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

Evolution of rituximab use over time in moderate-to-severe pemphigus: A two-centre retrospective study
Ann Dermatol Venereol. 2024 Mar 28;151(2):103265. doi: 10.1016/j.annder.2024.103265. Online ahead of print.NO ABSTRACTPMID:38552270 | DOI:10.1016/j.annder.2024.103265 (Source: Annales de Dermatologie et de Cenereologie)
Source: Annales de Dermatologie et de Cenereologie - March 29, 2024 Category: Dermatology Authors: M Hazard C Bedane O Ducharme P Bernard A-L Pham-Ledard Source Type: research

Evolution of rituximab use over time in moderate-to-severe pemphigus: A two-centre retrospective study
Ann Dermatol Venereol. 2024 Mar 28;151(2):103265. doi: 10.1016/j.annder.2024.103265. Online ahead of print.NO ABSTRACTPMID:38552270 | DOI:10.1016/j.annder.2024.103265 (Source: Annales de Dermatologie et de Venereologie)
Source: Annales de Dermatologie et de Venereologie - March 29, 2024 Category: Dermatology Authors: M Hazard C Bedane O Ducharme P Bernard A-L Pham-Ledard Source Type: research

Evolution of rituximab use over time in moderate-to-severe pemphigus: A two-centre retrospective study
Ann Dermatol Venereol. 2024 Mar 28;151(2):103265. doi: 10.1016/j.annder.2024.103265. Online ahead of print.NO ABSTRACTPMID:38552270 | DOI:10.1016/j.annder.2024.103265 (Source: Annales de Dermatologie et de Cenereologie)
Source: Annales de Dermatologie et de Cenereologie - March 29, 2024 Category: Dermatology Authors: M Hazard C Bedane O Ducharme P Bernard A-L Pham-Ledard Source Type: research

Maintenance treatment of pemphigus with rituximab in real life: A single-center study of 50 patients
CONCLUSION: Our study confirms the value of RTX maintenance therapy in pemphigus in real life.PMID:38537431 | DOI:10.1016/j.annder.2024.103264 (Source: Annales de Dermatologie et de Venereologie)
Source: Annales de Dermatologie et de Venereologie - March 27, 2024 Category: Dermatology Authors: A Louchez T El Aarbaoui A Weill O Gaudin L Jelti T Belmondo P Wolkenstein S Ingen-Housz-Oro Source Type: research

Maintenance treatment of pemphigus with rituximab in real life: A single-center study of 50 patients
CONCLUSION: Our study confirms the value of RTX maintenance therapy in pemphigus in real life.PMID:38537431 | DOI:10.1016/j.annder.2024.103264 (Source: Annales de Dermatologie et de Cenereologie)
Source: Annales de Dermatologie et de Cenereologie - March 27, 2024 Category: Dermatology Authors: A Louchez T El Aarbaoui A Weill O Gaudin L Jelti T Belmondo P Wolkenstein S Ingen-Housz-Oro Source Type: research

Maintenance treatment of pemphigus with rituximab in real life: A single-center study of 50 patients
CONCLUSION: Our study confirms the value of RTX maintenance therapy in pemphigus in real life.PMID:38537431 | DOI:10.1016/j.annder.2024.103264 (Source: Annales de Dermatologie et de Venereologie)
Source: Annales de Dermatologie et de Venereologie - March 27, 2024 Category: Dermatology Authors: A Louchez T El Aarbaoui A Weill O Gaudin L Jelti T Belmondo P Wolkenstein S Ingen-Housz-Oro Source Type: research

Single-cell transcriptome analysis reveals interaction between CCL19+ inflammatory keratinocytes and CCR7+ dendritic cells and B cells in pemphigus
Pemphigus is a complex autoimmune skin disorder, with an incidence of 0.6-32 per million annually (Kasperkiewicz et al., 2017). The central role of B cell dysfunction in pemphigus is established, but the contributions of other cells are still not well defined and are difficult to elucidate using conventional approaches. The availability of single-cell RNA sequencing (scRNA-seq) provides an excellent opportunity to explore these questions. (Source: Journal of Investigative Dermatology)
Source: Journal of Investigative Dermatology - March 25, 2024 Category: Dermatology Authors: Yiyi Wang, Shan Zhao, Xiwen Zhang, Dengmei Xia, Yue Xiao, Xingli Zhou, Tongying Zhan, Xuyang Xia, Yang Shu, Heng Xu, Wei Li Tags: Letters to the Editor Source Type: research

