Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma. It is characterized by deposition of collagen in the skin and, less commonly, in the kidneys, heart, lungs & stomach. Female to male ratio is 4:1. The peak age of onset is between 30-50 years. Diffuse Scleroderma - affects the skin as well as the heart, lungs, GI tract, and kidneys. Limited Scleroderma - mostly affects the skin of the face, neck and distal elbows and knees and late in the disease causes isolated pulmonary hypertension. CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiectasias) is associated with limited scleroderma. In the skin, systemic sclerosis causes hardening and scarring. The skin may appear tight, reddish or scaly. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage may weaken limbs and affect appearance.