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Progressive Systemic Scleroderma

definition

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.

synonyms

Progressive Systemic Scleroderma, Sclerodermia Progressiva, Systemic Sclerosis

UMLS

Diffuse Scleroderma, Generalised scleroderma, Generalized scleroderma, Progressive Scleroderma, Progressive Systemic Scleroses, PROGRESSIVE SYSTEMIC SCLEROSIS, PSS, Scleroderma diffuse, Scleroderma generalized, Scleroderma, Diffuse, Scleroderma, Progressive, Scleroderma, Systemic, Sclerodermia generalized, Sclerodermia progressive, Scleroses, Progressive Systemic, Scleroses, Systemic, Sclerosis, Progressive Systemic, Sclerosis, Systemic, Sclerosis, systemic progressive, Systemic scleroderma, Systemic Scleroses, Systemic Scleroses, Progressive, Systemic sclerosis, Systemic Sclerosis, Progressive

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