Dermatomyositis with Isolated Severe Skin Lesions Responds to IVIG Therapy

Intravenous immune globulin (IVIG) is a recommended therapy for corticoresistant or corticodependent dermatomyositis (DM), but only a few cases of difficult-to-treat DM with isolated skin involvement have been reported. Investigators at Hôpital Saint Louis in Paris conducted a retrospective single-center study of 27 patients treated with IVIG for severe DM skin lesions and no or minor muscle involvement, following failure of photoprotection and at least one line of treatment.

Nineteen of the 27 DM patients exhibited a major response to treatment with IVIG. Four patients experienced a partial response, and the remaining four patients had no response. The mean number of IVIG courses was 4.8 (range 1 to 15). Ten of the 19 responders (53%) relapsed, with a median time of 6.2 months after the last IVIG course; six of these patients were successfully re-treated with a new course of IVIG therapy.

The investigators concluded that IVIG may be an effective and safe treatment for DM with isolated skin involvement. Relapse occurred frequently in this patient series, but treatment with a new course of IVIG was successful. They called for controlled studies to confirm these results.