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. 06/02/09.
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)
Scleroderma-clinical and pathological advances. Skin sclerosis is no longer regarded as mandatory for the diagnosis of systemic sclerosis. It is likely that clinical trials performed over the next few years will transform the management of systemic sclerosis and help to dispel its reputation as one of the least treatable of the autoimmune rheumatic diseases. PubMed. Best Pract Res Clin Rheumatol. 2004 Jun;18(3):271-90. (Also see: Diffuse Scleroderma and Prof. Carol Black)
Scleroderma Care and Research Journal (PDF) This inaugural issue for physicians focuses on elevating the standards of care for scleroderma lung involvement. Articles include Interstitial Lung Disease in Systemic Sclerosis: Optimizing Evaluation and Management, as well as Pulmonary Hypertension Related to Systemic Sclerosis: A Primer for the Rheumatologist.Journal of the Scleroderma Clinical Trials Consortium (SCTC) Vol 1, No. 1, Autumn 2003 (Also see: Pulmonary Hypertension)
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