Clinical Rheumatology

, Volume 28, Issue 8, pp 979–984 | Cite as

Amyopathic dermatomyositis or dermatomyositis-like skin disease: retrospective review of 16 cases with amyopathic dermatomyositis

  • Hua CaoEmail author
  • Tanvi N. Parikh
  • Jie Zheng
Brief Report


Amyopathic dermatomyositis (ADM) is characterized by the presence of dermatomyositis (DM) for 6 months or more in individuals who have normal muscle enzymes and no clinically significant muscle weakness. The aim of the study was to investigate the initial laboratory data, clinical manifestations, complications, and clinical outcomes of patients with the diagnosis of ADM. We reported 16 cases with the cutaneous findings of dermatomyositis without clinical or laboratory evidence of muscle disease for at least 2 years after onset of the skin manifestations in the Department of Dermatology and Rheumatology at Shanghai Ruijin Hospital between 1998 and 2004. All patients had Gottron’s papules, periungual erythema/telangiectasia, and violaceous discoloration of the face, neck, upper chest, and back at some time during the course of their disease. Follow-up of 1 to 10 years after diagnosis found muscle weakness in three patients (18.75%) within 5 years of diagnosis. One patient (6.15%) was rediagnosed as chronic cutaneous lupus erythematosus (CCLE). Four patients (25%) had associated malignancies. Twelve patients (75%) had radiographic evidence indicative of interstitial fibrosis irrespective of respiratory symptoms. Patients with ADM appear to be at risk for developing the same potentially fatal disease complications as those patients with DM (e.g., interstitial lung disease and internal malignancy). These cases further emphasize that the cutaneous manifestations of dermatomyositis are pathognomonic for DM and we propose the term dermatomyositis-like skin disease as a better designation than amyopathic dermatomyositis to describe this distinctive subset of cutaneous symptoms. Dermatomyositis-like skin disease is a complex syndrome, which includes the characteristic cutaneous eruption of dermatomyositis without clinical evidence of muscle disease. Our findings suggest that patients diagnosed with this syndrome are at risk for fatal interstitial lung disease, malignancy, and/or delayed onset of DM or CCLE. Cautious systematic clinical trials should be considered for this group of patients.


Amyopathic dermatomyositis Dermatomyositis-like skin disease Interstitial lung disease Malignancy 





  1. 1.
    Pearson CM (1979) Polymyositis and dermatomyositis. In: McCarty DJ (ed) Arthritis and allied conditions: a textbook of rheumatology, 9th edn. Lea & Febiger, Philadelphia, pp 7412–7461Google Scholar
  2. 2.
    Kovacs SO, Kovacs SC (1998) Dermatomyositis. J Am Acad Dermatol 39:899–922PubMedCrossRefGoogle Scholar
  3. 3.
    Sontheimer RD (1999) Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol 11:475–482PubMedCrossRefGoogle Scholar
  4. 4.
    Euwer RL, Sontheimer RD (1991) Amyopathic dermatomyositis (dermatomyositis siné myositis). Presentation of six new cases and review of the literature. J Am Acad Dermatol 24:959–966PubMedCrossRefGoogle Scholar
  5. 5.
    Euwer RL, Sontheimer RD (1993) Amyopathic dermatomyositis: a review. J Invest Dermatol 100:124S–127SPubMedCrossRefGoogle Scholar
  6. 6.
    Sontheimer RD (2002) 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 46:626–636PubMedCrossRefGoogle Scholar
  7. 7.
    Gerami P, Schope JM, McDonald L et al (2006) 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 54:597–613PubMedCrossRefGoogle Scholar
  8. 8.
    Lam WW, Chan H, Chan YL et al (1999) MR imaging in amyopathic dermatomyositis. Acta Radiologica 40:69–72PubMedGoogle Scholar
  9. 9.
    Sontheimer RD (2002) Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin 20:387–408PubMedCrossRefGoogle Scholar
  10. 10.
    Chen YJ, Wu CY, Shen JL (2001) Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study. Br J Dermatol 144:825–831PubMedCrossRefGoogle Scholar
  11. 11.
    Peloro TM, Miller OF, Hahn TF et al (2001) Juvenile dermatomyositis: a retrospective review of a 30-year experience. J Am Acad Dermatol 45:28–34PubMedCrossRefGoogle Scholar
  12. 12.
    Won Huh J, Soon Kim D, Keun Lee C et al (2007) Two distinct clinical types of interstitial lung disease associated with polymyositis dermatomyositis. Respir Med 101:1761–1769PubMedCrossRefGoogle Scholar
  13. 13.
    Ye S, Chen XX, Lu XY et al (2007) Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study. Clin Rheumatol 26:1647–1654PubMedCrossRefGoogle Scholar
  14. 14.
    Marie I, Hachulla E, Cherin P et al (2002) Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 47:614–622PubMedCrossRefGoogle Scholar
  15. 15.
    Ideura G, Hanaoka M, Koizumi T et al (2007) Interstitial lung disease associated with amyopathic dermatomyositis: review of 18 cases. Respir Med 101:1406–1411PubMedCrossRefGoogle Scholar
  16. 16.
    Ozawa Y, Kurosaka D, Yokoyama T et al (2000) Therapeutic efficacy of cyclosporin A in four cases of amyopathic dermatomyositis with rapidly progressive interstitial pneumonia. Ryumachi 40:798–809PubMedGoogle Scholar
  17. 17.
    Takashi S, Okubo Y, Yamazaki Y et al (1999) Amyopathic dermatomyositis with interstitial pneumonia: effective treatment with cyclophosphamide pulse therapy. Nihon Kokyuki Gakkai Zasshi 37:647–651PubMedGoogle Scholar
  18. 18.
    Peng JC, Sheen TS, Hsu MM (1995) Nasopharyngeal carcinoma with dermatomyositis: analysis of 12 cases. Arch Otolaryngol Head Neck Surg 121:1298–1301PubMedGoogle Scholar
  19. 19.
    Richardson JB, Callen JP (1989) Dermatomyositis and malignancy. Med Clin North Am 73:1211–1220PubMedGoogle Scholar
  20. 20.
    Airio A, Pukkala E, Isomaki H (1995) Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol 22:1300–1303PubMedGoogle Scholar
  21. 21.
    El-Azhary RA, Pakzad SY (2002) Amyopathic dermatomyositis: retrospective review of 37 cases. J Am Acad Dermatol 46:560–565PubMedCrossRefGoogle Scholar
  22. 22.
    Sontheimer RD (2004) The management of dermatomyositis: current treatment options. Expert Opin Pharmacother 5:1083–1099PubMedCrossRefGoogle Scholar
  23. 23.
    Callen JP (2002) When and how should the patient with dermatomyositis or amyopathic dermatomyositis be assessed for possible cancer? Arch Dermatol 138:969–971PubMedCrossRefGoogle Scholar
  24. 24.
    Targoff IN, Trieu EP, Sontheimer RD et al (2000) Autoantibodies to 155 kD and Se antigens in patients with clinically-amyopathic dermatomyositis [abstract]. Arthritis Rheum 43(Suppl 9):S194Google Scholar

Copyright information

© Clinical Rheumatology 2009

Authors and Affiliations

  1. 1.Department of Dermatology and Rheumatology, Ruijin HospitalMedical School of Shanghai Jiao Tong UniversityShanghaiChina
  2. 2.Molecular Biology ProgramMemorial Sloan-Kettering Cancer CenterNew YorkUSA

Personalised recommendations