title: Overlapping gene expression profiles indicative of antigen processing and the interferon pathway characterize inflammatory fibrotic skin diseases.
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.
authors.affiliation: Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Annelies
authors.initials: A
authors.lastname: Limpers
authors.forename: Annet
authors.initials: A
authors.lastname: van Royen-Kerkhof
authors.forename: Joel A G
authors.initials: JA
authors.lastname: van Roon
authors.forename: Timothy R D J
authors.initials: TR
authors.lastname: Radstake
authors.forename: Jasper C A
authors.initials: JC
authors.lastname: Broen
mesh_terms: D000818:Animals; D017951:Antigen Presentation / Q000235:genetics; D004804:Eosinophils / Q000276:immunology*; D005355:Fibrosis; D006801:Humans; D007249:Inflammation / Q000235:genetics; D007372:Interferons / Q000276:immunology*; D046228:Microarray Analysis; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000235:genetics / Q000276:immunology / Q000378:metabolism*
chemical_list: D007372:Interferons