Livedoid Vasculopathy Successfully Treated With JAK Inhibitors

Abstract

Livedoid vasculopathy is a rare thrombotic disease of the small blood vessels. It manifests as fixed violaceous macules, noninflammatory retiform purpura, painful ulcers, and white atrophic scars on the lower extremities. The pathophysiology involves the type 1 interferon pathway. Many patients do not improve with oral anticoagulants. We report here two cases of patients with refractory livedoid vasculopathy treated successfully with JAK inhibitors. The first patient was treated with baricitinib with complete remission of leg ulcers after 4 months of treatment. The second patient was treated with upadacitinib and achieved complete remission after 6 months of treatment. Clinical and biological tolerability was excellent in both patients. JAK inhibitors, through their effect on the interferon pathway, may be a promising treatment for livedoid vasculopathy.