Sclerodema like disorder is/are caused by
High-Yield Explanation
Pathophysiology of Systemic sclerosis The cause of SScl is not completely understood. There is evidence for a genetic component and associations with alleles at the HLA locus have been found. The disease occurs in all ethnic groups and race may influence severity. Isolated cases have been repoed in which an SScl-like disease has been triggered by exposure to silica dust, vinyl chloride, epoxy resins and trichloroethylene. There is clear evidence of immunological dysfunction: T lymphocytes, especially those of the Th17 subtype, infiltrate the skin and there is abnormal fibroblast activation, leading to increased production of extracellular matrix in the dermis, primarily type I collagen. This results in symmetrical thickening, tightening and induration of the skin (scleroderma). Aerial and aeriolar narrowing occurs due to intimal proliferation and vessel wall inflammation. Endothelial injury causes release of vasoconstrictors and platelet activation, resulting in fuher ischaemia, which is thought to exacerbate the fibrotic process . Ref - Davidsons 23e p1037