allowing to reduce corticosteroids would fulfill an unmet medical need. Here, we provide evidence that epidermal growth factor receptor (EGFR) inhibition by erlotinib ameliorates pemphigus vulgaris immunoglobulin G (PV-IgG) -induced acantholysis in intact human epidermis. (Source: Journal of Investigative Dermatology)
Source: Journal of Investigative Dermatology - April 18, 2024 Category: Dermatology Authors: Desalegn Tadesse Egu, Thomas Schmitt, Nancy Ernst, Ralf Joachim Ludwig, Michael Fuchs, Matthias Hiermaier, Sina Moztarzadeh, Carla Sebasti à Morỏn, Enno Schmidt, Vivien Beyersdorfer, Volker Spindler, Letyfee Sarah Steinert, Franziska Vielmuth, Anna Mag Tags: Original Article Source Type: research
Understanding patient perspectives on vaccine decision making in adults with autoimmune bullous diseases: a qualitative study
AbstractPatients with autoimmune bullous diseases are at an increased risk of infection, both from the underlying skin disease and from immunosuppressive treatments. Limited information is available on vaccine beliefs and behaviors in dermatology patients and adults with autoimmune bullous diseases in particular. To understand vaccine decision making, identify perceived risks and benefits of vaccinations, and discuss individual experiences in patients with autoimmune bullous diseases in the United States. A qualitative study was performed utilizing semi-structured interviews, and analysis was conducted on NVivo. Patterns w...
Source: Archives of Dermatological Research - April 18, 2024 Category: Dermatology Source Type: research
Effectiveness and safety of immunoadsorption in the treatment of pemphigus vulgaris and pemphigus foliaceus
Br J Dermatol. 2024 Apr 17;190(5):e53. doi: 10.1093/bjd/ljae119.NO ABSTRACTPMID:38630932 | DOI:10.1093/bjd/ljae119 (Source: The British Journal of Dermatology)
Source: The British Journal of Dermatology - April 17, 2024 Category: Dermatology Source Type: research
Single-cell sequencing reveals distinct immune cell features in cutaneous lesions of pemphigus vulgaris and bullous pemphigoid
Clin Immunol. 2024 Apr 15:110219. doi: 10.1016/j.clim.2024.110219. Online ahead of print.ABSTRACTBullous pemphigoid (BP) and pemphigus vulgaris (PV) are two common subtypes of autoimmune bullous disease (AIBD). The key role of circulating autoreactive immune cells contributing to skin damage of AIBD has been widely recognized. Nevertheless, the immune characteristics in cutaneous lesions remain unclear. Here, we performed single-cell RNA sequencing (scRNA-seq) and single-cell VDJ sequencing (scRNA-seq) to generate transcriptional profiles for cells and T/B cell clonetype in skin lesions of BP and PV. We found that the prop...
Source: Clinical Immunology - April 17, 2024 Category: Allergy & Immunology Authors: Zhi Hu Meiling Zheng Ziyu Guo Wenhui Zhou Wenyu Zhou Nan Yao Guiying Zhang Qianjin Lu Ming Zhao Source Type: research
Effectiveness and safety of immunoadsorption in the treatment of pemphigus vulgaris and pemphigus foliaceus
Br J Dermatol. 2024 Apr 17;190(5):e53. doi: 10.1093/bjd/ljae119.NO ABSTRACTPMID:38630932 | DOI:10.1093/bjd/ljae119 (Source: The British Journal of Dermatology)
Source: The British Journal of Dermatology - April 17, 2024 Category: Dermatology Source Type: research
Single-cell sequencing reveals distinct immune cell features in cutaneous lesions of pemphigus vulgaris and bullous pemphigoid
Clin Immunol. 2024 Apr 15;263:110219. doi: 10.1016/j.clim.2024.110219. Online ahead of print.ABSTRACTBullous pemphigoid (BP) and pemphigus vulgaris (PV) are two common subtypes of autoimmune bullous disease (AIBD). The key role of circulating autoreactive immune cells contributing to skin damage of AIBD has been widely recognized. Nevertheless, the immune characteristics in cutaneous lesions remain unclear. Here, we performed single-cell RNA sequencing (scRNA-seq) and single-cell VDJ sequencing (scRNA-seq) to generate transcriptional profiles for cells and T/B cell clonetype in skin lesions of BP and PV. We found that the ...
