Prevalence of anti-endothelial cell antibodies in patients with Sneddon's syndrome

Background : Sneddon's syndrome consists of widespread livedo reticularis and ischemic cerebral manifestations. Its pathogenesis remains unclear. Endothelial cells could be the primary target tissue. Objective : Our aim was to determine the prevalence of anti-endothelial cell antibodies (AECA) in a large series of patients with Sneddon's syndrome. The results were compared with those of three groups of control subjects: 39 patients with active periarteritis nodosa, 20 patients hospitalized for stroke without livedo, and 28 healthy persons. Methods : AECA were detected with enzyme-linked immunosorbent assay with hybrid cells (EA.hy926) before and after absorption on epithelial cells ( A 549 8 ) to avoid false positivity from antibodies reacting with membranous epithelial antigens. Results : Twenty-two patients with Sneddon's syndrome had AECA (35%). Of the control subjects, 11 patients with active periarteritis nodosa (28%), 1 of 20 patients with a recent stroke without livedo, and no healthy persons had AECA. Conclusion : AECA were frequently found in patients with Sneddon's syndrome, in contrast to the patients with stroke without livedo. The clinical significance and involvement of these antibodies in the pathogenesis of endothelial lesions in Sneddon's syndrome remain to be ascertained.