Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Clinical manifestations of dermatomyositis and polymyositis in adults

Marc L Miller, MD
Ruth Ann Vleugels, MD, MPH
Section Editors
Ira N Targoff, MD
Jeremy M Shefner, MD, PhD
Jeffrey Callen, MD, FACP, FAAD
Deputy Editors
Monica Ramirez Curtis, MD, MPH
Abena O Ofori, MD


Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies, characterized by the shared features of proximal skeletal muscle weakness and by evidence of muscle inflammation [1-5]. DM, unlike PM, is associated with a variety of characteristic skin manifestations. A form of DM termed amyopathic DM (ADM, historically termed "dermatomyositis sine myositis") is a condition in which patients have characteristic skin findings of DM without weakness or abnormal muscle enzymes.

The clinical and serologic features of DM and PM vary among affected individuals and populations, depending upon immunogenetic and possibly other genetic factors [6,7]. The immune mechanisms and anatomic focus of injury within the muscle tissue in PM and DM appear distinct. The other major type of idiopathic inflammatory myopathy is inclusion body myositis.

The clinical and laboratory manifestations of DM and PM in adults will be reviewed here. The diagnosis, electrophysiologic and pathologic findings on diagnostic testing, differential diagnosis, and treatment of these diseases and of the related disorders that occur in children (known as juvenile DM and PM); the pathogenesis of inflammatory myopathies in adults and of juvenile DM and PM; the risk for malignancy in patients with DM and PM; and the clinical manifestations, diagnosis, and treatment of inclusion body myositis are discussed separately.

(See "Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults".)

(See "Initial treatment of dermatomyositis and polymyositis in adults" and "Treatment of recurrent and resistant dermatomyositis and polymyositis in adults" and "Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis".)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Jan 30, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362:971.
  2. Plotz PH, Dalakas M, Leff RL, et al. Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders. Ann Intern Med 1989; 111:143.
  3. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975; 292:344.
  4. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975; 292:403.
  5. Amato AA, Barohn RJ. Evaluation and treatment of inflammatory myopathies. J Neurol Neurosurg Psychiatry 2009; 80:1060.
  6. Shamim EA, Rider LG, Pandey JP, et al. Differences in idiopathic inflammatory myopathy phenotypes and genotypes between Mesoamerican Mestizos and North American Caucasians: ethnogeographic influences in the genetics and clinical expression of myositis. Arthritis Rheum 2002; 46:1885.
  7. O'Hanlon TP, Carrick DM, Targoff IN, et al. Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1, and -DQA1 allelic profiles distinguish European American patients with different myositis autoantibodies. Medicine (Baltimore) 2006; 85:111.
  8. Jacobson DL, Gange SJ, Rose NR, Graham NM. Epidemiology and estimated population burden of selected autoimmune diseases in the United States. Clin Immunol Immunopathol 1997; 84:223.
  9. Bohan A, Peter JB, Bowman RL, Pearson CM. Computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine (Baltimore) 1977; 56:255.
  10. Tymms KE, Webb J. Dermatopolymyositis and other connective tissue diseases: a review of 105 cases. J Rheumatol 1985; 12:1140.
  11. Cooper GS, Stroehla BC. The epidemiology of autoimmune diseases. Autoimmun Rev 2003; 2:119.
  12. Bernatsky S, Joseph L, Pineau CA, et al. Estimating the prevalence of polymyositis and dermatomyositis from administrative data: age, sex and regional differences. Ann Rheum Dis 2009; 68:1192.
  13. Bendewald MJ, Wetter DA, Li X, Davis MD. Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in Olmsted County, Minnesota. Arch Dermatol 2010; 146:26.
  14. Sekiguchi K, Kanda F, Oishi K, et al. HLA typing in focal myositis. J Neurol Sci 2004; 227:21.
  15. Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol 2006; 24:363.
  16. Callen JP. Cutaneous manifestations of dermatomyositis and their management. Curr Rheumatol Rep 2010; 12:192.
  17. Dugan EM, Huber AM, Miller FW, et al. Photoessay of the cutaneous manifestations of the idiopathic inflammatory myopathies. Dermatol Online J 2009; 15:1.
  18. Smith RL, Sundberg J, Shamiyah E, et al. Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops. J Rheumatol 2004; 31:1644.
  19. Gunawardena H, Wedderburn LR, Chinoy H, et al. Autoantibodies to a 140-kd protein in juvenile dermatomyositis are associated with calcinosis. Arthritis Rheum 2009; 60:1807.
