Pulsed intravenous immunoglobulin therapy in refractory ulcerated livedoid vasculopathy: seven cases and a literature review

Livedoid vasculopathy ( LV ) is a thrombotic vasculopathy of the skin of unknown origin. No treatment has been validated in this indication, but case reports demonstrated successful use of intravenous immunoglobulins ( IVIg ) in LV . We assessed the efficacy and tolerability of 2 g/kg IVIg therapy every month for 2∼3 cycles in patients with refractory LV . We analyzed the efficacy, side effects and recurrence after long‐term follow‐up (51.9 ± 14.0 months) in seven patients with LV treated with 2 g/kg of IVIg . Mean clinical score of sum of erythema, ulceration and pain index (each: 0–3) was 5.7 ± 0.9 before the therapy and significantly lower after therapy (1.1 ± 0.5) ( p = 0.001). Even after just one cycle of IVIg , the score decreased significantly from 5.7 ± 0.9 to 3.7 ± 0.9 ( p = 0.002), especially the pain score. In one patient, LV has not recurred for over 7 years; six patients experienced recurrence after a mean of 12.7 ± 2.8 months. Out of the six patients, two patients were re‐administered IVIg whereas the others were well controlled by conventional therapy. We propose that IVIg is a rapid, effective, and safe therapeutic option in LV refractory to other treatment modalities.