Article
Dermatology
Janet A. Fairley, Tyler P. Crowe, Maryam Fakhimi, Samuel J. Palmer, Mark A. Greiner, John W. Hellstein, Douglas J. Van Daele, Kelly N. Messingham
Summary: Mucous membrane pemphigoid is a diverse autoimmune disease with multiple autoantigens. A study evaluated the reactivity to different antigens in 70 patients and found that BP180 was the most common autoantigen. Reactivity to dermal antigens predicted a more severe disease and decreased response to rituximab. Confirmation of laminin 332 reactivity is important due to an increased risk of solid tumors. Ocular mucosae should be monitored in patients with IgA positivity.
JOURNAL OF INVESTIGATIVE DERMATOLOGY
(2023)
Article
Immunology
John Baker, Kristina Seiffert-Sinha, Animesh A. Sinha
Summary: This study found that HLA genotype, ethnicity, and antibody levels are correlated with the types of lesions in patients with pemphigus vulgaris. The findings highlight the impact of genetic factors on disease development and provide insights for improved clinical management strategies.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Immunology
Cristian Papara, Detlef Zillikens, Christian D. Sadik, Adrian Baican
Summary: Autoimmune blistering diseases, such as pemphigus and pemphigoid diseases (PPDs), are characterized by skin and mucous membrane blisters induced by autoantibodies. While microRNAs may play a significant role in the pathogenesis of these diseases, research on their involvement is still in its early stages. This suggests potential for miRNAs to uncover new aspects of PPDs and develop therapeutic options.
AUTOIMMUNITY REVIEWS
(2021)
Article
Multidisciplinary Sciences
Anna M. Sigmund, Markus Winkler, Sophia Engelmayer, Daniela Kugelmann, Desalegn T. Egu, Letyfee S. Steinert, Michael Fuchs, Matthias Hiermair, Mariya Y. Radeva, Franziska C. Bayerbach, Elisabeth Butz, Stefan Kotschi, Christoph Hudemann, Michael Hertl, Sunil Yeruva, Enno Schmidt, Amir S. Yazdi, Kamran Ghoreschi, Franziska Vielmuth, Jens Waschke
Summary: Pemphigus vulgaris is a life-threatening blistering skin disease caused by autoantibodies. Apremilast, a phosphodiesterase 4 inhibitor used in psoriasis, was found to prevent skin blistering in pemphigus vulgaris. It stabilizes keratinocyte adhesion and induces phosphorylation of plakoglobin, leading to the assembly of desmosomal plaques.
NATURE COMMUNICATIONS
(2023)
Review
Dermatology
Christoph T. Ellebrecht, Damian Maseda, Aimee S. Payne
Summary: Pemphigus and pemphigoid serve as models for understanding antibody-mediated autoimmune diseases in humans. Pemphigus is caused by IgG4 autoantibodies interfering with desmoglein interactions, while pemphigoid is caused by autoantibodies interfering with hemidesmosomal adhesion. Unraveling the mechanisms of these diseases has opened up opportunities for clinical trials to discover safer and more effective therapies.
JOURNAL OF INVESTIGATIVE DERMATOLOGY
(2022)
Article
Dermatology
Kaan Yilmaz, Onur Dikmen, Nina Van Beek, Jens U. Marquardt, Martha M. Kirstein, Detlef Zillikens, Enno Schmidt
Summary: This study found that oesophageal direct immunofluorescence samples are not very effective for the diagnosis of mucous membrane pemphigoid, with lower positivity rates and antibody responses compared to oral and cutaneous tissue samples.
ACTA DERMATO-VENEREOLOGICA
(2023)
Article
Dermatology
S. Staender, C. M. Hammers, A. Vorobyev, E. Schmidt, D. Zillikens, S. Ghorbanalipoor, K. Bieber, R. J. Ludwig, K. Kridin
Summary: The study investigated the histopathological characteristics of 136 patients with BP, finding that eosinophil-predominance was associated with higher seropositivity of anti-BP180 NC16A IgG. Lymphocyte-predominance in the inflammatory infiltrate predicted a more severe phenotype.
