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25-011177_CG_Dermatomyositis

Dermatomyositis

Clinical Presentation

Julie Paik, MD, MHS 
Reviewed on February 14, 2025
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Presentation of Different Types of Dermatomyositis

Classic DM (CDM) presents with pathognomonic cutaneous findings and progressive, symmetric proximal muscle weakness. Cutaneous disease usually precedes the appearance of myositis by 3–6 months in 30–50% of patients, while 10% of patients present with muscle symptoms before the development of skin symptoms. The onset of the disease is subacute, often waxing and waning.

Different types of Dermatomyositis (DM) present with different sets of symptoms (Table 2-1).

In some subtypes of DM, specific skin symptoms emerge, which can aid in the differential diagnosis and classification of different subtypes of DM:

  • Anti-MDA5-related DM: Palmar papules, skin ulcers
  • Anti-NXP2-related DM: Calcinosis
  • Anti-TIF1-γ-related DM: Palatal patch
  • Anti-SAE: Whole body erythroderma, ulcerative lesions
Enlarge 

Skin Manifestations

Pathognomonic skin findings observed in approximately 80% of patients include Gottron’s papules and sign, and heliotrope rash. Skin…

Presentation of Different Types of Dermatomyositis

Classic DM (CDM) presents with pathognomonic cutaneous findings and progressive, symmetric proximal muscle weakness. Cutaneous disease usually precedes the appearance of myositis by 3–6 months in 30–50% of patients, while 10% of patients present with muscle symptoms before the development of skin symptoms. The onset of the disease is subacute, often waxing and waning.

Different types of Dermatomyositis (DM) present with different sets of symptoms (Table 2-1).

In some subtypes of DM, specific skin symptoms emerge, which can aid in the differential diagnosis and classification of different subtypes of DM:

  • Anti-MDA5-related DM: Palmar papules, skin ulcers
  • Anti-NXP2-related DM: Calcinosis
  • Anti-TIF1-γ-related DM: Palatal patch
  • Anti-SAE: Whole body erythroderma, ulcerative lesions
Enlarge 

Skin Manifestations

Pathognomonic skin findings observed in approximately 80% of patients include Gottron’s papules and sign, and heliotrope rash. Skin manifestations that are characteristic of DM patients include V-neck sign and shawl sign, periungual erythema, cuticular hypertrophy, periungual telangiectasia, and poikiloderma in photo-exposed areas.

Pruritus is a frequent and often debilitating symptom in patients with DM. Significant and bothersome pruritus may be present in up to half of DM patients.

Skin ulcers can occur in dermatomyositis; and in particular in MDA5-associated DM, punched out ulcers over the joints, i.e. metacarpophalangeal joints (MCPs), can accompany the disease and tend to be recalcitrant to therapy.

Muscle Manifestations

Muscle weakness is a common presenting symptom in patients with DM, with a subacute onset with gradually progressive symmetric proximal muscle weakness involving the shoulders, hips, and neck muscles. Muscle pain and stiffness are less common. Patients may have difficulty with activities such as climbing stairs, getting up from a seated position, lifting objects, combing hair, and raising their head from a pillow. In severe and longstanding cases of DM, patients may have dysphagia and dysphonia.

