Studies of the microvascular endothelium in uninvolved skin of patients with systemic sclerosis: Direct evidence for a generalized microangiopathy

Various parameters for assessing endothelial cell (EC) metabolism, including immunohistochemistry and adenosine uptake, have been compared in the clinically uninvolved skin of patients with diffuse systemic sclerosis (DSS), CREST, incomplete CREST syndrome (ICREST), primary Raynaud's disease (1° RD) and normal controls. Evidence of platelet adhesion to EC, decreased EC storage of factor VIII-related antigen, changes in EC morphology and decreased adenosine uptake by EC, were found in the dermal microvasculature of normal skin of patients with DSS, CREST and ICREST, but not in 1° RD. These data indicate that a generalized microvascular endothelial dysfunction is present in the skin of patients with the systemic forms of scleroderma.