Dermatomyositis: Clinical features and pathogenesis

Dermatomyositis (DM) is an idiopathic inflammatory myopathy that is clinically heterogeneous and that can be difficult to diagnose. Cutaneous manifestations sometimes vary and may or may not parallel myositis and systemic involvement in time course or severity. Recent developments in our understanding of myositis-specific antibodies have the potential to change the diagnostic landscape of DM for dermatologists. Although phenotypic overlap exists, anti-Mi2, -MDA5, -NXP2, -TIF1, and -SAE antibodies may be correlated with distinct DM subtypes in terms of cutaneous manifestations, systemic involvement, and malignancy risk. This review highlights new findings on the DM-specific myositis-specific antibodies and their clinical associations in both adults and children. Dermatomyositis (DM) is an idiopathic inflammatory myopathy that is clinically heterogeneous and that can be difficult to diagnose. Cutaneous manifestations sometimes vary and may or may not parallel myositis and systemic involvement in time course or severity. Recent developments in our understanding of myositis-specific antibodies have the potential to change the diagnostic landscape of DM for dermatologists. Although phenotypic overlap exists, anti-Mi2, -MDA5, -NXP2, -TIF1, and -SAE antibodies may be correlated with distinct DM subtypes in terms of cutaneous manifestations, systemic involvement, and malignancy risk. This review highlights new findings on the DM-specific myositis-specific antibodies and their clinical associations in both adults and children. Journal Based CME Instructions and InformationJournal of the American Academy of DermatologyVol. 82Issue 2Preview Full-Text PDF Dermatomyositis: Clinical features and pathogenesisJournal of the American Academy of DermatologyVol. 83Issue 1PreviewTo the Editor: We were very pleased with the coverage by DeWane et al1 of anti–small ubiquitin-like modifier activating enzyme antibody–associated dermatomyositis (anti-SAE1) as a unique phenotype associated with early cutaneous findings typical of dermatomyositis, late muscle involvement, and adverse reactions to hydroxychloroquine. Recently, a series with patients with anti-SAE1 dermatomyositis from Japan was published by Inoue et al,2 detailing an interesting pattern of skin involvement observed in this condition, termed the “angel wing” sign. Full-Text PDF CME examinationJournal of the American Academy of DermatologyVol. 82Issue 2Preview Full-Text PDF