Dermatomyositis Treatment & Management

  • Author: Jeffrey P Callen, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Oct 12, 2011
 

Approach Considerations

Therapy for dermatomyositis involves both general measures and specific measures to control the muscle disease and the skin disease. In addition, some patients with dermatomyositis need treatment for other systemic manifestations or complications.

The muscle component is treated by administering corticosteroids, with or without an immunosuppressive agent. The skin disease is treated by avoiding sun exposure and by using sunscreens, topical corticosteroids, antimalarial agents,[27] or an agent such as methotrexate or mycophenolate mofetil.

Surgical care is usually unnecessary in the management of dermatomyositis. Some patients may request surgical removal of local areas of calcinosis. Inpatient care is needed for patients with fulminant dermatomyositis.

Children and adolescents are much more prone to the development of calcinosis. Aggressive and early treatment may prevent this complication.

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General Measures

Several general measures are helpful in the care of patients with dermatomyositis. Bed rest is often valuable for those with severe inflammation of the muscles.

In patients with muscle weakness, especially children, a program of physical therapy is useful to help prevent the contractures that can complicate the disease when patients do not fully move their joints.

For patients with dysphagia, elevating the head of their bed and having them avoid eating prior to bedtime are helpful. These simple maneuvers may prevent aspiration pneumonitis. Occasionally, nasogastric tube feeding is needed to increase caloric input.

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Treatment of Muscle Disease

The mainstay of therapy for the muscle disease is systemically administered corticosteroids.[28, 29, 30] Traditionally, prednisone (0.5-2 mg/kg/d) up to a dose of 60 mg/d is given as initial therapy. The drug should be slowly tapered to avoid relapse of the disease. Because most patients develop steroid-related toxic effects, many authorities administer an immunosuppressive or cytotoxic agent early in the course.[31]

The use of drugs such as methotrexate, azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, leflunomide, and chlorambucil has been reported to be steroid-sparing in some patients or in small open-label studies.[32] [33, 34, 35] Results with cyclophosphamide in severe cases have been disappointing.

For conditions that do not improve, the use of monthly high-dose intravenous immune globulin (IVIG) for 6 months has proved beneficial in the short term.[36, 37] In addition to its positive effects on refractory muscle and skin disease, IVIG has been reported to be beneficial for other systemic manifestations, including severe esophageal dysfunction.[38] One report suggested that rituximab, a chimeric antibody directed against CD20+ B cells, may be effective.[12] However, other reports have failed to produce positive results for the skin.

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Treatment of Skin Disease

Therapy of cutaneous disease of dermatomyositis is often difficult. Some patients with dermatomyositis present primarily with skin disease (ie, amyopathic dermatomyositis [ADM]), whereas others present with a muscle component that is controlled but with significant ongoing skin disease.

First-line therapy is to recognize that the patient is photosensitive and to prescribe sun avoidance and sun protection measures, including broad-spectrum sunscreens. The cutaneous component of dermatomyositis is exacerbated by sunlight and other sources of ultraviolet light; in addition, the muscle component may be exacerbated.

Hydroxychloroquine and chloroquine have been beneficial in small, open-label case studies;[39, 40] however, roughly 25-30% of patients with dermatomyositis who are treated with an antimalarial agent develop a drug eruption.[39]

Methotrexate is useful.[41] Mycophenolate mofetil has been reported to be useful as well.[32, 42, 43, 44, 45] Sirolimus may also be of value in some patients.[46] In addition, small case series or individual reports of successful management with leflunomide have recently appeared in the literature.[35]

IVIG not only benefits the muscle but also clears the skin lesions in the patients in whom it is used. Rituximab has been used for skin disease, but the results are mixed.[13]

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Management of Calcinosis

Calcinosis, a complication of dermatomyositis, is particularly likely to affect children and adolescents. Some believe that aggressive early treatment of the myositis may aid in preventing calcinosis. Once established, the process of calcinosis is debilitating in many patients. Although spontaneous remission is possible, it often takes many years to occur.

The use of the calcium channel blocker diltiazem (240 mg bid) is reportedly associated with gradual resolution of calcinosis in a small number of cases.[47] In addition, the use of an oral bisphosphonate might be helpful.[48] Intravenous (IV) pamidronate has been demonstrated in several cases to result in resolution of the calcinosis.[49] Colchicine, alendronate, and warfarin appear to be potentially beneficial for the resolution of calcinosis, though the data are not conclusive.

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Diet and Activity

A well-balanced diet is useful. Patients with severe muscle inflammation may need extra protein to balance their loss. Patients with dysphagia should avoid eating before bedtime; they may require a special diet, depending on the severity of the esophageal dysfunction.

Activity should be maintained as much as possible in patients with dermatomyositis; however, avoid vigorous physical training should be avoided when the myositis is active. Exercises to maintain the patient’s range of motion are advised. Sun avoidance and sun protection measures are recommended in patients with skin lesions.

