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

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Background: Livedoid vasculopathy (LV) is a chronic dermatosis characterized by recurrent painful ulceration of the lower limbs. Antiplatelet, anticoagulant, fibrinolytic therapies and anti-inflammatory agents have been reported to be helpful. However, no continuing benefit has been reported in any of these modalities. Recently, pulsed intravenous immunoglobulin (IVIg) therapy has been reported to be effective in some refractory cases. Objectives: Outcomes in 7 patients treated with IVIG for treatment-resistant ulcerated LV were retrospectively analyzed. Methods: IVIg therapy was performed monthly at a dose of 2.0 g/kg body weight divided over 3 to 5 consecutive days. Skin lesion before and after therapy was assessed by a clinical score (sum of 3 items; erythema 0~3, ulceration 0~3, and pain 0~3). Results: Six patients were treated by 3 cycles and one by 2 cycles. In all patients, significant regression of skin lesions was observed after therapy resulting in a decrease of the clinical score from 5.7±2.5 at the beginning to 1.0±1.4 after therapy (P =.002). Two patients had no recurrences for 1.5 and 3 years each, but 5 patients relapsed; the median time to relapse was 9.8 months. Two of them were retreated with IVIG and were improved consistently, and another 2 patients were controlled with conventional oral medication. Conclusion: We propose that IVIg is a rapid, effective, and safe therapeutic option in LV refractory to other treatment modalities.