References

  • Aggarwal, R., Schessl, J., Charles-Schoeman, C. et al. Safety and tolerability of intravenous immunoglobulin in patients with active dermatomyositis: results from the randomised, placebo-controlled ProDERM study. Arthritis Res Ther 26, 27 (2024).
  • ArgenX Press Release. Argenx Advances Clinical Development of Efgartigimod SC in Idiopathic Inflammatory Myopathies. November 20, 2024. https://argenx.com/content/dam/argenx-corp/newsroom/press-release-pdfs/PressRelease_argenx_ALKIVIA_Ph2_20241120.pdf.coredownload.inline.pdf
  • Bax CE, Maddukuri S, Ravishankar A, Pappas-Taffer L, Werth VP. Environmental triggers of dermatomyositis: a narrative review. Ann Transl Med. 2021 Mar;9(5):434. doi: 10.21037/atm-20-3719. PMID: 33842655; PMCID: PMC8033368.
  • Bottai M, Tjärnlund A, Santoni G, Werth VP, Pilkington C, de Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Danko K, Dimachkie MM, Feldman BM, García-De La Torre I, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinka M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Wook Song Y, Vencovsky J, Ytterberg SR, Miller FW, Rider LG, Lundberg IE; International Myositis Classification Criteria Project consortium, the Euromyositis register and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland). EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report. RMD Open. 2017 Nov 14;3(2):e000507. doi: 10.1136/rmdopen-2017-000507. PMID: 29177080; PMCID: PMC5687535.
  • Caravan S, Lopez CM, Yeh JE. Causes and Clinical Presentation of Drug-Induced Dermatomyositis: A Systematic Review. JAMA Dermatol. 2024;160(2):210–217. doi:10.1001/jamadermatol.2023.5418
  • Cassard L, Seraly N, Riegert M, Patel A, Fernandez AP. Dermatomyositis: Practical Guidance and Unmet Needs. Immunotargets Ther. 2024;13:151-172.
  • Chandra T, Aggarwal R. A Narrative Review of Acthar Gel for the Treatment of Myositis. Rheumatol Ther. 2023 Jun;10(3):523-537. doi: 10.1007/s40744-023-00545-1. Epub 2023 Mar 26. PMID: 36966453; PMCID: PMC10140234.
  • Chung MP, Paik JJ. Past, Present, and Future in Dermatomyositis Therapeutics. Curr Treatm Opt Rheumatol. 2022 Dec;8(4):71-90. doi: 10.1007/s40674-022-00193-6. Epub 2022 Jul 26. PMID: 38650607; PMCID: PMC11034924.
  • Cobos GA, Femia A, Vleugels RA. Dermatomyositis: An Update on Diagnosis and Treatment. Am J Clin Dermatol. 2020 Jun;21(3):339-353. doi: 10.1007/s40257-020-00502-6. PMID: 32096127.
  • Connolly CM, Gupta L, Fujimoto M, Machado PM, Paik JJ. Idiopathic inflammatory myopathies: current insights and future frontiers. Lancet Rheumatol. 2024 Feb;6(2):e115-e127. doi: 10.1016/S2665-9913(23)00322-3. PMID: 38267098.
  • Connolly CM, Paik JJ. Clinical pearls and promising therapies in myositis. Expert Rev Clin Immunol. 2023 Jul-Dec;19(7):797-811. doi: 10.1080/1744666X.2023.2212162. Epub 2023 May 18. PMID: 37158055; PMCID: PMC10330909.
  • Demortier J, Vautier M, Chosidow O, Gallay L, Bessis D, Berezne A, Cordel N, Schmidt J, Smail A, Duffau P, Jachiet M, Begon E, Gottlieb J, Chasset F, Graveleau J, Marque M, Cesbron E, Forestier A, Josse S, Kluger N, Beauchêne C, Le Corre Y, Pagis V, Rigolet A, Guillaume-Jugnot P, Authier FJ, Guilain N, Streichenberger N, Leonard-Louis S, Boussouar S, Landon-Cardinal O, Benveniste O, Allenbach Y. Anti-SAE autoantibody in dermatomyositis: original comparative study and review of the literature. Rheumatology (Oxford). 2023 Dec 1;62(12):3932-3939. doi: 10.1093/rheumatology/kead154. PMID: 37010495.