  20. Ceribelli A, Fredi M, Taraborelli M, et al. Anti-MJ/NXP-2 autoantibody specificity in a cohort of adult Italian patients with polymyositis/dermatomyositis. Arthritis Res Ther 2012; 14:R97.
  21. Stahl NI, Klippel JH, Decker JL. A cutaneous lesion associated with myositis. Ann Intern Med 1979; 91:577.
  22. Fiorentino D, Chung L, Zwerner J, et al. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol 2011; 65:25.
  23. Yamamoto T, Nishioka K. Flagellate erythema. Int J Dermatol 2006; 45:627.
  24. Iorizzo LJ 3rd, Jorizzo JL. The treatment and prognosis of dermatomyositis: an updated review. J Am Acad Dermatol 2008; 59:99.
  25. Jara M, Amérigo J, Duce S, Borbujo J. Dermatomyositis and flagellate erythema. Clin Exp Dermatol 1996; 21:440.
  26. Milisenda JC, Doti PI, Prieto-González S, Grau JM. Dermatomyositis presenting with severe subcutaneous edema: five additional cases and review of the literature. Semin Arthritis Rheum 2014; 44:228.
  27. de Merieux P, Verity MA, Clements PJ, Paulus HE. Esophageal abnormalities and dysphagia in polymyositis and dermatomyositis. Arthritis Rheum 1983; 26:961.
  28. Marie I, Hatron PY, Levesque H, et al. Influence of age on characteristics of polymyositis and dermatomyositis in adults. Medicine (Baltimore) 1999; 78:139.
  29. Denbow CE, Lie JT, Tancredi RG, Bunch TW. Cardiac involvement in polymyositis: a clinicopathologic study of 20 autopsied patients. Arthritis Rheum 1979; 22:1088.
  30. Lundberg IE. The heart in dermatomyositis and polymyositis. Rheumatology (Oxford) 2006; 45 Suppl 4:iv18.
  31. Danieli MG, Gelardi C, Guerra F, et al. Cardiac involvement in polymyositis and dermatomyositis. Autoimmun Rev 2016; 15:462.
  32. Tisseverasinghe A, Bernatsky S, Pineau CA. Arterial events in persons with dermatomyositis and polymyositis. J Rheumatol 2009; 36:1943.
  33. Rai SK, Choi HK, Sayre EC, Aviña-Zubieta JA. Risk of myocardial infarction and ischaemic stroke in adults with polymyositis and dermatomyositis: a general population-based study. Rheumatology (Oxford) 2016; 55:461.
  34. Larca LJ, Coppola JT, Honig S. Creatine kinase MB isoenzyme in dermatomyositis: a noncardiac source. Ann Intern Med 1981; 94:341.
  35. Badsha H, Gunes B, Grossman J, Brahn E. Troponin I Assessment of Cardiac Involvement in Patients With Connective Tissue Disease and an Elevated Creatine Kinase MB Isoform Report of Four Cases and Review of the Literature. J Clin Rheumatol 1997; 3:131.
  36. Kiely PD, Bruckner FE, Nisbet JA, Daghir A. Serum skeletal troponin I in inflammatory muscle disease: relation to creatine kinase, CKMB and cardiac troponin I. Ann Rheum Dis 2000; 59:750.
  37. Aggarwal R, Lebiedz-Odrobina D, Sinha A, et al. Serum cardiac troponin T, but not troponin I, is elevated in idiopathic inflammatory myopathies. J Rheumatol 2009; 36:2711.
  38. Love LA, Leff RL, Fraser DD, et al. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine (Baltimore) 1991; 70:360.
  39. Mimori T, Imura Y, Nakashima R, Yoshifuji H. Autoantibodies in idiopathic inflammatory myopathy: an update on clinical and pathophysiological significance. Curr Opin Rheumatol 2007; 19:523.
  40. Targoff IN. Autoantibodies and their significance in myositis. Curr Rheumatol Rep 2008; 10:333.
  41. Katzap E, Barilla-LaBarca ML, Marder G. Antisynthetase syndrome. Curr Rheumatol Rep 2011; 13:175.
  42. Targoff IN. Myositis specific autoantibodies. Curr Rheumatol Rep 2006; 8:196.
  43. Betteridge Z, Gunawardena H, North J, et al. Anti-synthetase syndrome: a new autoantibody to phenylalanyl transfer RNA synthetase (anti-Zo) associated with polymyositis and interstitial pneumonia. Rheumatology (Oxford) 2007; 46:1005.
  44. Mammen AL. Dermatomyositis and polymyositis: Clinical presentation, autoantibodies, and pathogenesis. Ann N Y Acad Sci 2010; 1184:134.
