Clinical efficacy of intravenous immunoglobulins for the treatment of dermatomyositis skin lesions without muscle disease

Background Treating dermatomyositis ( DM ) with isolated skin involvement is difficult and inconsistently performed. Intravenous immunoglobulins ( IVI g) are recommended for corticoresistant or corticodependant DM , but only a few cases of IVI g use in DM with isolated skin involvement have been reported. Design We performed a retrospective monocentric study of 27 patients who were treated with IVI g for severe DM skin lesions (no or minor muscle involvement) after failure of photoprotection and at least one line of treatment. Results Nineteen patients (70%) exhibited a major response, four patients exhibited a partial response and four patients exhibited no response, including two patients with grade 3 side effects (headaches). The mean number of IVI g courses was 4.8 (range 1–15). Ten patients (53%) relapsed, with a median time of 6.2 months after the last IVI g course. Six of these patients were successfully treated with a new IVI g course. Muscle disease developed in six patients. Conclusion IVI g may be an effective and safe treatment for DM with isolated skin involvement. Relapse occurred frequently, but treatment with a new course of IVI g was successful. Controlled studies are required to confirm these results.