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Generalized scleroderma

definition

Forme de sclérodermie généralisée heureusement rare chez l’enfant La forme limitée ou CREST syndrome est exceptionnelle chez l’enfant et associe une sclérodactylie, un syndrome de Raynaud , des calcifications sous cutanées des extrémitées et des telangiectasies. La forme systémique de pronostic très sévère :après une atteinte cutanée débutante se complique d’atteinte viscérale grave : extension au poumon, tube digestif, cœur et rein .

synonymes

Generalized scleroderma

UMLS

SCLERODERMIE GENERALISEE

images

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