Article
Dermatology
Emma Treco, Eunice Huan, Afshin Varzavand, Janet A. Fairley, Kelly N. Messingham
Summary: The study found that sCD48 is elevated in Bullous pemphigoid (BP) patients and that patients with mild-moderate disease have higher levels of sCD48 than those with severe disease. Furthermore, there is an inverse relationship between sCD48 and disease activity, serum BP180 IgE, and peripheral eosinophil numbers.
EXPERIMENTAL DERMATOLOGY
(2023)
Article
Dermatology
Yen-Chi Shih, Huijie Yuan, Jia Shen, Jie Zheng, Meng Pan
Summary: The study found that anti-BP230 IgE is more frequently observed in topical-steroid-therapy-resistant patients with bullous pemphigoid, and it prefers the R1 domain of BP230, which is not included in commercially available testing kits. Detection of anti-BP230 IgE can serve as an indicator for initiating systemic steroid therapy.
JOURNAL OF DERMATOLOGY
(2021)
Article
Immunology
Hiroshi Koga, Kwesi Teye, Arisa Sugawara, Masahiro Tsutsumi, Norito Ishii, Takekuni Nakama
Summary: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease with a prominent role of T helper 2 (Th2) cells. IL-9 is elevated in the serum and lesions of BP, and could be a biomarker of BP.
FRONTIERS IN IMMUNOLOGY
(2023)
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
Dermatology
Chao Sun, Suying Feng
Summary: Bullous pemphigoid is a common autoimmune bullous disease that primarily affects the elderly population and has a high mortality rate. It is clinically characterized by intensely pruritic and widespread bullous lesions, with limited alternative therapy options due to unclear pathophysiological mechanisms of itching. This review aims to explain crucial concepts in the pathogenesis of pruritus in bullous pemphigoid.
INTERNATIONAL JOURNAL OF DERMATOLOGY
(2021)
Review
Immunology
Tianmeng Yan, Zhenying Zhang
Summary: Bullous pemphigoid (BP) is an autoimmune blistering disease primarily affecting elderly individuals. It presents with heterogeneous manifestations, typically characterized by microscopically subepidermal separation and mixed inflammatory infiltrate. The development mechanism of BP remains unclear. B cells are involved in pathogenic autoantibody production, and other immune cells such as T cells, type II inflammatory cytokines, eosinophils, mast cells, neutrophils, and keratinocytes also contribute to the pathogenesis of BP. This review focuses on the roles and crosstalk between innate and adaptive immune cells in BP.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medicine, General & Internal
Wu Han Toh, Hua-En Lee, Chun-Bing Chen
Summary: Bullous pemphigoid (BP) is a common autoimmune bullous disease that mainly affects elderly patients with multiple conditions. The pathogenesis of BP is driven by type 2 inflammation, involving CD4+ T cells, IgE-secreting B cells, and granulocytes. This review discusses recent understandings of BP pathogenesis and the use of rituximab, omalizumab, and dupilumab as treatment options. It also highlights emerging targeted therapeutic approaches for BP.
FRONTIERS IN MEDICINE
(2023)
Review
Immunology
Luyao Zhang, Zihua Chen, Lanting Wang, Xiaoqun Luo
Summary: Bullous pemphigoid (BP), an autoimmune disease prevalent in the elderly, significantly impacts their health and life quality. Traditional therapy using corticosteroids comes with side effects, prompting the development of targeted drugs for type 2 inflammatory diseases. This review focuses on the role of type 2 inflammation in BP's pathogenesis and explores potential therapeutic targets and medications.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Giovanni Di Zenzo, Giovanna Floriddia, Sabrina Rossi, Feliciana Mariotti, Alessia Primerano, Angelo Giuseppe Condorelli, Biagio Didona, Daniele Castiglia
Summary: This study reports a case of a patient with hereditary epidermolysis bullosa (EB) who also developed an autoimmune response to basement membrane components. Diagnosis of bullous pemphigoid (BP) was confirmed by detecting antibodies in the patient's skin and serum. Corticosteroid therapy led to remission of BP manifestations. EB patients presenting rapid disease worsening should be investigated for the development of a concomitant autoimmune blistering disease.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Medicine, General & Internal
Xinyi Chen, Wenlin Zhao, Hongzhong Jin, Li Li
Summary: Bullous pemphigoid (BP) patients with mucosal involvement tend to have more serious conditions and their disease is more difficult to control. Potential risk factors for mucous involvement include earlier age at onset, drugs, cancer, and blood/serum biomarkers. Understanding these risk factors may benefit earlier diagnosis and the development of novel treatments.
FRONTIERS IN MEDICINE
(2021)
Article
Dermatology
A. Lamberts, N. Kotnik, G. F. H. Diercks, J. M. Meijer, G. Di Zenzo, H. H. Pas, M. F. Jonkman, B. F. Gibbs, U. Raap, B. Horvath
Summary: Total IgE levels were elevated in a significant percentage of both NBP and BP patients, with IgE being present in both serum and skin. The study suggests a common IgE-dependent mechanism in both diseases, such as pruritus, but further research is needed to determine if IgE contributes to blister formation in BP.
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Hui Fang, Shuai Shao, Ke Xue, Xu Yuan, Pei Qiao, Jieyu Zhang, Tianyu Cao, Yixin Luo, Xiaocui Bai, Wenjing Li, Caixia Li, Hongjiang Qiao, Erle Dang, Gang Wang
Summary: Bullous pemphigoid (BP) patients exhibit abnormal neutrophil extracellular traps (NETs) in circulation, which are closely correlated with disease severity. The study demonstrates that neutrophils from BP patients display enhanced spontaneous NETs formation. Moreover, elevated levels of NETs in patients boost autoantibody production.
Review
Immunology
Haeun Ko, Chan Johng Kim, Sin-Hyeog Im
Summary: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that mainly affects women in their reproductive years. Type 2 immune responses are involved during the pathology of SLE.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Dermatology
Adele Salemme, Luca Fania, Alessandra Scarabello, Marzia Caproni, Angelo Valerio Marzano, Emanuele Cozzani, Claudio Feliciani, Clara De Simone, Manuela Papini, Rosanna Rita Satta, Aurora Parodi, Feliciana Mariotti, Stefania Lechiancole, Giovanni Genovese, Francesca Passarelli, Francesca Festa, Barbara Bellei, Alessia Provini, Donatella Sordi, Sabatino Pallotta, Damiano Abeni, Cinzia Mazzanti, Biagio Didona, Giovanni Di Zenzo
Summary: This study clinically and immunologically characterized a large cohort of GABP patients and found that they exhibit peculiar features, including a noninflammatory phenotype, reduced humoral responses to BP180 and BP230 antigens, and IgG reactivity targeting multiple BP180 epitopes.
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2022)
Article
Immunology
Marina Alexandre, Gerome Bohelay, Thomas Gille, Christelle Le Roux-Villet, Isaac Soued, Florence Morin, Frederic Caux, Sabine Grootenboer-Mignot, Catherine Prost-Squarcioni
Summary: In this study, the effectiveness of omalizumab (OMZ) in patients with refractory bullous pemphigoid and mucous membrane pemphigoid was evaluated. The results showed that OMZ induced a rapid improvement in symptoms, allowed disease control, and reduced concomitant therapies. This is the largest retrospective study on the use of OMZ in the treatment of bullous pemphigoid and mucous membrane pemphigoid.
FRONTIERS IN IMMUNOLOGY
(2022)