[A woman with skin abnormalities and muscle weakness].

A 54-year-old woman presented to the emergency department with progressive proximal muscle weakness and a symmetric skin rash. Physical examination demonstrated a heliotrope rash, Gottron lesions, mechanic's hands and symmetrical erythema of the face, neck and upper legs. The diagnosis 'dermatomyositis' was established.

No effects of bosentan on microvasculature in patients with limited cutaneous systemic sclerosis.

The endothelium-derived vasoconstrictor molecule endothelin-1 (ET-1) has been suggested to play a role in the pathogenesis of Raynaud's phenomenon (RP) and systemic sclerosis (SSc). We studied the effect of bosentan on microvascular structure and function in patients with RP secondary to limited cutaneous SSc in a mechanistic pilot study. In this single center, open study, 15 patients with limited cutaneous SSc were treated with bosentan for 16 weeks with a follow-up period of 4 weeks.

Early atherosclerosis in systemic sclerosis and its relation to disease or traditional risk factors.

Introduction: Several systemic autoimmune diseases are associated with an increased prevalence of atherosclerosis which could not be explained by traditional risk factors alone. In systemic sclerosis (SSc), microvascular abnormalities are well recognized. Previous studies have suggested an increased prevalence of macrovascular disease as well.