Autoimmune-Associated Livedoid Vasculopathy: Response to Immunomodulatory Therapy

Livedoid vasculopathy (LV) is a rare, chronic occlusive dermopathy causing recurrent painful ulcerations, impaired healing, and significant morbidity. Historically considered a thrombotic microangiopathy, emerging evidence suggests an autoimmune or mixed pathogenesis in some patients. We present the case of a 55-year-old woman with LV and serologic features of undifferentiated connective tissue disease (UCTD) who achieved complete, durable remission using intravenous immunoglobulin (IVIG) and hydroxychloroquine (HCQ). After 16 years of refractory disease, including failure of multiple anticoagulant, antiplatelet, and immunosuppressive regimens, her ulcers resolved completely and have not recurred for more than two years. This case underscores the need to evaluate for autoimmune features in LV, highlights IVIG as an effective treatment, and demonstrates that HCQ may serve as a valuable adjunct in refractory autoimmune-associated LV.