eMedicine Specialties > Dermatology > Connective Tissue Diseases

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 13, 2009

Follow-up

Further Inpatient Care

  • Inpatient care is needed for patients with fulminant disease.

Further Outpatient Care

  • Monitoring of the activity of the disease is necessary on at least a monthly basis. Repeat measurements of muscle enzyme levels may aid in the assessment of the activity of the myositis, along with clinical assessment of strength. Machines are available that can aid in the quantification of the strength, but they are not widely used.
  • An assessment of the skin disease is by a physical examination in conjunction with a history. A new validated measure, known as the Cutaneous Dermatomyositis Area and Severity Index (C-DASI), for assessing skin disease might be useful, particularly when performing clinical studies in the future.34
  • Annual physical examinations are useful to monitor for potential toxicity from therapy or for a malignancy.
    • Malignancy evaluations should be conducted for at least the first 3 years following diagnosis and at other times if symptoms develop or the patient's disease is poorly responsive to therapy. The testing selected should be chosen based on the patient's age, sex, race, and other symptoms or findings. After 3 years, patients should be monitored as any other person of the same age, race, and sex.
    • Women should be screened for ovarian cancer.18

Complications

  • Calcinosis may be a complication in children and adolescents with dermatomyositis (see Media File 8). Its presence has been linked to a delay in diagnosis and to therapy that is less aggressive.
Calcinosis due to dermatomyositis in childhood ca...

Calcinosis due to dermatomyositis in childhood can be seen in this patient who had active dermatomyositis 15 years prior to the time of this photograph.

[ CLOSE WINDOW ]
Calcinosis due to dermatomyositis in childhood ca...

Calcinosis due to dermatomyositis in childhood can be seen in this patient who had active dermatomyositis 15 years prior to the time of this photograph.

  • Contractures can occur if the patient is immobile.

Prognosis

  • The prognosis depends on the severity of the myopathy,35  the presence of a malignancy, and/or the presence of cardiopulmonary involvement.
    • Patients with dermatomyositis who have malignancy, cardiac involvement, or pulmonary involvement and those with dermatomyositis who are elderly (>60 y) have a poorer prognosis.
    • The disease may spontaneously remit in as many as 20% of 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.
  • The Myositis Association of America is a resource on inflammatory myopathies for patients and the medical community.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose (Early in the course, the skin disease may be misdiagnosed as eczema, psoriasis, or lupus erythematosus.)
  • Failure to recognize an associated malignancy
  • Failure to inform about or monitor for potential toxicity of therapies
 


More on Dermatomyositis

Overview: Dermatomyositis
Differential Diagnoses & Workup: Dermatomyositis
Treatment & Medication: Dermatomyositis
Follow-up: Dermatomyositis
Multimedia: Dermatomyositis
References
[ CLOSE WINDOW ]

References

  1. Callen JP. Dermatomyositis. Lancet. Jan 1 2000;355(9197):53-7. [Medline].

  2. Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. Sep-Oct 2006;24(5):363-73. [Medline].

  3. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. Feb 13 1975;292(7):344-7. [Medline].

  4. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. Feb 20 1975;292(8):403-7. [Medline].

  5. Airio A, Pukkala E, Isomäki H. Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol. Jul 1995;22(7):1300-3. [Medline].

  6. Chow WH, Gridley G, Mellemkjaer L, McLaughlin JK, Olsen JH, Fraumeni JF Jr. Cancer risk following polymyositis and dermatomyositis: a nationwide cohort study in Denmark. Cancer Causes Control. Jan 1995;6(1):9-13. [Medline].

  7. Hill CL, Zhang Y, Sigurgeirsson B, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. Jan 13 2001;357(9250):96-100. [Medline].

  8. Sigurgeirsson B, Lindelof B, Edhag O, Allander E. Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study. N Engl J Med. Feb 6 1992;326(6):363-7. [Medline].

  9. Sontheimer RD. Skin manifestations of systemic autoimmune connective tissue disease: diagnostics and therapeutics. Best Pract Res Clin Rheumatol. Jun 2004;18(3):429-62. [Medline].

  10. Dalakas MC. Molecular immunology and genetics of inflammatory muscle diseases. Arch Neurol. Dec 1998;55(12):1509-12. [Medline].

  11. Kasteler JS, Callen JP. Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed. JAMA. Dec 28 1994;272(24):1939-41. [Medline].

  12. Okiyama N, Kohsaka H, Ueda N, et al. Seborrheic area erythema as a common skin manifestation in Japanese patients with dermatomyositis. Dermatology. 2008;217(4):374-7. [Medline].

  13. Pachman LM, Veis A, Stock S, et al. Composition of calcifications in children with juvenile dermatomyositis: association with chronic cutaneous inflammation. Arthritis Rheum. Oct 2006;54(10):3345-50. [Medline].

  14. Lutz J, Huwiler KG, Fedczyna T, et al. Increased plasma thrombospondin-1 (TSP-1) levels are associated with the TNF alpha-308A allele in children with juvenile dermatomyositis. Clin Immunol. Jun 2002;103(3 Pt 1):260-3. [Medline].