Beyond The Skin: B Cells in Pemphigus Vulgaris, Tolerance and Treatment
Br J Dermatol. 2024 Mar 20:ljae107. doi: 10.1093/bjd/ljae107. Online ahead of print.ABSTRACTPemphigus vulgaris is a rare autoimmune bullous disease characterized by blistering of the skin and mucosa owing to the presence of autoantibodies against the desmosome proteins desmoglein 3 and occasionally in conjunction with desmoglein 1. Fundamental research into the pathogenesis of PV has revolutionized its treatment and outcome with rituximab, a B-cell-depleting therapy. The critical contribution of B cells to the pathogenesis of pemphigus is well accepted. However, the exact pathomechanism, mechanisms of onset, disease course...
Source: The British Journal of Dermatology - March 20, 2024 Category: Dermatology Authors: Anne-Lise Strandmoe Jeroen Bremer Gilles F H Diercks Antoni Gosty ński Emanuele Ammatuna Hendri H Pas Marjan Wouthuyzen-Bakker Gerwin A Huls Peter Heeringa Jon D Laman Barbara Horv áth Source Type: research

Beyond The Skin: B Cells in Pemphigus Vulgaris, Tolerance and Treatment
Br J Dermatol. 2024 Mar 20:ljae107. doi: 10.1093/bjd/ljae107. Online ahead of print.ABSTRACTPemphigus vulgaris is a rare autoimmune bullous disease characterized by blistering of the skin and mucosa owing to the presence of autoantibodies against the desmosome proteins desmoglein 3 and occasionally in conjunction with desmoglein 1. Fundamental research into the pathogenesis of PV has revolutionized its treatment and outcome with rituximab, a B-cell-depleting therapy. The critical contribution of B cells to the pathogenesis of pemphigus is well accepted. However, the exact pathomechanism, mechanisms of onset, disease course...
Source: The British Journal of Dermatology - March 20, 2024 Category: Dermatology Authors: Anne-Lise Strandmoe Jeroen Bremer Gilles F H Diercks Antoni Gosty ński Emanuele Ammatuna Hendri H Pas Marjan Wouthuyzen-Bakker Gerwin A Huls Peter Heeringa Jon D Laman Barbara Horv áth Source Type: research

Regenerative potential of platelet concentrates in chronic oral mucosal lesions
J Oral Biol Craniofac Res. 2024 Mar-Apr;14(2):216-221. doi: 10.1016/j.jobcr.2024.02.007. Epub 2024 Mar 6.ABSTRACTChronic oral mucosal diseases (COMDs) represent a significant challenge for clinicians and patients. They are commonly associated with chronic pain and negative effects on healing and patient's quality of life. Regenerative medicine including the use of biological autologous blood-derived substances (e.g., platelet concentrates [PCs]), has been reported to improve healing and reduce pain in orthopedic and maxillofacial surgeries as well as chronic oral mucosal diseases. In this review, we aim to describe the dif...
Source: Cell Research - March 15, 2024 Category: Cytology Authors: Abdel-Hameed Hijazi Faez Saleh Al-Hamed Faleh Tamimi Sadeq A Al-Maweri Nader Hamdan David J Psutka Peter Ta Hagen Klieb Source Type: research

Efficacy and Safety of Rilzabrutinib in Pemphigus: PEGASUS Phase 3 Randomized Study
Pemphigus is a rare, but life-threatening autoimmune disease requiring long-term treatment that minimizes corticosteroid (CS) exposure while providing consistent disease control. The phase 2 pemphigus study of oral, reversible, covalent Bruton tyrosine kinase (BTK) inhibitor rilzabrutinib demonstrated rapid and sustained efficacy with well-tolerated safety. (Source: Journal of Investigative Dermatology)
Source: Journal of Investigative Dermatology - March 15, 2024 Category: Dermatology Authors: Dedee F. Murrell, Fr édéric Caux, Aikaterini Patsatsi, Owen Hagino, Lidia Rudnicka, Snejina Vassileva, Soner Uzun, Jenny Ye, Karl Yen, Puneet Arora, Steven G. Gourlay, Pascal Joly, Victoria P. Werth Tags: Original Article Source Type: research

Red crusty plaques in a young man
Dermatol Online J. 2023 Oct 15;29(5). doi: 10.5070/D329562411.ABSTRACTPemphigus foliaceus is a superficial blistering disorder characterized by erosions and scaling in a seborrheic distribution. The condition typically occurs in healthy individuals but issues arise from delayed diagnosis. Many cases remain undiagnosed or misdiagnosed due to the lack of awareness of the condition. With use of common diagnostic tools, pemphigus foliaceus can be easily identified and monitored. Histological analysis exhibits "chicken wire" patterning along keratinocytes in the upper epidermis, whereas immunofluorescence study displays subcorn...
Source: Dermatol Online J - March 13, 2024 Category: Dermatology Authors: Mishma Farsi Cassandra M Johnson Kelly Segars Jonathan Rhim Source Type: research