dermatomyositis

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Introduction

Atrophy, edema or fibrosis of the skin accompanied by non-suppurative inflammation of striated muscle.

Dermatitis & polymyositis generally become apparent at the same time; however, skin or muscle involvement alone may occur initially.

* features distinguishing dermatomyositis from polymyositis

Etiology

Epidemiology

  • all ages
  • adults: female > male

Pathology

Genetics

  • no familial association[4]

Clinical manifestations

* virtually diagnostic for dermatomyositis[4]

# images[24][25]

Diagnostic criteria

Laboratory

* features distinguishing dermatomyositis from polymyositis

Diagnostic procedures

Radiology

* essential

Complications

Differential diagnosis

Management

More general terms

More specific terms

Additional terms

References

  1. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 878
  2. Color Atlas and Synopsis of Clinical Dermatology, Common and Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 328-331
  3. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 788-89
  4. 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.12 4.13 4.14 4.15 4.16 4.17 4.18 4.19 4.20 4.21 4.22 4.23 4.24 4.25 4.26 4.27 4.28 4.29 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
    Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  5. Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol. 2006 Apr;54(4):597-613. Epub 2006 Jan 23. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/16546580
  6. Fathi M, Lundberg IE, Tornling G. Pulmonary complications of polymyositis and dermatomyositis. Semin Respir Crit Care Med. 2007 Aug;28(4):451-8. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17764062
  7. Ytterberg SR. Treatment of refractory polymyositis and dermatomyositis. Curr Rheumatol Rep. 2006 Jun;8(3):167-73. PMID: https://www.ncbi.nlm.nih.gov/pubmed/16901073
  8. Iorizzo LJ 3rd, Jorizzo JL. The treatment and prognosis of dermatomyositis: an updated review. J Am Acad Dermatol. 2008 Jul;59(1):99-112 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18423790
  9. Callen JP. Cutaneous manifestations of dermatomyositis and their management. Curr Rheumatol Rep. 2010 Jun;12(3):192-7 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20425525
  10. Fardet L, Dupuy A, Gain M, Kettaneh A et al Factors associated with underlying malignancy in a retrospective cohort of 121 patients with dermatomyositis. Medicine (Baltimore). 2009 Mar;88(2):91-7 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19282699
  11. Fathi M, Vikgren J, Boijsen M et al Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology. Arthritis Rheum. 2008 May 15;59(5):677-85. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18438901
  12. Gunawardena H, Betteridge ZE, McHugh NJ. Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression. Rheumatology (Oxford). 2009 Jun;48(6):607-12 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19439503
  13. Lundberg IE, Forbess CJ. Mortality in idiopathic inflammatory myopathies. Clin Exp Rheumatol. 2008 Sep-Oct;26(5 Suppl 51):S109-14. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19026152
  14. Selva-O'Callaghan A, Grau JM, Gamez-Cenzano C, Conventional cancer screening versus PET/CT in dermatomyositis/ polymyositis. Am J Med. 2010 Jun;123(6):558-62. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20569766
  15. Walker UA. Imaging tools for the clinical assessment of idiopathic inflammatory myositis. Curr Opin Rheumatol. 2008 Nov;20(6):656-61 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18946324
  16. 16.0 16.1 Amato AA, Griggs RC. Treatment of idiopathic inflammatory myopathies. Curr Opin Neurol. 2003 Oct;16(5):569-75. PMID: https://www.ncbi.nlm.nih.gov/pubmed/14501840
  17. 17.0 17.1 Hill CL, Zhang Y, Sigurgeirsson B et al Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001 Jan 13;357(9250):96-100. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11197446
