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Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease characterised by hardening (''sclero'') of the skin (''derma''). In the more severe form, it also affects internal organs. Limited scleroderma involves cutaneous manifestations that mainly affect the hands, arms and face. It was previously called CREST syndrome in reference to 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. The prognosis is generally good for limited cutaneous scleroderma persons who escape lung complications, but is worse for those with the diffuse cutaneous disease, particularly in older age and for 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%. The cause of scleroderma is unknown.〔 It is an autoimmune condition, in which the body's immune system attacks healthy tissues.〔 Strong associations with certain mutations in HLA genes have been identified. Strong environmental influences have also been implicated in the etiology of scleroderma. == Signs and symptoms == Potential signs and symptoms include:〔〔〔 * Cardiovascular: Raynaud's phenomenon (is the presenting symptom in 70% of affected persons, occurs in 95% of affected individuals at some time during their illness); healed pitting ulcers on the fingertips; skin and mucousal telangiectasis; palpitations, irregular heart rate and fainting due to conduction abnormalities, hypertension and congestive heart failure. * Digestive: gastroesophageal reflux disease, bloating, indigestion, loss of appetite, diarrhoea alternating with constipation, sicca syndrome and its complications, loosening of teeth and hoarseness (due to acid reflux). * Pulmonary: progressive worsening of shortness of breath, chest pain (due to pulmonary artery hypertension) and dry, persistent cough due to interstitial lung disease. * Musculoskeletal: joint, muscle aches, loss of joint range of motion, carpal tunnel syndrome and muscle weakness. * Genitourinary: erectile dysfunction, dyspareunia, scleroderma renal crises and kidney failure. * Other: facial pain due to trigeminal neuralgia, hand paraesthesias, headache, stroke, fatigue, calcinosis and weight loss. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Scleroderma」の詳細全文を読む スポンサード リンク
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