Successful Treatment of Livedo Vasculitis with Beraprost Sodium: A Possible Mechanism of Thrombomodulin Upregulation

Abstract

BACKGROUND: Livedo vasculitis is thought to be a thrombogenic disorder. We demonstrated that thrombomodulin (TM) expression on the endothelial cells in livedo vasculitis was markedly reduced, while blood tests of coagulation and fibrinolytic activities were within the normal range. OBJECTIVE: Since prostacyclin (PGI2) upregulates the TM expression of endothelial cells, we tried a PGI2 analogue for the treatment of livedo vasculitis. METHOD: Four patients with livedo vasculitis were started on a regimen of beraprost sodium (120 micrograms daily). Additional intake of low-dose aspirin was combinated with a maintenance dose of beraprost sodium (60 micrograms daily). RESULT: All 4 patients experienced a clinical improvement with a combination therapy with beraprost sodium and low-dose aspirin. CONCLUSION: We propose that PGI2 analogue therapy is useful for the treatment of livedo vasculitis, since the drug upregulates TM expression in this disorder.