Diffuse systemic sclerosis

**Definition:** Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of Systemic Sclerosis (SSc) characterised by truncal and acral skin fibrosis with an early and significant incidence of diffuse involvement (interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myocardial involvement).

**Long definition:** Systemic sclerosis (SSc) is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs. SSc is associated with prominent autoimmune and inflammatory features and altered vascular function. Diffuse cutaneous SSc is associated with progressive skin induration, starting in the fingers and ascending from distal to proximal extremities, the face, and the trunk. These patients are at risk for early pulmonary fibrosis and acute renal involvement. Most patients also manifest characteristic abnormalities in gastrointestinal tract and heart.