REVIEW   Free access

Italian Journal of Dermatology and Venereology 2023 April;158(2):84-98

DOI: 10.23736/S2784-8671.23.07458-3

Copyright © 2023 EDIZIONI MINERVA MEDICA

language: English

Dermatomyositis: a comprehensive review of clinical manifestations, serological features, and therapeutic approaches

Dario DIDONA ¹ ✉, Farzan SOLIMANI ^{2, 3}, Raffaele D. CAPOSIENA CARO ⁴, Antonio M. SEQUEIRA SANTOS ¹, Julia HINTERSEHER ¹, Jacqueline KUSSINI ¹, Tomas CUNHA ¹, Michael HERTL ¹, Biagio DIDONA ⁵

¹ Department of Dermatology and Allergology, Philipps University of Marburg, Marburg, Germany; ² Berlin Institute of Health at Charité-Universitätsmedizin Berlin BIH, Biomedical Innovation Academy, BIH Charité Clinician Scientist Program, Berlin, Germany; ³ Department of Dermatology, Venereology and Allergology, Charité-Universitätsmedizin Berlin, Berlin, Germany; ⁴ Clinic of Dermatology, Maggiore Hospital, University of Trieste, Trieste, Italy; ⁵ Department of Dermatology, Istituto Dermopatico dell’Immacolata (IDI)-IRCCS, Rome, Italy

Dermatomyositis (DM) is an autoimmune disorder, which belongs to a group of rare autoimmune dermatoses characterized by different skin features and variable muscle involvement. We recognize four main variants of DM: classic DM, clinically amyopathic DM, paraneoplastic DM, and juvenile DM. Clinically, patients show several skin features, but heliotrope rash, and violaceous papules located at the interphalangeal or metacarpophalangeal joints (Gottron’s papules) are the most frequently observed. Together with skin features, patients show muscle involvement, most commonly with symmetrical weakness of the proximal muscles. DM belongs to the facultative paraneoplastic dermatoses and a wide range of solid or hematologic malignancies can be detected in DM patients. Serologically, a wide range of autoantibodies can be detected in patients with DM. Indeed, distinct serotypes can be related to specific phenotypes with specific clinical features, carrying a different risk for systemic involvement and for malignancies. Systemic corticosteroids are still considered the first-line approach, but several steroid-sparing agents, such as methotrexate, azathioprine or mycophenolate mofetil, have been reported as effective in treating DM. Furthermore, new class of drugs, such as monoclonal antibodies, purified immunoglobulins or Janus kinase inhibitors are becoming more relevant in the clinical practice or are currently under investigation. In this work, we aim to offer a clinical overview of the diagnostic workout, the characteristics of DM variants, the role of autoantibodies in DM, and the management of this life-threatening systemic disorder.

KEY WORDS: Amyopathic dermatomyositis; Autoantibodies; Dermatomyositis; Myositis