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Progressive Systemic Scleroderma


A chronic, progressive dermatosis characterized by boardlike hardening and immobility of the affected skin, with visceral involvement, especially of lungs, esophagus, kidneys and heart. It may be accompanied by calcinosis, Raynaud's phenomenon, and telangiectasis (CREST syndrome). It includes acrosclerosis and sclerodactyly. The pattern of scleroderma occuring in childhood differs from that in the adult. Childhood onset of progressive scleroderma is rare.


Progressive Systemic Scleroderma, Sclerodermia Progressiva, Systemic Sclerosis


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diagnosis: Progressive Systemic Scleroderma