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Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults

John Varga, MD
Section Editor
John S Axford, DSc, MD, FRCP, FRCPCH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


The term scleroderma is used to describe the presence of thickened, hardened skin (from the Greek “scleros”) [1]. Scleroderma is the hallmark feature of a heterogeneous group of conditions, the classification of which is discussed in more detail separately. (See "Overview and classification of scleroderma disorders".)

Scleroderma may be a clinical feature of limited anatomic extent affecting only the skin and subjacent tissues, or it may be associated with systemic involvement. When the characteristic skin disorder is associated with internal organ involvement, the disease is termed systemic sclerosis (SSc). SSc is subcategorized further into diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) on the basis of the extent and distribution of skin involvement. lcSSc is commonly associated with the CREST syndrome [2]. (See 'Limited cutaneous SSc' below.)

Other disorders that may either have typical scleroderma skin changes or those that have scleroderma-like cutaneous involvement are considerations in the differential diagnosis of SSc. (See "Diagnosis and differential diagnosis of systemic sclerosis (scleroderma) in adults".)

The systemic manifestations of SSc are diverse. Most prominent are abnormalities of the circulation (most notably Raynaud phenomenon) and involvement of multiple organ systems, including the musculoskeletal, renal, pulmonary, cardiac, and gastrointestinal systems, with fibrotic and/or vascular complications.

The prevalence rates of scleroderma-like conditions range from 4 to 489 cases per million individuals [3,4]. Incidence figures for SSc are 0.6 to 122 per million persons per year; the actual prevalence is probably at the high end of the range noted above [4]. There are regional differences in incidence. For example, higher rates are seen in the United States and Australia than in Japan or Europe, and in blacks than whites [4].

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Literature review current through: Nov 2017. | This topic last updated: Jan 06, 2017.
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