Intravenous immunoglobulins in difficult-to-treat ulcerated livedoid vasculopathy: five cases and a literature review

Background Livedoid vasculopathy (LV) is a thrombotic vasculopathy of the skin of unknown origin. No treatment has been validated in this indication, but case reports suggest the successful use of intravenous immunoglobulins (IVIG) in LV. Methods Outcomes in five patients treated with IVIG for treatment-resistant ulcerated LV were retrospectively analyzed. Results Treatment with IVIG induced complete remission (based on clinical evaluation and a pain-related visual analog scale) in four patients but was ineffective in one patient. Three patients relapsed; the median time to relapse was 10.7months. Re-treatment with IVIG in these three patients was successful. Conclusions These cases confirm previous reports that IVIG seems to be a rapid, effective, and safe treatment for patients with idiopathic refractory ulcerated LV. However, a placebo-controlled study is mandatory to confirm these results.