An uncommon case of diffuse cutaneous systemic sclerosis

Systemic sclerosis (SSc) is a chronic connective tissue disorder of immune dysregulation characterised by widespread fibrosis of skin and internal organs, small vessel vasculopathy, and presence of intracellular autoantibodies. It is rare disorder with worldwide distribution sparing no ethnic group.
It has marked clinical heterogeneity in its presentation and severity. Systemic sclerosis is classified as limited cutaneous systemic sclerosis (lcSSc), diffuse cutaneous systemic sclerosis (dcSSc) and Systemic sclerosis sine scleroderma (SSS), a rare type without skin involvement. dcSSc has the worst prognosis because of internal organ involvement. There is no definitive therapy available to arrest or reverse the progression of fibrosis. Management is essentially symptomatic. Various immunosuppressive agents have been used in a bid to reduce the fibrosis and improve organ function.
We report a case of dcSSc who had signs of widespread dermal involvement along with distinct facial features and characteristic scleroderma biomarkers. Investigations have ruled out any definitive systemic involvement. The management has been as per the EULAR/ACR guidelines. The case is being reported for its rarity in general population and also to highlight the significance of mutually exclusive autoantibodies in classifying subsets of the disease.