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Pathogenesis and New Therapeutic Approaches for Fibrosing Diseases—the Skin as a Model System

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: closed (15 October 2023) | Viewed by 4211

Special Issue Editors

Prof. Dr. Thomas Krieg

E-Mail Website
Guest Editor
Department for Dermatology and Venerology, University of Cologne, 50923 Koln, Germany
Interests: skin sclerosing diseases; skin fibrosis; degenerative skin changes; wound healing
Prof. Dr. Armando Gabrielli

E-Mail Website
Guest Editor
Fondazione di Medicina Molecolare e Terapia Cellulare, Università Politecnica delle Marche, 60126 Ancona, Italy
Interests: systemic sclerosis; scleroderma; autoimmune diseases; autoimmunity

Special Issue Information

Dear Colleagues,

The skin represents an exceptional suitable system to follow disease development and to study molecular and cellular mechanisms. It has been demonstrated that data obtained in the skin reflect general mechanisms also acting in other organs.

In this Special Issue, we concentrate on fibrosing skin diseases, describe their heterogeneity, and discuss novel therapeutic approaches generated by a better understanding of pathogenetic mechanisms. Most of these diseases are due to early vascular damage followed by an inflammatory reaction, which finally leads to fibrosis. There have been many important developments addressing these concepts, which lead to new therapies of organ complications in these diseases, which have created major implications for the quality of life of the patients.

Although some information stems from the experimental work carried out in recent years on systemic sclerosis, we have nonetheless been careful to also highlight wider implications, especially for collateral diseases in which fibrosis also represents a significant phenomenon.

Since IJMS is a journal of molecular science, purely clinical studies will not be suitable for our journal. Clinical submissions with biomolecular experiments, however, are welcomed.

Prof. Dr. Thomas Krieg
Prof. Dr. Armando Gabrielli
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 100 words) can be sent to the Editorial Office for announcement on this website.

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. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. 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

  • systemic sclerosis

  • scleroderma
  • morphea
  • fibrosis
  • pathogenesis
  • treatment
  • vasculopathy
  • collagen
  • experimental animals
  • molecular mechanisms

Published Papers (2 papers)

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Research

16 pages, 2007 KiB  
Article
A Positive Feedback Loop Exists between Estradiol and IL-6 and Contributes to Dermal Fibrosis
by DeAnna Baker Frost, Alisa Savchenko, Naoko Takamura, Bethany Wolf, Roselyn Fierkens, Kimberly King and Carol Feghali-Bostwick
Int. J. Mol. Sci. 2024, 25(13), 7227; https://doi.org/10.3390/ijms25137227 - 30 Jun 2024
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Abstract
Systemic sclerosis (SSc) is characterized by dermal fibrosis with a female predominance, suggesting a hormonal influence. Patients with SSc have elevated interleukin (IL)-6 levels, and post-menopausal women and older men also have high estradiol (E2) levels. In the skin, IL-6 increases the enzymatic [...] Read more.
Systemic sclerosis (SSc) is characterized by dermal fibrosis with a female predominance, suggesting a hormonal influence. Patients with SSc have elevated interleukin (IL)-6 levels, and post-menopausal women and older men also have high estradiol (E2) levels. In the skin, IL-6 increases the enzymatic activity of aromatase, thereby amplifying the conversion of testosterone to E2. Therefore, we hypothesized that an interplay between E2 and IL-6 contributes to dermal fibrosis. We used primary dermal fibroblasts from healthy donors and patients with diffuse cutaneous (dc)SSc, and healthy donor skin tissues stimulated with recombinant IL-6 and its soluble receptor (sIL-6R) or E2. Primary human dermal fibroblasts and tissues from healthy donors stimulated with IL-6+sIL-6R produced E2, while E2-stimulated dermal tissues and fibroblasts produced IL-6. Primary dermal fibroblasts from healthy donors treated with IL-6+sIL-6R and the aromatase inhibitor anastrozole (ANA) and dcSSc fibroblasts treated with ANA produced less fibronectin (FN), type III collagen A1 (Col IIIA1), and type V collagen A1 (Col VA1). Finally, dcSSc dermal fibroblasts treated with the estrogen receptor inhibitor fulvestrant also generated less FN, Col IIIA1, and Col VA1. Our data show that IL-6 exerts its pro-fibrotic influence in human skin in part through E2 and establish a positive feedback loop between E2 and IL-6. Full article
(This article belongs to the Special Issue Pathogenesis and New Therapeutic Approaches for Fibrosing Diseases—the Skin as a Model System)
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18 pages, 4675 KiB  
Article
Single-Cell Transcriptome Analysis Identifies Subclusters with Inflammatory Fibroblast Responses in Localized Scleroderma
by Giffin Werner, Anwesha Sanyal, Emily Mirizio, Theresa Hutchins, Tracy Tabib, Robert Lafyatis, Heidi Jacobe and Kathryn S. Torok
Int. J. Mol. Sci. 2023, 24(12), 9796; https://doi.org/10.3390/ijms24129796 - 6 Jun 2023
Cited by 10 | Viewed by 3324
Abstract
Localized scleroderma (LS) is an autoimmune disease with both inflammatory and fibrotic components causing an abnormal deposition of collagen in the skin and underlying tissue, often leading to disfigurement and disability. Much of its pathophysiology is extrapolated from systemic sclerosis (SSc) since the [...] Read more.
Localized scleroderma (LS) is an autoimmune disease with both inflammatory and fibrotic components causing an abnormal deposition of collagen in the skin and underlying tissue, often leading to disfigurement and disability. Much of its pathophysiology is extrapolated from systemic sclerosis (SSc) since the histopathology findings in the skin are nearly identical. However, LS is critically understudied. Single-cell RNA sequencing (scRNA seq) technology provides a novel way to obtain detailed information at the individual cellular level, overcoming this barrier. Here, we analyzed the affected skin of 14 patients with LS (pediatric and adult) and 14 healthy controls. Fibroblast populations were the focus, since they are the main drivers of fibrosis in SSc. We identified 12 fibroblast subclusters in LS, which overall had an inflammatory gene expression (IFN and HLA-associated genes). A myofibroblast-like cluster (SFRP4/PRSS23) was more prevalent in LS subjects and shared many upregulated genes expressed in SSc-associated myofibroblasts, though it also had strong expression of CXCL9/10/11, known CXCR3 ligands. A CXCL2/IRF1 cluster identified was unique to LS, with a robust inflammatory gene signature, including IL-6, and according to cell communication analysis are influenced by macrophages. In summary, potential disease-propagating fibroblasts and associated gene signatures were identified in LS skin via scRNA seq. Full article
(This article belongs to the Special Issue Pathogenesis and New Therapeutic Approaches for Fibrosing Diseases—the Skin as a Model System)
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