Ulcerative Heliotrope Rash in Antimelanoma Differentiation-Associated Gene 5 Dermatomyositis

Antimelanoma differentiation-associated gene 5 dermatomyositis (anti-MDA5 DM) is an amyopathic subtype of DM that presents with the classic cutaneous findings of DM, such as a heliotrope rash, Gottron papules, and the shawl sign, combined with mucocutaneous ulcerations. This subtype of DM also is highly associated with rapidly progressive interstitial lung disease (ILD). We report 2 cases of a heretofore novel presentation of the overlap of these cutaneous features in the form of an ulcerative heliotrope rash; furthermore, the rash was associated with progression of ILD in both cases. Finally, we review the current state of clinical care and research related to anti-MDA5 DM in terms of clinical presentations, diagnostic methods, and treatment options.