Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects.

Important points regarding DM and C-ADM are as follows: C-ADM is a working functional designation for patients having the skin-only and skin-predominant subsets of DM, amyopathic DM, and hypomyopathic DM. C-ADM seems to have approximately 10% the incidence of classic DM in whites and possibly a higher incidence in Asians. Some patients who present with C-ADM, with or without subclinical laboratory abnormalities, can slowly progress to develop symptomatic muscle weakness over a period of years, whereas others go for 10 to 20 years and longer without the appearance of muscle weakness. C-ADM patients are at risk for potentially life-threatening complications of classic DM, such as interstitial lung disease, which may occur in up to 10% of C-ADM patients. This risk seems to be even greater in some ethnic subgroups (e.g., Japanese). C-ADM patients may also be at increased risk for internal malignancy and until further studies are carried out to confirm the statistical significance of this association, all such patients should have a thorough evaluation for internal malignancy, identical to the approach currently used in classic DM patients. Dermatologists are in the best position initially to diagnose C-ADM patients and can contribute greatly to their overall management and quality of life. Ongoing vigilance is required, however, for complications that can arise in C-ADM patients including potentially fatal interstitial lung disease, internal malignancy, delayed onset of muscle weakness from myositis, and complications of systemic drug therapy. Topical therapy with broad-spectrum sunscreens, anti-inflammatories, and antipruritics should be maximized during the initial management of the cutaneous manifestations of either classic DM or C-ADM. Single-agent or combined aminoquinoline antimalarial therapy represents the safest initial form of systemic therapy for DM-specific skin disease occurring in any clinical setting; however, this approach tends to be less effective in general than for cutaneous LE. There is a theoretical rationale for and limited preliminary successful anecdotal experience with the use of anti-TNF-alpha therapy in refractory cases of classic DM and C-ADM. Cautious systematic clinical trials in this area should be considered.