Systemic sclerosis (SSc) is an uncommon connective tissue disorder characterised by multisystem involvement, heterogeneous clinical manifestations, a chronic and often progressive course, and significant disability and mortality. Patients with SSc can be broadly grouped into diffuse cutaneous (dcSSc) and limited cutaneous (lcSSc) subsets defined by the pattern of skin involvement, as well as clinical and laboratory features. The constellation of calcinosis cutis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, seen in some lcSSc patients, is termed the CREST syndrome. In some patients, Raynaud’s phenomenon and other characteristic features of SSc occur in absence of skin thickening. This syndrome has been termed SSc sine scleroderma.

Keywords: Raynaud’s phenomenon, sclerodactyly, CREST syndrome, lcSSC, dcSSC, SSc sine scleroderma.

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Divya Manikanta Swaroop Palla