Source: Clinical Immunology - April 17, 2024 Category: Allergy & Immunology Authors: Zhi Hu Meiling Zheng Ziyu Guo Wenhui Zhou Wenyu Zhou Nan Yao Guiying Zhang Qianjin Lu Ming Zhao Source Type: research
Role of auto-antibodies in the mechanisms of dengue pathogenesis and its progression: a comprehensive review
Arch Microbiol. 2024 Apr 15;206(5):214. doi: 10.1007/s00203-024-03954-0.ABSTRACTA complex interaction among virulence factors, host-genes and host immune system is considered to be responsible for dengue virus (DENV) infection and disease progression. Generation of auto-antibodies during DENV infection is a major phenomenon that plays a role in the pathophysiology of dengue hemorrhagic fever and dengue shock syndrome. Hemostasis, thrombocytopenia, hepatic endothelial dysfunction, and autoimmune blistering skin disease (pemphigus) are different clinical manifestations of dengue pathogenesis; produced due to the molecular mi...
Source: Archives of Microbiology - April 14, 2024 Category: Microbiology Authors: Tanusree Ghorai Avipsha Sarkar Anirban Roy Bijita Bhowmick Debadatta Nayak Satadal Das Source Type: research
Delayed diagnosis of pemphigus vulgaris in rural Nepal due to healthcare inaccessibility and harmful traditional practices: A case report
Key Clinical MessageEarly intervention is imperative for potentially fatal dermatologic diseases such as pemphigus vulgaris. In rural Nepal, limited public awareness, home remedies, and delays in healthcare access lead to poor outcomes. Although biopsy confirms the diagnosis, experienced dermatologists can make an accurate clinical diagnosis when characteristic skin lesions are present. (Source: Clinical Case Reports)
Source: Clinical Case Reports - April 12, 2024 Category: General Medicine Authors: Amisha Adhikari,
Jameel Akhtar Mikrani,
Samata Nepal,
Ashish Rauniyar,
Manish Chaudhary,
Alok Atreya Tags: CASE REPORT Source Type: research
Paraneoplastic pemphigus in a patient with T-cell lymphoma: a case report
CONCLUSION: This case highlights the rare association between T-cell lymphoma and PNP. Clinicians should also remain vigilant for the possibility of PNP in lymphomas that are not of B-cell lineage.PMID:38576992 | PMC:PMC10990377 | DOI:10.1097/MS9.0000000000001920 (Source: Annals of Medicine)
Source: Annals of Medicine - April 5, 2024 Category: Internal Medicine Authors: Yohannis Derbew Molla Isak Omer Answar Biruk Mulat Worku Amanuel Kassa Tadesse Elias Manaye Tefera Bewketu Abebe Alemu Gebrehiwot Lema Legese Samuel Addisu Abera Source Type: research
Pemphigus Vulgaris
A man in his mid-40s was referred with a 2-week history of painful erosions in his mouth followed by spontaneous fragile blisters on his trunk and extremities, resulting in painful erosions. Physical examination demonstrated oral lesions, widespread skin erosions, crusted lesions, and flaccid bullae over his body (Figure 1). (Source: Mayo Clinic Proceedings)
Source: Mayo Clinic Proceedings - April 1, 2024 Category: Internal Medicine Authors: Nika Kianfar, Maryam Daneshpazhooh, Julia S. Lehman Tags: Medical image Source Type: research
ECMO Bridge to Bilateral Lung Transplant for Castleman Disease
Introduction: Castleman disease (CD) is a rare, non-clonal lymphoproliferative disorder. Paraneoplastic pemphigus (PNP) associated with bronchiolitis obliterans (BO) is a rare complication that can lead to end-stage lung disease. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , J.P. Villamizar, T. Machuca, D. Buitrago, M. Pipkin, S. Manickavel, N. Sinha, A. Pelaez, J. Salgado, J. Fernandez Source Type: research
