1. 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. See Also: CREST syndrome.
2.
Syn: scleroderma.
3. CREST
SYNDROME: a variant of systemic sclerosis characterized by calcinosis,
Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia.
