Pulsed intravenous immunoglobulin therapy in livedoid vasculitis: An open trial evaluating 9 consecutive patients

Abstract

Livedoid vasculitis (LV) usually presents with painful, slowly healing ulcerations of the lower limbs. The precise pathophysiology of this relatively rare disease remains obscure. Therapeutic strategies usually include rheologic, anti-inflammatory, or immunosuppressive agents. However, no continuing benefit has been reported in any of these modalities. Recently, encouraging case reports about the successful use of intravenous immunoglobulin (IVIg) in LV have been published. We initiated an open single center trial to investigate the efficacy and safety of IVIg in LV. Nine patients with LV, 7 of whom were refractory to other treatment modalities, were included. Therapy with IVIg at a dose of 0.5 g/kg body weight per day over 2 or 3 consecutive days was performed monthly. Skin involvement before and after therapy was assessed by means of a clinical score. In all patients, significant regression of skin lesions was observed after therapy resulting in a decrease of the clinical score (including differential semiquantitative assessment of erythema, ulceration, and pain) from 6.5 +/- 1.7 at the beginning to 1.3 +/- 1.2 after therapy (P <.001). IVIg was well tolerated and therapy was finished in all patients. In all patients clinical evaluation revealed a marked improvement of erythema, pain, and healing of areas of active ulceration. Although this was an open non-controlled study, we propose that IVIg is a promising therapeutic option in LV refractory to other treatment modalities.