Scleroderma classification
|
Scleroderma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Scleroderma classification On the Web |
|
American Roentgen Ray Society Images of Scleroderma classification |
|
Risk calculators and risk factors for Scleroderma classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Classification
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.
Diffuse scleroderma
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 lungs and gastrointestinal tract), and is generally more life threatening.
Limited scleroderma/CREST syndrome
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.
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.
The limited form is often referred to as CREST syndrome.[1] "CREST" is an acronym for the five main features:
Morphea/linear scleroderma
Morphea/linear scleroderma involves isolated patches of hardened skin - there generally is no internal organ involvement.