| progressive familial scleroderma | A syndrome characterised by calcinosis cutis, Raynaud's phenomenon, sclerodactyly, and telangiectasia; usually due to scleroderma; autosomal dominant form of progressive systemic sclerosis. (05 Mar 2000) |
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| scleroderma | <dermatology> Hardening of skin. (04 Mar 1998) |
| scleroderma, circumscribed | A chronic, localised hardening and thickening of the skin. Lesions may be categorised as morphea (guttate, profunda, pansclerotic) or linear (with or without melorheostosis or hemiatrophy). It is twice as common in women as in men. The condition is characterised by skin ischemia, lymphocytic infiltrates, swollen collagen bundles, and thickening of the dermis with reduction of subcutaneous fat. (12 Dec 1998) |
| scleroderma, systemic | A chronic, progressive dermatosis characterised by boardlike hardening and immobility of the affected skin, with visceral involvement, especially of lungs, oesophagus, kidneys and heart. It may be accompanied by calcinosis, raynaud's phenomenon, and telangiectasis (crest syndrome). It includes acrosclerosis and sclerodactyly. (12 Dec 1998) |
| oesophagus: scleroderma | <radiology> Females (80%), 35-55 years of age, decreased LES pressure, decreased peristalsis, smooth muscle atrophy with or without loose fibrosis, dermatomyositis may include involvement of upper 1/3 (striated), dysphagia to solids more than liquids (steakhouse syndrome), wide-open LES or HH, with or without basilar pulmonary fibrosis, other GI sites associated with CREST syndrome (12 Dec 1998) |
| localised scleroderma | A skin lesion that is characterised by the presence of localised, indurated, slightly depressed areas of thickened dermal tissue that may be white or yellow in colour and surrounded by a pink or purplish halo. See: in cutaneus scleroderma. (27 Sep 1997) |