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Biomolecules
Special Issues
Molecular Insights into Treatment and Prognosis of Autoimmune Bullous Diseases
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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: closed (15 March 2026) | Viewed by 3546

Special Issue Editors

Dr. Kristina Seiffert-Sinha

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
Dr. Animesh A. Sinha

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

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 250 words) can be sent to the Editorial Office for assessment.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomolecules is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

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

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Published Papers (3 papers)

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Research

20 pages, 10045 KB  
Article
Complement Activation May Drive the Pathogenicity of Anti-α6 and Anti-β4 Integrin Antibodies In Vivo
by Gefei Du, Shirin Emtenani, Dennis Niese, Jian Liu, Ferdinand Gebauer, Neele J. Dunst, Aysun Gökce, Kristina Spaniol, Florian Groeber-Becker, Jelena Šimunović, Mislav Novokmet, Gerd Geerling, Kyle T. Amber, Markus H. Hoffmann, Ralf J. Ludwig, Katja Bieber, Stephanie Goletz, Gang Zhou, Enno Schmidt and Sabrina Patzelt
Biomolecules 2026, 16(3), 417; https://doi.org/10.3390/biom16030417 - 12 Mar 2026
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Abstract
Autoantibodies targeting α6β4 integrin have been identified in individual patients with mucous membrane pemphigoid (MMP). Reactivity against α6 integrin has been associated with oral lesions, while anti-β4 integrin reactivity has been linked to ocular involvement. However, the pathogenic effects of these antibodies have [...] Read more.
Autoantibodies targeting α6β4 integrin have been identified in individual patients with mucous membrane pemphigoid (MMP). Reactivity against α6 integrin has been associated with oral lesions, while anti-β4 integrin reactivity has been linked to ocular involvement. However, the pathogenic effects of these antibodies have not been fully elucidated. Here, we investigated the pathogenic potential of anti-α6 and anti-β4 integrin IgG both in vitro and in vivo. Immune complexes of anti-α6 and anti-β4 integrin induced the release of reactive oxygen species from normal human leukocytes and stimulated CXCL2 secretion in cultured murine C5N keratinocytes. In vivo, repeated injections of IgG against a recombinant fragment of β4 integrin into C57BL/6 mice led to palpebral conjunctival swelling and mild oral lesions. The latter was observed following injection of IgG against a recombinant fragment of α6 integrin. Histopathological analysis revealed subepithelial inflammatory infiltrates without evidence of split formation. Direct immunofluorescence microscopy showed linear deposits of IgG at the basement membrane zone in most tissues, whereas C3 deposition was largely absent. This lack of complement activation was corroborated by a complement fixation assay, which confirmed that IgG against α6 and β4 integrin failed to induce C3 deposition in normal murine conjunctivae, buccal mucosa, or skin. Collectively, these findings indicate that IgG autoantibodies against α6 and β4 integrin exhibit pathogenic activity in vitro and induce mild disease in vivo, possibly due in part to relatively inefficient complement activation in this model. Full article
(This article belongs to the Special Issue Molecular Insights into Treatment and Prognosis of Autoimmune Bullous Diseases)
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14 pages, 3704 KB  
Article
Selective Complement Inhibition in Anti-p200 Pemphigoid: Immune Infiltrate Profiles and Therapeutic Implications Compared to Bullous Pemphigoid
by Shirin Emtenani, Tina Rastegar Lari, Charlotte Kiehne, Nina van Beek, Maike M. Holtsche and Enno Schmidt
Biomolecules 2026, 16(2), 182; https://doi.org/10.3390/biom16020182 - 23 Jan 2026
Viewed by 736
Abstract
Anti-p200 pemphigoid is an autoimmune blistering disease (AIBD) caused by autoantibodies against laminin β4 and/or γ1, and clinically resembles bullous pemphigoid (BP) as well as the inflammatory variant of epidermolysis bullosa acquisita (EBA). All three diseases show IgG and/or C3 deposition along the [...] Read more.
Anti-p200 pemphigoid is an autoimmune blistering disease (AIBD) caused by autoantibodies against laminin β4 and/or γ1, and clinically resembles bullous pemphigoid (BP) as well as the inflammatory variant of epidermolysis bullosa acquisita (EBA). All three diseases show IgG and/or C3 deposition along the cutaneous basement membrane zone (BMZ). Although complement activation is central to BP and EBA pathogenesis, its role in anti-p200 pemphigoid remains unclear. To investigate this, we analyzed inflammatory infiltrates in lesional and perilesional skin from anti-p200 pemphigoid patients (n = 11), revealing a neutrophil-predominant pattern, with mixed neutrophil–eosinophil infiltrates in 81% of cases, which contrasted with the eosinophil-rich infiltrates typical of BP. Infiltrating neutrophils expressed C5aR1 and C5aR2. Complement fixation test (CFT) of patient sera demonstrated C3c deposition at the BMZ in 40% (20/50) of anti-p200 pemphigoid cases and 87% (13/15) of BP cases. Patients in both cohorts could be stratified into high, mild, and non-complement-fixating groups. Pharmacological inhibition of C1s (sutimlimab), C3 (compstatin), C5 (tesidolumab), or C5aR1 (avacopan) significantly blocked C3c or C5 deposition in vitro. These findings indicate that selective blockade of the classical, alternative, or terminal complement pathways effectively prevents BMZ complement deposition, highlighting pathway-specific complement inhibition as a potential therapeutic strategy for anti-p200 pemphigoid. Full article
(This article belongs to the Special Issue Molecular Insights into Treatment and Prognosis of Autoimmune Bullous Diseases)
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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 1321
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
(This article belongs to the Special Issue Molecular Insights into Treatment and Prognosis of Autoimmune Bullous Diseases)
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