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Localised scleroderma (also referred to as morphea), unlike systemic scleroderma, is characterised by induration of the skin (sclerosis), resulting from fibrosis of the dermis and sometimes of the underlying tissues, with no known aetiology. Localised scleroderma is distinguished from systemic scleroderma by lack of sclerodactyly, Raynaud’s disease, microvascular lesions visible on capillaroscopy and lack of internal organ involvement (no gastrointestinal, pulmonary or pulmonary artery involvement) .
The incidence of localised scleroderma ranges from 0.4 to 2.7 per 100 000 people and it is more common in white female adults and children. The increase in cutaneous collagen and extracellular matrix components is thought to be caused by an autoimmune process. The process may be initiated by endothelial cell activation resulting in an increase in the expression of endothelial adhesion molecules (VCAM-1, ICAM-1 and E-selectin) and the recruitment of lymphocytes which produce profibrogenic (TGF-β, IL-4 and IL-6) and pro-inflammatory cytokines. TGF-β increases the production of collagen, fibronectin and proteoglycans by myofibroblasts, and decreases the production of proteases (MMP1 and MMP9) responsible for collagen degradation.
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