What are the types of systemic sclerosis?

Updated: Jun 24, 2020
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Systemic sclerosis is a complex and heterogeneous disease with clinical forms ranging from limited skin involvement (limited cutaneous systemic sclerosis) to forms with diffuse skin sclerosis and severe and often progressive internal organ involvement (diffuse cutaneous systemic sclerosis), and occasionally a fulminant course (fulminant systemic sclerosis).

Limited cutaneous systemic sclerosis involves areas distal to the elbows and knees but may involve the face and neck. CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias—although not all are needed for the disorder to be called CREST) is an older term used to describe this subset of limited cutaneous systemic sclerosis.

Diffuse cutaneous systemic sclerosis refers to skin thickening affecting the trunk and the skin of the extremities proximal to the elbows and knees besides involvement of the face. There are rare cases of typical systemic sclerosis internal organ involvement in the absence of clinically apparent cutaneous involvement, a clinical subset known as “scleroderma sine scleroderma”.

Systemic sclerosis involvement is most obvious in the skin; however, the gastrointestinal tract as well as the respiratory, renal, cardiovascular, musculoskeletal, endocrine, and genitourinary systems are frequently involved.

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