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Updated: May 4, 2026

Vasodilation of Isolated Vessels and the Isolation of the Extracellular Matrix of Tight-skin Mice
Published on: March 24, 2017
Overlapping gene expression profiles indicative of antigen processing and the interferon pathway characterize
Annelies Limpers1, Annet van Royen-Kerkhof, Joel A G van Roon
1Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
Abstract:
Inflammatory fibrotic disorders have been of high interest both for dermatologists and rheumatologists. Although the phenotypic end stage of this group of diseases is ultimately the same, namely fibrosis, patients present with different clinical features and are often treated with distinct therapeutic modalities. This review addresses whether there is evidence for different underlying molecular pathways in the various inflammatory fibrotic diseases such as localized scleroderma, pediatric lichen sclerosus, adult lichen sclerosus, eosinophilic fasciitis and systemic sclerosis. To investigate this, a large number of gene expression microarray studies performed on skin or fibroblasts from patients with these aforementioned diseases were described, (re-)analysed, and compared. As suspected by the heterogeneous phenotype, most diseases showed unique gene expression features. Intriguingly, a clear overlap was observed between adult and pediatric lichen sclerosus and localized scleroderma, in antigen processing and the interferon pathway. Delineating the cause and consequence of these pathways may generate novel tools to better characterize and more effectively treat these patients.
Insights
This review compares gene expression in inflammatory fibrotic disorders. While most diseases show unique molecular pathways, adult and pediatric lichen sclerosus, and localized scleroderma share similarities in antigen processing and interferon pathways.
Area of Science:
- Dermatology and Rheumatology
- Molecular Biology
- Genomics
Background:
- Inflammatory fibrotic disorders share a common endpoint (fibrosis) but present with diverse clinical features and treatments.
- Understanding distinct molecular pathways is crucial for targeted therapies in conditions like scleroderma and lichen sclerosus.
Purpose of the Study:
- To investigate and compare the underlying molecular pathways in various inflammatory fibrotic diseases.
- To determine if shared or unique gene expression profiles exist across conditions such as localized scleroderma, pediatric and adult lichen sclerosus, eosinophilic fasciitis, and systemic sclerosis.
Main Methods:
- A comprehensive analysis and comparison of numerous gene expression microarray studies.
- Utilized data from skin and fibroblast samples of patients diagnosed with the specified inflammatory fibrotic disorders.
Main Results:
- Most inflammatory fibrotic diseases exhibit unique gene expression signatures, reflecting their heterogeneous phenotypes.
- A significant overlap in gene expression was identified between adult lichen sclerosus, pediatric lichen sclerosus, and localized scleroderma, particularly concerning antigen processing and interferon pathways.
Conclusions:
- The findings suggest distinct molecular underpinnings for different inflammatory fibrotic diseases, despite clinical similarities.
- Identifying shared pathways, like those involving antigen processing and interferon signaling, offers potential for novel diagnostic and therapeutic strategies.
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