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systemic sclerosis

 

Definition: a systemic disease characterized by formation of hyalinized and thickened collagenous fibrous tissue, with thickening of the skin and adhesion to underlying tissues (especially of the hands and face), dysphagia due to loss of peristalsis and submucosal fibrosis of the esophagus, dyspnea due to pulmonary fibrosis, myocardial fibrosis, and renal vascular changes resembling those of malignant hypertension; Raynaud phenomenon, atrophy of the soft tissues, and osteoporosis of the distal phalanges (acrosclerosis), sometimes with gangrene at the ends of the digits, are common findings. The term progressive systemic sclerosis is commonly used and is appropriate for cases with initially widespread skin involvement including the trunk. However, when skin involvement is limited to the distal extremities and face, there is often prolonged delay in appearance of visceral manifestations.

Synonym(s): scleroderma

See Also: CREST syndrome

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© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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