  18. Airio A , Kautiainen H, Hakala M. Prognosis and mortality of polymyositis and dermatomyositis patients. Clin Rheumatol. 2006 Mar;25(2):234-9. Epub 2006 Feb 14 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16477398
  19. 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 Jul;68(7):1192-6 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18713785
  20. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003 Sep 20;362(9388):971-82. PMID: https://www.ncbi.nlm.nih.gov/pubmed/14511932
  21. Marie I, Hachulla E, Hatron PY et al Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis. J Rheumatol. 2001 Oct;28(10):2230-7. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11669162
  22. 22.0 22.1 Morganroth PA, Kreider ME, Okawa J, Taylor L, Werth VP. Interstitial lung disease in classic and skin-predominant dermatomyositis: a retrospective study with screening recommendations. Arch Dermatol. 2010 Jul;146(7):729-38. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20644033
  23. Zaba LC, Fiorentino DF. Skin disease in dermatomyositis. Curr Opin Rheumatol. 2012 Nov;24(6):597-601. PMID: https://www.ncbi.nlm.nih.gov/pubmed/22907594
  24. 24.0 24.1 Femia AN, Diamond HS (images) Medscape: Dermatomyositis http://emedicine.medscape.com/article/332783-overview
  25. 25.0 25.1 DermNet NZ. Dermatomyositis (images) http://dermnetnz.org/immune/dermatomyositis.html
  26. Iaccarino L, Ghirardello A, Bettio S et al The clinical features, diagnosis and classification of dermatomyositis. J Autoimmun. 2014 Feb-Mar;48-49:122-7. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24467910
  27. 27.0 27.1 Charrow A, Vleugels, RA Cutaneous Ulcerations in Anti-MDA5 Dermatomyositis N Engl J Med 2019; 381:465. August 1, 2019 PMID: https://www.ncbi.nlm.nih.gov/pubmed/31365803 https://www.nejm.org/doi/full/10.1056/NEJMicm1816147
  28. 28.0 28.1 Ingram I FDA Approves IVIG for Dermatomyositis. Octagam 10% lands indication for adults following results of ProDERM trial. MedPage Today July 20, 2021 https://www.medpagetoday.com/rheumatology/generalrheumatology/93660
  29. 29.0 29.1 29.2 NEJM Knowledge+ Question of the Week. March 19, 2024 https://knowledgeplus.nejm.org/question-of-week/354/
    Olazagasti JM, Baez PJ, Wetter DA, Ernste FC Cancer risk in dermatomyositis: a meta-analysis of cohort studies. Am J Clin Dermatol. 2015 Apr;16(2):89-98. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25721032
    Oldroyd AGS, Allard AB, Callen JP et al A systematic review and meta-analysis to inform cancer screening guidelines in idiopathic inflammatory myopathies. Rheumatology (Oxford). 2021 Jun 18;60(6):2615-2628. PMID: https://www.ncbi.nlm.nih.gov/pubmed/33599244 PMCID: PMC8213426 Free PMC article.
    Cobos GA, Femia A, Vleugels RA. Dermatomyositis: An Update on Diagnosis and Treatment. Am J Clin Dermatol. 2020 Jun;21(3):339-353. PMID: https://www.ncbi.nlm.nih.gov/pubmed/32096127 Review.
  30. 30.0 30.1 30.2 Stone CJ, Faden DF, Xie L et al Application of Risk-Based Cancer Screening in Patients With Dermatomyositis. JAMA Dermatol. 2024 Sep 25:e243355.. PMID: https://www.ncbi.nlm.nih.gov/pubmed/39320903
    Maderal AD, Femia A. Clinical Application of New Risk-Based Cancer Screening in Patients With Dermatomyositis. JAMA Dermatol. 2024 Sep 25. https://jamanetwork.com/journals/jamadermatology/fullarticle/2823599 PMID: https://www.ncbi.nlm.nih.gov/pubmed/39320904 No abstract available. https://jamanetwork.com/journals/jamadermatology/fullarticle/2823601
  31. Oldroyd AGS, Callen JP, Chinoy H et al International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening: an International Myositis Assessment and Clinical Studies Group (IMACS) initiative. International Myositis Assessment and Clinical Studies Group Cancer Screening Expert Group; Aggarwal R. Nat Rev Rheumatol. 2023 Dec;19(12):805-817. PMID: https://www.ncbi.nlm.nih.gov/pubmed/37945774 Review. https://www.nature.com/articles/s41584-023-01045-w
  32. NINDS Dermatomyositis Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Dermatomyositis-Information-Page

Patient information

dermatomyositis patient information