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02.06.2014
Scleroderma

Definition

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Definition

Scleroderma, also known as systemic sclerosis is a chronic systemic autoimmune disease, that primarily affects the skin (hence the suffix, "derma"), is characterised by sclerosis (hence the prefix, "sclero"), that is hardening of the skin. 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, oesophagus, 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.

 

 

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