CADM represents a significant subset of the DM population. As with classic DM, the cutaneous manifestations of CADM often represent a therapeutic challenge. A subset of patients with CADM has underlying malignancies, and these may differ from those typically associated with classic DM. Differences in serological abnormalities, cutaneous manifestations and response to first treatment among patients with CADM with and without malignancy were found, and suggest distinct pathophysiologies among CADM subsets. Characterization of this cohort expands the knowledge about this unique DM subset.

Use of IVIG resulted in improvement of refractory cutaneous DM in the vast majority of patients relatively soon after initiation and regardless of DM subtype or clinical manifestations. Additionally, IVIG allowed decrease or discontinuation of immunosuppressive medications in 80% of patients. These findings suggest that IVIG can be a clinically efficacious and cost-effective treatment for refractory cutaneous DM and warrants prospective study.