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Consultations

Consultations may be indicated with the following:

  • Rheumatologist
  • Dermatologist
  • Neurologist
  • Medical or surgical oncologist (for patients with cancer)
  • Internal medicine specialist or pediatrician (depending on patient age)
  • Pulmonologist

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.

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Long-Term Monitoring

Disease activity must be monitored on at least a monthly basis. Repeat measurements of muscle enzymes may facilitate 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.

The skin component of dermatomyositis is assessed by means physical examination in conjunction with history taking. The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), the Dermatomyositis Skin Severity Index (DSSI), and the Cutaneous Assessment Tool (CAT) skin indices have been developed as outcome instruments. It appears that the CDASI may be a useful outcome measure for studies of cutaneous dermatomyositis; further testing to compare the responsiveness of all 3 measures is necessary.[50]

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 after diagnosis. A report by Hill et al suggested that the risk of malignancy never returns to baseline, even after 3 years; thus, vigilance is still warranted.[5] Selection of testing should be based on 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 in the same manner as any other person of their same age, race, and sex.

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Contributor Information and Disclosures
Author

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), 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 Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh 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: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Additional Contributors

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of 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 for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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. 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].

  4. 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].

  5. 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].

  6. Sigurgeirsson B, Lindelöf 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].

  7. Antiochos BB, Brown LA, Li Z, Tosteson TD, Wortmann RL, Rigby WF. Malignancy is associated with dermatomyositis but not polymyositis in Northern New England, USA. J Rheumatol. Dec 2009;36(12):2704-10. [Medline].

  8. Fardet L, Dupuy A, Gain M, et al. Factors associated with underlying malignancy in a retrospective cohort of 121 patients with dermatomyositis. Medicine (Baltimore). Mar 2009;88(2):91-7. [Medline].

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

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

  11. Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD. 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. Apr 2006;54(4):597-613. [Medline].

  12. Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum. Feb 2005;52(2):601-7. [Medline].

  13. 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].

  14. Banker BQ, Victor M. Dermatomyositis (systemic angiopathy) of childhood. Medicine (Baltimore). Jul 1966;45(4):261-89. [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. 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]. [Full Text].

  17. 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].

  18. 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].

  19. Noël 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].

  20. O'Hanlon T, Koneru B, Bayat E, Love L, Targoff I, Malley J, et al. Immunogenetic differences between Caucasian women with and those without silicone implants in whom myositis develops. Arthritis Rheum. Nov 2004;50(11):3646-50. [Medline].

  21. 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. Jan 2010;146(1):26-30. [Medline]. [Full Text].

  22. Na SJ, Kim SM, Sunwoo IN, Choi YC. Clinical characteristics and outcomes of juvenile and adult dermatomyositis. J Korean Med Sci. Aug 2009;24(4):715-21. [Medline]. [Full Text].

  23. Seidler AM, Gottlieb AB. Dermatomyositis induced by drug therapy: a review of case reports. J Am Acad Dermatol. Nov 2008;59(5):872-80. [Medline].

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

  25. Trallero-Araguás E, Labrador-Horrillo M, Selva-O'Callaghan A, et al. Cancer-associated myositis and anti-p155 autoantibody in a series of 85 patients with idiopathic inflammatory myopathy. Medicine (Baltimore). Jan 2010;89(1):47-52. [Medline].

  26. 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].

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

  28. Iorizzo LJ 3rd, Jorizzo JL. The treatment and prognosis of dermatomyositis: an updated review. J Am Acad Dermatol. Jul 2008;59(1):99-112. [Medline].

  29. Chérin P. [Current therapy for polymyositis and dermatomyositis]. Rev Med Interne. Jun 2008;29 Spec No 2:9-14. [Medline].

  30. Hengstman GJ, van den Hoogen FH, van Engelen BG. Treatment of the inflammatory myopathies: update and practical recommendations. Expert Opin Pharmacother. May 2009;10(7):1183-90. [Medline].

  31. 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].

  32. 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].

  33. 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].

  34. 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].

  35. Boswell JS, Costner MI. Leflunomide as adjuvant treatment of dermatomyositis. J Am Acad Dermatol. Mar 2008;58(3):403-6. [Medline].

  36. 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].

  37. Danieli MG, Calcabrini L, Calabrese V, Marchetti A, Logullo F, Gabrielli A. Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis. Autoimmun Rev. Dec 2009;9(2):124-7. [Medline].

  38. Marie I, Menard JF, Hatron PY, et al. Intravenous immunoglobulins for steroid-refractory esophageal involvement related to polymyositis and dermatomyositis: a series of 73 patients. Arthritis Care Res (Hoboken). Dec 2010;62(12):1748-55. [Medline].

