eMedicine Specialties > Rheumatology > Systemic Rheumatic Disease

Dermatomyositis: Follow-up

Author: Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Contributor Information and Disclosures

Updated: Apr 14, 2009

Follow-up

Further Inpatient Care

  • Inpatient care is needed for patients with fulminant dermatomyositis (DM).

Further Outpatient Care

  • Monitoring disease activity is necessary at least on a monthly basis. Repeat measurements of muscle enzymes may aid in assessment of the activity of the myositis, along with clinical assessment of patients' strength. Machines that can aid in the quantification of strength are available but are not used widely.
  • Skin disease of dermatomyositis is assessed with physical examination in conjunction with history. A system for judging the activity and damage associated with dermatomyositis is being developed.
  • Annual physical examinations are useful to monitor for potential toxicity due to therapy or for the presence of a malignancy.
  • Malignancy evaluations should be conducted for at least the first 3 years following diagnosis. A report by Hill et al (2001) suggested that the risk of malignancy never returns to baseline, even after 3 years; thus, vigilance is still warranted.17 The testing selected should be chosen based n the patient's age, sex, race, and other symptoms or findings. Female patients with dermatomyositis should be screened for ovarian cancer. After 3 years, patients should be monitored as any other person of their same age, race, and sex.

Inpatient & Outpatient Medications

  • Prednisone is a first-line therapy for dermatomyositis. The dose is altered depending on the response of the patient's condition.
  • Immunosuppressive/cytotoxic agents are used as steroid-sparing agents for muscle disease of dermatomyositis. Methotrexate has been demonstrated to be useful for skin disease, even in the absence of significant muscle disease. Mycophenolate mofetil may also be useful for cutaneous disease.
  • Antimalarials, particularly hydroxychloroquine, may be useful for cutaneous disease. Patients with dermatomyositis may be at an increased risk of drug eruptions.
  • Intravenous immunoglobulin is used in patients in whom therapy with corticosteroids and immunosuppressives fails. This agent is useful for both the skin and the muscles.
  • Biologic therapies including rituximab and TNF antagonists might be useful. However, their widespread use is discouraged until adequate, preferably controlled studies, demonstrate efficacy and safety.

Transfer

  • Patients with dermatomyositis may be served better by a physician or a team of physicians who have experience in managing this relatively rare disorder. Transfer to a tertiary center is often warranted for initial care and even for follow-up care.

Deterrence/Prevention

  • Skin disease of dermatomyositis is exacerbated by sunlight and other sources of ultraviolet light. In addition, muscle disease may be exacerbated. Sunscreens and sun protective measures are helpful in avoiding flares of dermatomyositis.

Complications

  • Calcinosis may complicate dermatomyositis in children and adolescents.
  • Contractures can occur if the patient is immobile.

Prognosis

  • Patients with dermatomyositis who have malignancy, cardiac involvement, or pulmonary involvement or who are elderly (ie, >60 y) have a poorer prognosis.
  • The disease may spontaneously remit in as many as 20% of affected patients. About 5% of patients have a fulminant progressive course with eventual death. Therefore, many patients require long-term therapy.

Patient Education

  • Physical therapy and rehabilitative measures are necessary in selected patients.
  • Sun protective measures are necessary for patients with skin disease.
  • Patients may visit The Myositis Association Web site for more information.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose: Skin disease of dermatomyositis may be misdiagnosed as eczema, psoriasis, or LE early in the disease course.
  • Failure to recognize an associated malignancy
  • Failure to inform the patient about the potential toxicity of therapies

Special Concerns

  • In older patients with dermatomyositis, the potential for an associated malignancy increases. Assessment for malignancy should be performed regularly, and the frequency should be based on the patient's sex, age, and race.
  • Children and adolescents are much more prone to the development of calcinosis. Aggressive and early treatment may prevent this complication.
 


More on Dermatomyositis

Overview: Dermatomyositis
Differential Diagnoses & Workup: Dermatomyositis
Treatment & Medication: Dermatomyositis
Follow-up: Dermatomyositis
Multimedia: Dermatomyositis
References
Further Reading
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References

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Further Reading

The reader is referred to articles by Callen and Wortmann (2006), 18 Iorizzo and Joizzo (2008), 19 and Krathen et al (2008) 20 for further reading.

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Keywords

dermatomyositis, idiopathic inflammatory myopathy, IIM, dermatomyositis sine myositis, amyopathic dermatomyositis, ADM, juvenile dermatomyositis, childhood dermatomyositis, DM, polymyositis, PM, DM-sine myositis, myositis with malignancy, childhood DM, childhood PM, myositis, postmyopathic DM, postmyopathic dermatomyositis, complement-mediated vascular inflammation, terminal attack complex, calcinosis, heliotrope rash, Gottron papules

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Contributor Information and Disclosures

Author

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

Medical Editor

Kristine M Lohr, MD, MS, Program Director, Professor, Department of Internal Medicine, Division of Rheumatology and Women's Health, University of Kentucky School of Medicine
Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Medical Women's Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center
Lawrence H Brent, MD is a member of the following medical societies: American Association of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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