Molecular Insights into Treatment and Prognosis of Autoimmune Bullous Diseases

A special issue of Biomolecules (ISSN 2218-273X). This special issue belongs to the section "Molecular Medicine".

Deadline for manuscript submissions: 15 March 2026 | Viewed by 1083

Special Issue Editors


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Guest Editor
Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA
Interests: autoimmunity; blistering; gene expression; immunome; keratinocyte; pathomechanism; proteome; skin

E-Mail Website
Guest Editor
Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA
Interests: autoimmunity; blistering; gene expression; immunome; keratinocyte; pathomechanism; proteome; skin

Special Issue Information

Dear Colleagues,

Autoimmune bullous diseases are a group of skin disorders that are thought to result from an autoantibody attack against intercellular adhesion molecules or components of the basement membrane in the skin as well as mucosal surfaces. While they are clinically and immunopathologically heterogenous, they are all associated with a high degree of morbidity and occasional mortality both from untreated disease in itself as well as mainly from their immunosuppressive treatment. In light of the success of anti-CD20 therapy, there has been a recent surge of clinical trials in this field with an aim of reducing autoantibodies, but outcomes have been underwhelming thus far, highlighting the need to focus again on the basic mechanisms of these diseases on the cellular and molecular levels.

The aim of this Special Issue is to delve into the cellular and molecular aspects of autoimmune bullous diseases and to link genomic and proteomic data with clinical outcomes. We are interested in in vitro, in vivo, and pre-clinical studies from genetics and gene expression, cellular immune responses (both in the peripheral blood as well as in target tissues), proteomics to the mechanisms of acantholysis or the loss of keratinocyte cell adhesion. We also encourage reviews that summarize the state of the art in disease pathogenesis and mechanisms of target damage.

We look forward to receiving your contributions.

Dr. Kristina Seiffert-Sinha
Dr. Animesh A. Sinha
Guest Editors

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Keywords

  • autoimmunity
  • blistering
  • gene expression
  • immunome
  • keratinocyte
  • pathomechanism
  • proteome
  • skin

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Published Papers (1 paper)

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Research

13 pages, 31335 KB  
Article
Bullous Pemphigoid Develops Independently of DAP12
by Manuela Pigors, Sabrina Patzelt, Maëlys Brudey, Shirin Emtenani, Stanislav Khil’chenko, Mayumi Kamaguchi, Niklas Reichhelm, Melissa Parker, Katja Bieber, Ralf J. Ludwig and Enno Schmidt
Biomolecules 2025, 15(11), 1549; https://doi.org/10.3390/biom15111549 - 5 Nov 2025
Viewed by 516
Abstract
The adaptor molecule DNAX-activating protein of 12 kDa (DAP12) is broadly expressed in innate immune cells, but its role in autoimmunity remains unclear due to its dual regulatory functions. We investigated the contribution of the DAP12 pathway to bullous pemphigoid (BP), the most [...] Read more.
The adaptor molecule DNAX-activating protein of 12 kDa (DAP12) is broadly expressed in innate immune cells, but its role in autoimmunity remains unclear due to its dual regulatory functions. We investigated the contribution of the DAP12 pathway to bullous pemphigoid (BP), the most common autoimmune blistering disease, using a mouse model induced by transfer of anti-type XVII collagen (Col17) IgG. Repeated anti-Col17 IgG injections over 12 days produced comparable disease activity in DAP12-deficient and wildtype mice (n = 17/group), indicating that disease induction occurs independently of DAP12 signaling. Flow cytometry and immunofluorescence analysis of lesional skin further revealed a strong upregulation of the DAP12-associated triggering receptors expressed on myeloid cells (TREM) 1 in wildtype BP lesions, whereas TREM2+ cell frequencies in anti-Col17 IgG-treated wildtype and DAP12 knock-out animals were significantly lower than in healthy controls. Additional flow cytometry analysis demonstrated altered inflammatory infiltrates with notably reduced frequencies of Siglec-f+ eosinophils in DAP12-deficient vs. wildtype lesional skin. In addition, pharmacological inhibition of PI3Kδ, a downstream kinase of the DAP12/TREM pathway, did not affect disease progression in anti-Col17 IgG-induced BP. Collectively, these findings indicate that while DAP12 signaling modulates local immune cell composition, the DAP12/TREM1/2-axis does not influence overall disease activity in experimental BP. Full article
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