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2021)
Article
Multidisciplinary Sciences
Neha Thakur, Debajyoti Chatterjee, Anubha Dev, Rahul Mahajan, Sanjeev Handa, Dipankar De
Summary: Immunohistochemistry (IHC) for C3d and C4d can help differentiate between bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP). C3d has better diagnostic importance in BP, while C3d and C4d have lower diagnostic significance in MMP.
SCIENTIFIC REPORTS
(2023)
Article
Dentistry, Oral Surgery & Medicine
Hiroyasu Endo
Summary: The study aimed to explore the clinical characteristics of BP180NC16a autoantibody-positive mucous membrane pemphigoid. The results showed that BP180NC16a reactivity was associated with the clinical phenotype characterized by only oral mucosal lesions or combined oral mucosal and skin lesions. BP180NC16a autoantibody might be useful as a serum marker to predict low-risk mucous membrane pemphigoid.
Article
Dentistry, Oral Surgery & Medicine
Hiroyasu Endo, Terry D. Rees, Hideo Niwa, Kayo Kuyama, Maya Oshima, Tae Serizawa, Shigeo Tanaka, Morio Iijima, Masamichi Komiya
Summary: The study aimed to evaluate upper aerodigestive tract involvement in patients with mucous membrane pemphigoid associated with desquamative gingivitis, finding a significant association between symptoms, high oral disease activity score, and linear IgA deposition with upper aerodigestive tract lesions.
Review
Immunology
Connor Cole, Keshavamurthy Vinay, Luca Borradori, Kyle T. Amber
Summary: Bullous pemphigoid is an autoimmune blistering disease caused by autoantibodies. While typically considered complement-mediated, recent evidence suggests complement-independent mechanisms may also contribute to tissue damage and blister formation, opening new perspectives for targeted treatments.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Shan Cao, Xiaochen Cui, Jianke Li, Futang Pan, Xiaoxiao Yan, Qing Yang, Mingfei Chen, Shengji Zhou, Donghong Du, Weiwei Wang, Yuanhang Sun, Zhongxiang Shi, Mei Wu, Baoqi Yang, Furen Zhang
Summary: This study investigated nail changes in patients with pemphigus and bullous pemphigoid and compared them with a control group. The results showed that nail changes were frequently observed in patients with these diseases, and the type and onset time of the changes may indicate the severity of the diseases. Nail changes could be a potential marker for disease progression.
FRONTIERS IN MEDICINE
(2022)
Review
Immunology
Katja Bieber, Khalaf Kridin, Shirin Emtenani, Katharina Boch, Enno Schmidt, Ralf J. Ludwig
Summary: Pemphigus and pemphigoid diseases are autoimmune bullous diseases characterized by autoantibodies targeting adhesion molecules in the skin and mucous membranes. Personalized medicine aims to tailor medical decisions based on individual patient's predicted response or risk factors. Milestones in personalized medicine for pemphigus and pemphigoid include verifying autoimmune pathogenesis, identifying autoantigens, and utilizing chimeric autoantigen receptor (CAAR) T cells for treatment.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Dermatology
Jenny Giang, Martijn B. A. van Doorn, Gilles F. H. Diercks, Santiago Rodriguez de Cordoba, Thierry P. P. van den Bosch, Marco W. J. Schreurs, Felix Poppelaars, Jeffrey Damman
Summary: This study investigates complement activation in BP skin biopsies and serum samples. The authors found activation of classical and alternative complement pathways, but not lectin pathway. Inhibition of C1 and factor B selectively reduced complement deposition, while inhibition of C3 and C5 reduced both deposition and release of complement.
EXPERIMENTAL DERMATOLOGY
(2023)
Article
Dermatology
Khalaf Kridin, Christoph M. Hammers, Ralf J. Ludwig, Erez Onn, Yochai Schonmann, Abed Abu-Elhija, Dana Tzur Bitan, Enno Schmidt, Orly Weinstein, Arnon D. Cohen
Summary: There is a bidirectional association between bullous pemphigoid (BP) and atopic dermatitis (AD) and allergic rhinitis (AR). A history of AD and AR increases the risk of developing BP, while BP patients are at an increased risk of subsequent AD. BP patients with comorbid AD and AR are more likely to be managed with drugs and corticosteroids, and have reduced all-cause mortality.
