Scleroderma, also known as systemic sclerosis, is a long term autoimmune disease that results in hardening of the skin. In the more severe form, it also affects internal organs. The cause is unknown. The underlying mechanism involves the body's immune system attacking healthy tissues. There is a strong associations with certain mutations in HLA genes. Environmental factors have also been implicated. Limited scleroderma involves mainly the skin of the hands, arms and face. It was previously called CREST syndrome an acronym of the following common manifestations: calcinosis (the deposition of calcium nodules in the skin), raynaud's phenomenon (exaggerated vasoconstriction in the hands, with fingers undergoing white-blue-red color transitions in the cold), esophageal dysfunction (leading to difficulty swallowing), sclerodactyly (skin thickening on the fingers), and telangiectasias (dilated capillaries on the face, hands and mucous membranes). Diffuse scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart and/or lungs. This form of scleroderma can be quite disabling. There are no treatments for scleroderma itself, but individual organ system complications are treated. Outcomes are generally good for limited scleroderma of the skin when their are no lung complications. They are worse for those with the diffuse skin disease, particularly in older age and males. Death occurs most often from lung, heart and kidney complications. In diffuse cutaneous disease, five-year survival is 70% and 10-year survival is 55%. Scleroderma was first described in 1753 by Carlo Curzio of Ospedale degli Incurabili, Naples.

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