Scleroderma is a chronic autoimmune disease that involves the skin and connective tissue. There are two types, localized and systemic. In the localized type, the skin shows one or more patches of sclerosis (thickening and hardening). The systemic type involves the skin and the connective tissue. This article deals with the systemic forms of scleroderma.
Systemic forms of scleroderma include progressive systemic sclerosis (PSS), also known as systemic sclerosis (SS), and the CREST syndrome. Systemic scleroderma involves body systems such as the esophagus, intestines, lungs, heart and kidneys.
Diffuse scleroderma is a term which describes systemic sclerosis and skin changes on many parts of the body. Tight, glossy skin may be present on the trunk and upper arms as well as on the face, chest and extremities.
The CREST syndrome is an acronym made up of the first letters of the five most prominent manifestations of this form of scleroderma. They are: calcinosis (accumulation of calcium salts under the skin), Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly and telangiectasia. Systemic scleroderma can occur in both men and women, and it typically affects women in their thirties and forties. Each case is different, and the severity of the disease varies among patients.
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