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Overview and classification of scleroderma disorders

Christopher P Denton, MD
Section Editor
John S Axford, DSc, MD, FRCP, FRCPCH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


The scleroderma disorders comprise a heterogeneous group of conditions linked by the presence of thickened, sclerotic skin lesions [1]. However, the other manifestations of these conditions are quite diverse. These differences have required the development of a classification system that takes into account the different potential complications, prognoses, and management strategies for patients with these disorders.

The simplest division of the scleroderma-related disorders is into localized and systemic forms of the disease (table 1 and table 2). The term systemic sclerosis (SSc) is more appropriate for the latter forms; it emphasizes that frequent involvement of internal organs is generally the most important manifestation of these conditions.

Disorders other than SSc can cause skin thickening. As examples, scleroderma-like skin changes can occur in some endocrine disorders (eg, diabetes mellitus and hypothyroidism), in end-stage renal disease (nephrogenic systemic fibrosis), and in conjunction with inflammatory (eg, eosinophilic fasciitis) and infiltrative (eg, amyloid) disorders. Sclerodermatous skin changes may occur as a result of exposure to certain drugs, toxins, and environmental exposures. These and other causes of sclerodermatous skin changes or non-sclerodermatous skin thickening are discussed separately. (See "Diagnosis and differential diagnosis of systemic sclerosis (scleroderma) in adults", section on 'Differential diagnosis'.)

This topic will provide an overview of the classification schema of scleroderma. Classification systems that have been developed for diagnostic and research purposes are also discussed.


Localized scleroderma can be divided into linear scleroderma (en coup de sabre) and localized and generalized morpheae (table 1). (See "Localized scleroderma in childhood".)


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Literature review current through: Sep 2016. | This topic last updated: Jan 9, 2015.
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