  45. Gerami P, Schope JM, McDonald L, et al. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol 2006; 54:597.
  46. Euwer RL, Sontheimer RD. Amyopathic dermatomyositis (dermatomyositis siné myositis). Presentation of six new cases and review of the literature. J Am Acad Dermatol 1991; 24:959.
  47. Stonecipher MR, Jorizzo JL, White WL, et al. Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis? J Am Acad Dermatol 1993; 28:951.
  48. Sontheimer RD. Clinically amyopathic dermatomyositis: what can we now tell our patients? Arch Dermatol 2010; 146:76.
  49. Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol 1999; 11:475.
  50. Sontheimer RD. Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis siné myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 2002; 46:626.
  51. Galimberti F, Li Y, Fernandez AP. Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy-associated risk factors in a specific tertiary-care-centre cohort. Br J Dermatol 2016; 174:158.
  52. Dawkins MA, Jorizzo JL, Walker FO, et al. Dermatomyositis: a dermatology-based case series. J Am Acad Dermatol 1998; 38:397.
  53. Cao H, Pan M, Kang Y, et al. Clinical manifestations of dermatomyositis and clinically amyopathic dermatomyositis patients with positive expression of anti-melanoma differentiation-associated gene 5 antibody. Arthritis Care Res (Hoboken) 2012; 64:1602.
  54. Ringel RA, Brick JE, Brick JF, et al. Muscle involvement in the scleroderma syndromes. Arch Intern Med 1990; 150:2550.
  55. Hochberg MC, Feldman D, Stevens MB. Adult onset polymyositis/dermatomyositis: an analysis of clinical and laboratory features and survival in 76 patients with a review of the literature. Semin Arthritis Rheum 1986; 15:168.
  56. Reichlin M, Arnett FC Jr. Multiplicity of antibodies in myositis sera. Arthritis Rheum 1984; 27:1150.
  57. Brouwer R, Hengstman GJ, Vree Egberts W, et al. Autoantibody profiles in the sera of European patients with myositis. Ann Rheum Dis 2001; 60:116.
  58. Koenig M, Fritzler MJ, Targoff IN, et al. Heterogeneity of autoantibodies in 100 patients with autoimmune myositis: insights into clinical features and outcomes. Arthritis Res Ther 2007; 9:R78.
  59. Chinoy H, Salway F, Fertig N, et al. In adult onset myositis, the presence of interstitial lung disease and myositis specific/associated antibodies are governed by HLA class II haplotype, rather than by myositis subtype. Arthritis Res Ther 2006; 8:R13.
  60. Kroll M, Otis J, Kagen L. Serum enzyme, myoglobin and muscle strength relationships in polymyositis and dermatomyositis. J Rheumatol 1986; 13:349.
  61. Bombardieri S, Clerico A, Riente L, et al. Circadian variations of serum myoglobin levels in normal subjects and patients with polymyositis. Arthritis Rheum 1982; 25:1419.
  62. Drake LA, Dinehart SM, Farmer ER, et al. Guidelines of care for dermatomyositis. American Academy of Dermatology. J Am Acad Dermatol 1996; 34:824.
  63. Plotz PH, Rider LG, Targoff IN, et al. NIH conference. Myositis: immunologic contributions to understanding cause, pathogenesis, and therapy. Ann Intern Med 1995; 122:715.
  64. Miller T, Al-Lozi MT, Lopate G, Pestronk A. Myopathy with antibodies to the signal recognition particle: clinical and pathological features. J Neurol Neurosurg Psychiatry 2002; 73:420.
  65. Jablonska S, Blaszyk M. Scleromyositis (scleroderma/polimyositis overlap) is an entity. J Eur Acad Dermatol Venereol 2004; 18:265.
  66. Greenberg SA, Sanoudou D, Haslett JN, et al. Molecular profiles of inflammatory myopathies. Neurology 2002; 59:1170.
  67. Mozaffar T, Pestronk A. Myopathy with anti-Jo-1 antibodies: pathology in perimysium and neighbouring muscle fibres. J Neurol Neurosurg Psychiatry 2000; 68:472.
  68. Gunawardena H, Betteridge ZE, McHugh NJ. Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression. Rheumatology (Oxford) 2009; 48:607.
  69. Stone KB, Oddis CV, Fertig N, et al. Anti-Jo-1 antibody levels correlate with disease activity in idiopathic inflammatory myopathy. Arthritis Rheum 2007; 56:3125.
