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| ==Overview== | | ==Overview== |
| ==Classification== | | ==Classification== |
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| There are three major forms of scleroderma: diffuse, limited (''CREST syndrome'') and morphea/linear. Diffuse and limited scleroderma are both a [[systemic]] disease, whereas the linear/morphea form is localized to the skin. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.) There is also a subset of the systemic form known as "systemic scleroderma sine scleroderma", meaning the usual skin involvement is not present.
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| ===Diffuse scleroderma===
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| Diffuse scleroderma (progressive systemic sclerosis) is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the [[lung]]s and [[gastrointestinal tract]]), and is generally more life threatening.
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| ===Limited scleroderma/CREST syndrome===
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| The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.
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| In typical cases of limited scleroderma, [[Raynaud's phenomenon]] may precede scleroderma by several years. Raynaud's phenomenon is due to [[vasoconstriction]] of the small [[arteries]] of exposed peripheries - particularly the hands and feet - in the cold. It is classically characterised by a triphasic colour change - first white, then blue and finally red on rewarming. The scleroderma may be limited to the fingers - known as [[sclerodactyly]].
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| The limited form is often referred to as CREST [[syndrome]].<ref>{{cite journal |author=Winterbauer RH |title=Multiple telangiectasia, Raynaud'S phenomenon, sclerodactyly, and subcutanious calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia |journal=Bulletin of the Johns Hopkins Hospital |volume=114 |issue= |pages=361-83 |year=1964 |pmid=14171636 |doi=}}</ref> "CREST" is an acronym for the five main features:
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| * [[Calcinosis|'''C'''alcinosis]]
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| * [[Raynaud's phenomenon|'''R'''aynaud's syndrome]]
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| * [[Esophagus|'''E'''sophageal]] dysmotility
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| * [[Sclerodactyly|'''S'''clerodactyly]]
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| * [[Telangiectasia|'''T'''elangiectasia]]
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| ===Morphea/linear scleroderma===
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| [[Morphea]]/linear scleroderma involves isolated patches of hardened skin - there generally is no internal organ involvement.
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| ==References== | | ==References== |