According to the Scleroderma Foundation, scleroderma is a chronic systemic autoimmune disease characterized by hardening (“sclero”) of the skin (“derma”), although it may also affect internal organs. There is currently no cure for scleroderma, but effective treatments for some forms of the disease and its wide array of symptoms are available.
The six types of scleroderma can be separated into two major classifications: localized scleroderma and systemic sclerosis (SSc). However, patients can have different symptoms and different combinations of illness.
Localized scleroderma is characterized by inflammation and thickening of the skin from excessive collagen deposition. Relatively mild, only a few areas of the body are affected — usually the skin or muscles. Localized scleroderma rarely becomes systemic, Internal organs are typically not affected.
Classified as a form of localized scleroderma, morphea usually involves isolated patches of hardened skin of varying sizes, shapes, and color that can disappear and come back at any time. There is no internal organ involvement.
This kind of localized scleroderma is usually characterized by a line of thickened skin that may affect the bones and muscles underneath it. It appears most commonly on the arms, legs, or forehead, although it is possible for linear scleroderma to occur in other areas. Usually appearing in childhood, linear scleroderma may affect the growth of limbs.
Systemic scleroderma (systemic sclerosis)
Systemic sclerosis (SSc) is a multi-system autoimmune disease that results in fibrosis (development of scar tissue), vascular damage, and abnormal connective tissue growth in many parts of the body including muscles and joints. These abnormalities lead to the breakdown of the skin, subcutaneous tissue, muscles, and internal organs such as the heart, lungs, kidneys, and intestinal tract.
In diffuse scleroderma, more areas of the skin becomes thickened. Skin on the arms, legs, and trunk are more likely to be involved. Diffuse scleroderma patients have a higher risk of developing “sclerosis” or fibrous hardening of the internal organs. In this case, skin thickening occurs rapidly.
About half of all scleroderma patients have this slower developing and less harmful form of scleroderma. The skin thickening is less widespread and typically confined to the fingers, hands, and face. In some cases, limited scleroderma can also affect the digestive tract, lungs, or heart. Usually, it develops slowly over years. Limited scleroderma is also known as CREST syndrome. “CREST” is an acronym for the following conditions that occur in limited sclerosis: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and elangiectasia
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