[Scleroderma presenting as oedema (authors transl)]

The authors report 8 cases of scleroderma with oedema in the initial stages which could be divided amongst three groups on the basis of their clinical, biological, histological characteristics and course: normal systemic scleroderma, mixed connective tissue disease, Shulmans syndrome. The relationship between one of these cases and Buschke's scleroderma is discussed. These cases of scleroderma are characterized by diagnostic difficulties in the initial phase and by their relatively favourable vital prognosis (one death only). Corticosteroids may be effective to some extent in the presence of mixed connective tissue disease of Shulman's syndrome.