Current Rheumatology Reports

, 16:465 | Cite as

Amyopathic Dermatomyositis: Definitions, Diagnosis, and Management

  • Elizabeth E. Bailey
  • David F. Fiorentino
Inflammatory Muscle Disease (RG Cooper, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Inflammatory Muscle Disease


Amyopathic dermatomyositis can be a challenging diagnosis because patients lack traditional muscle findings. “Clinically amyopathic” dermatomyositis (CADM) accounts for the presence of subclinical muscle disease in some of these patients. These patients represent a substantial minority of dermatomyositis cases and have similar co-morbidities to “classic” dermatomyositis patients, including interstitial lung disease and malignancy. Clinically amyopathic dermatomyositis patients should not be considered as a distinct clinical entity from “classic” dermatomyositis, as they share antibody sub-types and associated co-morbidities, likely representing clinical spectrum of a common disease. It is essential for the clinician to be familiar with the clinical presentation of clinically amyopathic dermatomyositis, in order to facilitate early, accurate diagnosis and appropriate clinical management.


Dermatomyositis Clinically amyopathic Hypomyopathic Premyopathic 


Compliance with Ethics Guidelines

Conflict of Interest

Elizabeth E. Bailey and David F. Fiorentino declare that they have no conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


  1. 1.
    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;146:26–30.PubMedCrossRefGoogle Scholar
  2. 2.
    Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med. 1975;292(344–347):403–7.PubMedCrossRefGoogle Scholar
  3. 3.
    Sontheimer RD. Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin. 2002;20:387–408.PubMedCrossRefGoogle Scholar
  4. 4.
    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;54:597–613.PubMedCrossRefGoogle Scholar
  5. 5.
    Ghazi E, Sontheimer RD, Werth VP. The importance of including amyopathic dermatomyositis in the idiopathic inflammatory myositis spectrum. Clin Exp Rheumatol. 2013;31:128–34.PubMedCentralPubMedGoogle Scholar
  6. 6.
    Sontheimer RD. Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis sine myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol. 2002;46:626–36.PubMedCrossRefGoogle Scholar
  7. 7.
    Euwer RL, Sontheimer RD. Amyopathic dermatomyositis (dermatomyositis sine myositis): presentation of six new cases and review of the literature. J Am Acad Dermatol. 1991;24:959–66.PubMedCrossRefGoogle Scholar
  8. 8.
    Euwer RL, Sontheimer RD. Dermatologic aspects of myositis. Curr Opin Rheumatol. 1994;6:583–9.PubMedCrossRefGoogle Scholar
  9. 9.
    El-Azhary RA, Pakzad SY. Amyopathic dermatomyositis: retrospective review of 37 cases. J Am Acad Dermatol. 2002;46:560–5.PubMedCrossRefGoogle Scholar
  10. 10.
    Cao H, Parikh TN, Zheng J. Amyopathic dermatomyositis or dermatomyositis-like skin disease: retrospective review of 16 cases with amyopathic dermatomyositis. Clin Rheumatol. 2009;28:979–84.PubMedCrossRefGoogle Scholar
  11. 11.
    Gitiaux C, Kostallari E, Lafuste P, et al. Whole microvascular unit deletions in dermatomyositis. Ann Rheum Dis. 2013;72:445–52.PubMedCrossRefGoogle Scholar
  12. 12.
    Stonecipher MR, Jorizzo JL, Monu J, Walker F, Sutej PG. Dermatomyositis with normal muscle enzyme concentrations: a single-blind study of the diagnostic value of magnetic resonance imaging and ultrasound. Arch Dermatol. 1994;130:1294–9.PubMedCrossRefGoogle Scholar
  13. 13.
    Lam WWM, Chan H, Chan YL, et al. MR imaging in amyopathic dermatomyositis. Acta Radiol. 1999;40:69.PubMedCrossRefGoogle Scholar
  14. 14.
    Park JH, Olsen NJ, King L, et al. Use of magnetic resonance imaging and P-31 magnetic resonance spectroscopy to detect and quantify muscle dysfunction in the amyopathic and myopathic variants of dermatomyositis. Arthritis Rheum. 1995;38:68–77.PubMedCrossRefGoogle Scholar
  15. 15.
    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;52:1571–6.PubMedCrossRefGoogle Scholar
  16. 16.
    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. 2010;49:433–40.PubMedCrossRefGoogle Scholar
  17. 17.
    Hoshino K, Muro Y, Sugiura K, et al. Anti-MDA5 and anti-TIF1-gamma antibodies have clinical significance for patients with dermatomyositis. Rheumatology. 2010;49:1726–33.PubMedCrossRefGoogle Scholar
  18. 18.
    Koga T, Fujikawa K, Horai Y, et al. The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatology. 2012;51:1278–84.PubMedCrossRefGoogle Scholar
  19. 19.
    Hamaguchi Y, Fujimoto M, Matsushita T, et al. Common and distinct clinical features in adult patients with anti-aminoacyl-tRNA synthetase antibodies: heterogeneity within the syndrome. PLoS ONE. 2013;8:e60442.PubMedCentralPubMedCrossRefGoogle Scholar
  20. 20.
    Cottin V, Thivolet-Bejui F, Reynaud-Gaubert M, et al. Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis, and polymyositis. Eur Respir J. 2003;22:245–50.PubMedCrossRefGoogle Scholar
  21. 21.
    Kang EH, Lee EB, Shin KC, Im CH, Chung DH, Han SK, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis, and amyopathic dermatomyositis. Rheumatology. 2005;44:1282–6.PubMedCrossRefGoogle Scholar
  22. 22.
    Mukae H, Ishimoto H, Sakamoto N, et al. Clinical differences between interstitial lung disease associated with clinically amyopathic dermatomyositis and classic dermatomyositis. Chest. 2009;136:1341–7.PubMedCrossRefGoogle Scholar
  23. 23.
    Yamasaki Y, Yamada H, Ohkubo M, et al. Longterm survival and associated risk factors in patients with adult-onset idiopathic inflammatory myopathies and amyopathic dermatomyositis: experience in a single institute in Japan. J Rheumatol. 2011;38:1636–43.PubMedCrossRefGoogle Scholar
  24. 24.
    Ye S, Chen X, Lu X, et al. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study. Clin Rheumatol. 2007;26:1647–54.PubMedCrossRefGoogle Scholar
  25. 25.
    Chen YJ, Wu CY, Shen JL. Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study. Br J Dermatol. 2001;144:825–31.PubMedCrossRefGoogle Scholar
  26. 26.
    Azuma K, Hidehiro Y, Ohkubo M, et al. Incidence and predictive factors for malignancies in 136 Japanese patients with dermatomyositis, polymyositis, and clinically amyopathic dermatomyositis. Mod Rheumatol. 2011;21:178–83.PubMedCrossRefGoogle Scholar
  27. 27.
    Fung WKJ, Chan HLH, Lam WMW. Amyopathic dermatomyositis in Hong Kong – association with nasopharyngeal carcinoma. Int J Dermatol. 1998;37:659–63.PubMedCrossRefGoogle Scholar
  28. 28.
    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. 2013;65:1316–24.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of DermatologyStanford University School of MedicineRedwood CityUSA

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