  70. Kalluri M, Sahn SA, Oddis CV, et al. Clinical profile of anti-PL-12 autoantibody. Cohort study and review of the literature. Chest 2009; 135:1550.
  71. Betteridge ZE, Gunawardena H, McHugh NJ. Novel autoantibodies and clinical phenotypes in adult and juvenile myositis. Arthritis Res Ther 2011; 13:209.
  72. Targoff IN, Johnson AE, Miller FW. Antibody to signal recognition particle in polymyositis. Arthritis Rheum 1990; 33:1361.
  73. Hengstman GJ, ter Laak HJ, Vree Egberts WT, et al. Anti-signal recognition particle autoantibodies: marker of a necrotising myopathy. Ann Rheum Dis 2006; 65:1635.
  74. Christopher-Stine L, Casciola-Rosen LA, Hong G, et al. A novel autoantibody recognizing 200-kd and 100-kd proteins is associated with an immune-mediated necrotizing myopathy. Arthritis Rheum 2010; 62:2757.
  75. Kao AH, Lacomis D, Lucas M, et al. Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy. Arthritis Rheum 2004; 50:209.
  76. Seelig HP, Moosbrugger I, Ehrfeld H, et al. The major dermatomyositis-specific Mi-2 autoantigen is a presumed helicase involved in transcriptional activation. Arthritis Rheum 1995; 38:1389.
  77. Casciola-Rosen LA, Pluta AF, Plotz PH, et al. The DNA mismatch repair enzyme PMS1 is a myositis-specific autoantigen. Arthritis Rheum 2001; 44:389.
  78. Sato S, Hirakata M, Kuwana M, et al. Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum 2005; 52:1571.
  79. Sato S, Hoshino K, Satoh T, et al. RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive interstitial lung disease. Arthritis Rheum 2009; 60:2193.
  80. Nakashima R, Imura Y, Kobayashi S, et al. The RIG-I-like receptor IFIH1/MDA5 is a dermatomyositis-specific autoantigen identified by the anti-CADM-140 antibody. Rheumatology (Oxford) 2010; 49:433.
  81. Chen Z, Cao M, Plana MN, et al. Utility of anti-melanoma differentiation-associated gene 5 antibody measurement in identifying patients with dermatomyositis and a high risk for developing rapidly progressive interstitial lung disease: a review of the literature and a meta-analysis. Arthritis Care Res (Hoboken) 2013; 65:1316.
  82. Moghadam-Kia S, Oddis CV, Sato S, et al. Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With Amyopathic and Myopathic Dermatomyositis. Arthritis Care Res (Hoboken) 2016; 68:689.
  83. Hamaguchi Y, Kuwana M, Hoshino K, et al. Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional study. Arch Dermatol 2011; 147:391.
  84. Targoff IN, Mamyrova G, Trieu EP, et al. A novel autoantibody to a 155-kd protein is associated with dermatomyositis. Arthritis Rheum 2006; 54:3682.
  85. Fujimoto M, Hamaguchi Y, Kaji K, et al. Myositis-specific anti-155/140 autoantibodies target transcription intermediary factor 1 family proteins. Arthritis Rheum 2012; 64:513.
  86. Satoh M, Chan JY, Ross SJ, et al. Autoantibodies to transcription intermediary factor TIF1β associated with dermatomyositis. Arthritis Res Ther 2012; 14:R79.
  87. Kaji K, Fujimoto M, Hasegawa M, et al. Identification of a novel autoantibody reactive with 155 and 140 kDa nuclear proteins in patients with dermatomyositis: an association with malignancy. Rheumatology (Oxford) 2007; 46:25.
  88. Chinoy H, Fertig N, Oddis CV, et al. The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis. Ann Rheum Dis 2007; 66:1345.
  89. Gold HL, Smith GP. Clinical significance of p155 antibody in dermatomyositis. Osteoarthritis Dermatol 2013; 1:1.
  90. Betteridge ZE, Gunawardena H, Chinoy H, et al. Clinical and human leucocyte antigen class II haplotype associations of autoantibodies to small ubiquitin-like modifier enzyme, a dermatomyositis-specific autoantigen target, in UK Caucasian adult-onset myositis. Ann Rheum Dis 2009; 68:1621.
  91. Nimelstein SH, Brody S, McShane D, Holman HR. Mixed connective tissue disease: a subsequent evaluation of the original 25 patients. Medicine (Baltimore) 1980; 59:239.
  92. May DA, Disler DG, Jones EA, et al. Abnormal signal intensity in skeletal muscle at MR imaging: patterns, pearls, and pitfalls. Radiographics 2000; 20 Spec No:S295.