Localized scleroderma: actual insights and new biomarkers

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 (miRNA-let-7a, miRNA-7, miRNA-196a, miRNA-155, miRNA-483-5p), 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.