  39. Pelle MT, Callen JP. Adverse cutaneous reactions to hydroxychloroquine are more common in patients with dermatomyositis than in patients with cutaneous lupus erythematosus. Arch Dermatol. Sep 2002;138(9):1231-3; discussion 1233. [Medline].

  40. Woo TY, Callen JP, Voorhees JJ, et al. Cutaneous lesions of dermatomyositis are improved by hydroxychloroquine. J Am Acad Dermatol. Apr 1984;10(4):592-600. [Medline].

  41. 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].

  42. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology (Oxford). Mar 2005;44(3):386-9. [Medline].

  43. Pisoni CN, Cuadrado MJ, Khamashta MA, Hughes GR, D'Cruz DP. Mycophenolate mofetil treatment in resistant myositis. Rheumatology (Oxford). Mar 2007;46(3):516-8. [Medline].

  44. 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].

  45. Waldman MA, Callen JP. Self-resolution of Epstein-Barr virus-associated B-cell lymphoma in a patient with dermatomyositis following withdrawal of mycophenolate mofetil and methotrexate. J Am Acad Dermatol. Aug 2004;51(2 Suppl):S124-30. [Medline].

  46. Nadiminti U, Arbiser JL. Rapamycin (sirolimus) as a steroid-sparing agent in dermatomyositis. J Am Acad Dermatol. Feb 2005;52(2 Suppl 1):17-9. [Medline].

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

  48. 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].

  49. Slimani S, Abdessemed A, Haddouche A, Ladjouze-Rezig A. Complete resolution of universal calcinosis in a patient with juvenile dermatomyositis using pamidronate. Joint Bone Spine. Jan 2010;77(1):70-2. [Medline].

  50. 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]. [Full Text].

  51. The Muscle Study Group. A randomized, pilot trial of etanercept in dermatomyositis. Ann Neurol. Sep 2011;70(3):427-436. [Medline]. [Full Text].

 
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Heliotrope flower, for which characteristic manifestation of dermatomyositis is named.
Heliotrope rash in a woman with dermatomyositis.
Gottron papules and nailfold telangiectasia are present in this patient with dermatomyositis.
These lesions on dorsal hands demonstrate photodistribution of dermatomyositis. Note sparing of interdigital web spaces.
Diffuse alopecia with scaly scalp dermatosis is common in patients with dermatomyositis.
Dermatomyositis is often associated with a poikiloderma in a photodistribution.
Histopathology of dermatomyositis is interface dermatitis.
Calcinosis caused by dermatomyositis in childhood can be observed in patient who had active dermatomyositis 15 years before time of this photograph.
Histopathology of dermatomyositis showing inflammatory myopathic changes with a predominantly perivascular chronic inflammatory infiltrate.
Calcifying panniculitis in patient with dermatomyositis.
Ulceration over dorsal and lateral fingers in patient with dermatomyositis.
Hematoxylin and eosin paraffin section shows polymyositis. Longitudinal section shows dense, chronic, endomysial inflammatory infiltrate. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin frozen section shows polymyositis. Endomysial chronic inflammation is present among intact myofibers that are remarkable only for increased variability of fiber size. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin section shows polymyositis. Patient had dense endomysial inflammation that contains abundance of plasma cells, which can be observed in patients with chronic polymyositis. Two necrotic myofibers, characterized by dense eosinophilic staining, are observed. Focal fatty infiltration of muscle is present in lower left quadrant of photomicrograph. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin section shows polymyositis. Photomicrograph illustrates attack on nonnecrotic myofiber by autoaggressive T lymphocytes. On left, central myofiber is intact. On right, it is obliterated by segmental inflammatory attack. If immunohistochemistry were performed, expected findings would include admixture of CD8 T lymphocytes and macrophages in inflammatory process. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin shows dermatomyositis. In dermatomyositis, inflammation is characteristically perivascular and perimysial. Vessel oriented approximately vertically in center has mild perivascular chronic inflammatory infiltrate. Endothelium is plump; wall is not necrotic. A few lymphocytes in wall of vessel are probably in transit from lumen to external aspect of vessel. Some observers may interpret this finding as vasculitis, but it is certainly neither necrotizing vasculitis nor arteritis. Image courtesy of Roberta J. Seidman, MD.
Hematoxylin and eosin frozen section shows perifascicular atrophy in dermatomyositis. Fascicles in this sample show atrophy, predominantly at periphery, along connective-tissue border. Ischemia is considered to cause perifascicular atrophy. This finding is characteristic of dermatomyositis, mostly associated with juvenile form but also observed in adult form. Image courtesy of Roberta J Seidman, MD.
Immunofluorescence for membrane attack complex of complement (MAC) in dermatomyositis. Bright ring of yellow-green fluorescence at center represents MAC in wall of microvessel. Finding was not present after treatment with steroids. Image courtesy of Roberta J Seidman, MD.
 
 
 
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