  15. Werth VP, Callen JP, Ang G, Sullivan KE. Associations of tumor necrosis factor alpha and HLA polymorphisms with adult dermatomyositis: implications for a unique pathogenesis. J Invest Dermatol. Sep 2002;119(3):617-20. [Medline].

  16. Daoud MS, Gibson LE, Pittelkow MR. Hydroxyurea dermopathy: a unique lichenoid eruption complicating long-term therapy with hydroxyurea. J Am Acad Dermatol. Feb 1997;36(2 Pt 1):178-82. [Medline].

  17. Noel B. Lupus erythematosus and other autoimmune diseases related to statin therapy: a systematic review. J Eur Acad Dermatol Venereol. Jan 2007;21(1):17-24. [Medline].

  18. Whitmore SE, Rosenshein NB, Provost TT. Ovarian cancer in patients with dermatomyositis. Medicine (Baltimore). May 1994;73(3):153-60. [Medline].

  19. Callen JP. The value of malignancy evaluation in patients with dermatomyositis. J Am Acad Dermatol. Feb 1982;6(2):253-9. [Medline].

  20. Smith ES, Hallman JR, DeLuca AM, Goldenberg G, Jorizzo JL, Sangueza OP. Dermatomyositis: a clinicopathological study of 40 patients. Am J Dermatopathol. Feb 2009;31(1):61-7. [Medline].

  21. Ang GC, Werth VP. Combination antimalarials in the treatment of cutaneous dermatomyositis: a retrospective study. Arch Dermatol. Jul 2005;141(7):855-9. [Medline].

  22. [Best Evidence] Choy EH, Hoogendijk JE, Lecky B, Winer JB. Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis. Cochrane Database Syst Rev. Jul 20 2005;CD003643. [Medline].

  23. Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Arch Dermatol. Jan 2006;142(1):65-9. [Medline].

  24. Kasteler JS, Callen JP. Low-dose methotrexate administered weekly is an effective corticosteroid-sparing agent for the treatment of the cutaneous manifestations of dermatomyositis. J Am Acad Dermatol. Jan 1997;36(1):67-71. [Medline].

  25. Villalba L, Hicks JE, Adams EM, et al. Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum. Mar 1998;41(3):392-9. [Medline].

  26. Dalakas MC, Illa I, Dambrosia JM, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med. Dec 30 1993;329(27):1993-2000. [Medline].

  27. Woo TY, Callen JP, Voorhees JJ, Bickers DR, Hanno R, Hawkins C. Cutaneous lesions of dermatomyositis are improved by hydroxychloroquine. J Am Acad Dermatol. Apr 1984;10(4):592-600. [Medline].

  28. Zieglschmid-Adams ME, Pandya AG, Cohen SB, Sontheimer RD. Treatment of dermatomyositis with methotrexate. J Am Acad Dermatol. May 1995;32(5 Pt 1):754-7. [Medline].

  29. Rowin J, Amato AA, Deisher N, Cursio J, Meriggioli MN. Mycophenolate mofetil in dermatomyositis: is it safe?. Neurology. Apr 25 2006;66(8):1245-7. [Medline].

  30. Chung L, Genovese MC, Fiorentino DF. A pilot trial of rituximab in the treatment of patients with dermatomyositis. Arch Dermatol. Jun 2007;143(6):763-7. [Medline].

  31. Huber A, Gaffal E, Bieber T, Tuting T, Wenzel J. Treatment of recalcitrant dermatomyositis with efalizumab. Acta Derm Venereol. 2006;86(3):254-5. [Medline].

  32. Oliveri MB, Palermo R, Mautalen C, Hubscher O. Regression of calcinosis during diltiazem treatment in juvenile dermatomyositis. J Rheumatol. Dec 1996;23(12):2152-5. [Medline].

  33. Ambler GR, Chaitow J, Rogers M, McDonald DW, Ouvrier RA. Rapid improvement of calcinosis in juvenile dermatomyositis with alendronate therapy. J Rheumatol. Sep 2005;32(9):1837-9. [Medline].

  34. Klein RQ, Bangert CA, Costner M, et al. Comparison of the reliability and validity of outcome instruments for cutaneous dermatomyositis. Br J Dermatol. Sep 2008;159(4):887-94. [Medline].

  35. Marie I, Hatron PY, Levesque H, et al. Influence of age on characteristics of polymyositis and dermatomyositis in adults. Medicine (Baltimore). May 1999;78(3):139-47. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

dermatomyositis, idiopathic inflammatory myopathy, dermatomyositis sine myositis, amyopathic dermatomyositis, juvenile dermatomyositis, childhood dermatomyositis, polymyositis, vesiculobullous erosive lesions, exfoliative erythroderma, heliotrope rash, Gottron papules, poikiloderma, calcinosis

[ CLOSE WINDOW ]

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

Kathleen David-Bajar, MD, Former Consultant to the Army Surgeon General, Department of Dermatology, Brooke Army Medical Center
Kathleen David-Bajar, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.