Localized scleroderma: actual insights and new biomarkers

Abstract

Localized scleroderma (LS, morphea, limited scleroderma, focal scleroderma) is a chronic autoimmune disease characterized by a progressive damage to the connective tissue with a predominance of fibrosclerotic disorders in the skin and the subcutaneous tissue. In addition surrounding structures may be affected: fascia, muscle, and bone tissues. This review reflects the current understanding about limited scleroderma, its pathogenesis, diagnosis, new biomarkers, and information about the possibilities of its transition to systemic scleroderma. The following new biomarkers have been identified: galactosylated IgG (Ig-Gal), progranulin (PGRN), chemokine CCXL 18, various types of microRNA , periostin, and myelin basic protein (MBP). Knowledge about new biomarkers of LS will help us to explore the patients' predisposition to the development of systemic scleroderma. In addition, by acting on these biomarkers, it is possible to prevent the progression of LS in the early stages and its transition to systemic scleroderma. The review also presents the current understanding of autoantibodies in LS and their correlation with clinical signs of the disease.

Limited Cutaneous Scleroderma: A Case Report

“…The disease typically affects the skin's outermost layers, which harden and thicken [ 9 ]. LoS can result in significant impairment due to joint contractures and subcutaneous atrophy, although never being lethal [ 10 ]. Extracutaneous involvements in patients with LoS are described as including Raynaud's phenomenon, localised hair loss at the affected site, ocular involvement, neurological involvement, gastroesophageal reflux disease (GERD), esophagitis (documented by upper gastrointestinal endoscopy), abnormal pulmonary function testing (PFP), restrictive lung disease, cough or dyspnea, and fascia or muscle involvement.…”

Differentiating Activity From Damage—The Morphological Challenge of Morphea

“…While some imaging modalities have been validated, they are expensive, inefficient, highly specialized, and not readily available. Biomarkers such as serum chemokine ligand 9, chemokine ligand 18, and interferon γ–inducible protein 10 have been recently highlighted in a gene expression study . However, no routinely available laboratory tests reliably reflect disease activity.…”

Biomarkers in skin autoimmunity—An update on localised scleroderma

“…6,29 The main objective of this review is to give an update on relevant biomarkers for diagnosing and studying LS. In addition to previously reviewed biomarkers, 30 we also include genomic studies, cell surface and endothelial biomarkers, and give a brief overview of upcoming clinical trials that aim at genetic studies of LS.…”