CREST Syndrome does not necessarily have any skin tightening at all. The CREST term has fallen into disuse by experts, who were frustrated because many doctors were just diagnosing CREST and failing to identify further, whether it was Limited or Diffuse Scleroderma.
Limited means that the skin involvement is limited to the hands (although the face and neck may also be involved).
Diffuse is when skin tightening also occurs above the wrists (or elbows, see below).
Systemic Sclerosis sine Scleroderma is when there is internal organ fibrosis, but no skin tightening.
Limited Scleroderma (CREST Syndrome). For some people, the problems caused by limited scleroderma are minor. For others, limited scleroderma can be life altering and even fatal. Mayo Clinic.
Diagnosis of Limited Scleroderma
Limited Scleroderma is diagnosed when there is tight skin limited to the fingers, along with either pitting digital ulcers (secondary to Raynaud's) and/or lung fibrosis. This is considered to be a "milder" form of Scleroderma. The skin of the face and neck may also be involved in Limited Scleroderma.
Limited Scleroderma often causes Raynaud's and esophageal problems. Occasionally other internal organ involvement occurs, but usually only after many years of the disease. The onset and progression of Limited Scleroderma is usually very slow, and the outlook in general is very good. (1)
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