  • Fiorentino D, Mangold AR, Werth VP, Christopher-Stine L, Femia A, Chu M, Musiek ACM, Sluzevich JC, Graham LV, Fernandez AP, Aggarwal R, Rieger K, Page KM, Li X, Hyde C, Rath N, Sloan A, Oemar B, Banerjee A, Salganik M, Banfield C, Neelakantan S, Beebe JS, Vincent MS, Peeva E, Vleugels RA. Efficacy, safety, and target engagement of dazukibart, an IFNβ specific monoclonal antibody, in adults with dermatomyositis: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial. Lancet. 2025 Jan 11;405(10473):137-146. doi: 10.1016/S0140-6736(24)02071-3. PMID: 39798982.
  • Kronzer, V. L., Kimbrough, B. A., Crowson, C. S., Davis, J. M., Holmqvist, M., & Ernste, F. C. (2023). Incidence, Prevalence, and Mortality of Dermatomyositis: A Population-Based Cohort Study. Arthritis Care and Research, 75(2), 348-355.
  • Lim D, Fiorentino D, Werth V. Current concepts and advances in dermatomyositis: a dermatological perspective. Clin Exp Rheumatol. 2023 Mar;41(2):359-369. doi: 10.55563/clinexprheumatol/ue71ku. Epub 2023 Jan 3. PMID: 36622138.
  • Oldroyd, A.G.S., Callen, J.P., Chinoy, H. et al. International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening: an International Myositis Assessment and Clinical Studies Group (IMACS) initiative. Nat Rev Rheumatol 19, 805–817 (2023). https://doi.org/10.1038/s41584-023-01045-w.
  • Oldroyd AGS, Lilleker JB, Amin T, Aragon O, Bechman K, Cuthbert V, Galloway J, Gordon P, Gregory WJ, Gunawardena H, Hanna MG, Isenberg D, Jackman J, Kiely PDW, Livermore P, Machado PM, Maillard S, McHugh N, Murphy R, Pilkington C, Prabu A, Rushe P, Spinty S, Swan J, Tahir H, Tansley SL, Truepenny P, Truepenny Y, Warrier K, Yates M, Papadopoulou C, Martin N, McCann L, Chinoy H; British Society for Rheumatology Standards, Audit and Guidelines Working Group. British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy. Rheumatology (Oxford). 2022 May 5;61(5):1760-1768. doi: 10.1093/rheumatology/keac115. PMID: 35355064; PMCID: PMC9398208.
  • Paik JJ, Lubin G, Gromatzky A, Mudd PN Jr, Ponda MP, Christopher-Stine L. Use of Janus kinase inhibitors in dermatomyositis: a systematic literature review. Clin Exp Rheumatol. 2023 Mar;41(2):348-358. doi: 10.55563/clinexprheumatol/hxin6o. Epub 2022 Jun 28. PMID: 35766013; PMCID: PMC10105327.
  • Patil A, Lu J, Kassir M, Babaei M, Goldust M. Adult and juvenile dermatomyositis treatment. J Cosmet Dermatol. 2023 Feb;22(2):395-401. doi: 10.1111/jocd.15363. Epub 2022 Sep 16. PMID: 36065712.
  • Qudsiya Z, Waseem M. Dermatomyositis. [Updated 2023 Aug 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
  • Sevim E, Kobrin D, Casal-Dominguez M, Pinal-Fernandez I. A comprehensive review of dermatomyositis treatments - from rediscovered classics to promising horizons. Expert Rev Clin Immunol. 2024 Feb;20(2):197-209. doi: 10.1080/1744666X.2023.2270737. Epub 2024 Jan 21. PMID: 37842905; PMCID: PMC11611049.
  • Valenzuela A, Chung L, Casciola-Rosen L, Fiorentino D. Identification of clinical features and autoantibodies associated with calcinosis in dermatomyositis. JAMA Dermatol. 2014 Jul;150(7):724-9. doi: 10.1001/jamadermatol.2013.10416. PMID: 24869801; PMCID: PMC4351704.
  • Wolstencroft PW, Casciola-Rosen L, Fiorentino DF. Association Between Autoantibody Phenotype and Cutaneous Adverse Reactions to Hydroxychloroquine in Dermatomyositis. JAMA Dermatol. 2018 Oct 1;154(10):1199-1203. doi: 10.1001/jamadermatol.2018.2549. PMID: 30140893; PMCID: PMC6233745.
  • Zhao X, Si S. Five genes as diagnostic biomarkers of dermatomyositis and their correlation with immune cell infiltration. Front Immunol. 2023 Jan 18;14:1053099. doi: 10.3389/fimmu.2023.1053099. PMID: 36742332; PMCID: PMC9889851.
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