Objective: Livedoid vasculopathy is a skin disease characterized by recurrent painful ulcerations of the lower leg leading to scar formation (atrophie blanche). Ulceration results from thrombosis of the cutaneous microcirculation and is often preceded by irregular broken circles of skin discoloration (livedo racemosa) in the lower extremities. Intense local ischemic pain, ulcerations, and irreversible scarring have a severe impact on patients' quality of life. There are currently no approved treatments for livedoid vasculopathy, making off-label therapy the only option. The German S1 guideline for treatment of livedoid vasculopathy recommends anticoagulation with low-molecular-weight heparins, rivaroxaban, and other direct oral anticoagulants as first-line therapy. Approach: We present a single-center follow-up study of 26 patients with livedoid vasculopathy (following STROBE). Patients treated according to the German S1 guideline consented to be monitored with a cross-sectional study questionnaire providing data on demographics, treatment protocol, disease course (pain, disease activity, and relapses), quality of life (Dermatology Life Quality Index score), and daily life impact. Results: Prolonged guideline-followed treatment of livedoid vasculopathy leads to effective management of pain and disease activity. Patients report therapy satisfaction and profit by sustained benefits in quality of life. Innovation: In this study, we analyzed the long-term efficacy of guideline-followed treatment in patients with livedoid vasculopathy over a period of up at least 3 months to more than 24 months. Conclusion: Guideline-followed treatment with anticoagulants like rivaroxaban is an effective long-term therapy option for patients with livedoid vasculopathy.
Keywords: atrophie blanche; guideline; leg ulcer; rivaroxaban; wound healing.