Objective/Background Livedoid vasculopathy (LV) is a rare vasculopathy that affects the lower extremities, causing painful recurrent ulcerations, livedo reticularis and atrophie blanche.1 It might be idiopathic or present in association with hypercoagulable states or vascular disorders in 50-70%.2 Despite increased treatment options, patients often face suboptimal treatment outcomes.3 Data based on systematic review for treatment outcomes of LV are initially needed to identify gaps of knowledge and inform next steps in clinical trial design.

Methods We searched major databases from January 2017 to October 2023 using key terms related to Livedoid Vasculopathy and its treatment outcomes. We included case reports, series, cross-sectional and retrospective studies (excluding reviews and articles without defined treatment outcomes). Analysis followed PRISMA guidelines with two reviewers independently assessing studies. Conflicts were resolved by a third party.

Results Out of 614 articles identified, 44 were included in the final review, most of which were case reports or series. In total, there were 216 participants (155 female, 61 male) with median age 44 years (14-73).

Rivaroxaban was the most reported treatment (n=58), showing complete (n=18, 31%) or partial (n=40, 68%) ulcer healing and complete pain resolution in the 19% reported. Other factor Xa inhibitors (n=4) led to complete wound healing and pain relief in our review. IVIG (n=36) showed promising results in wound and pain resolution, though some patients did not achieve adequate healing (n=4, 11%). Less common treatments like hyperbaric oxygen therapy (n=3), anti-TNF (n=1), and others also achieved complete healing and pain improvement.

Conclusion A variety of therapeutic strategies for LV have shown promise, but data to support any specific regimen remains lacking. In recent literature, Rivaroxaban was the most used therapy with 82.2% (60/73) of patients achieving rapid healing and pain relief.4 IVIG disrupts inflammatory cytokines, modulates lymphocytes, and neutralizes autoantibodies, making it a potential treatment for LV due to the presence of these markers in LV patients.5 Future directions consist of determining which patients (with primary or secondary LV) might benefit from single or combined therapies and establishing standard of care for this condition that can also be used as a comparator arm in clinical trials.