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Dermatomyositis Treatment & Management

  • Author: Alisa N Femia, MD; Chief Editor: Herbert S Diamond, MD  more...
Updated: Jan 20, 2016

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, typically with an immunosuppressive agent. The skin disease is treated by avoiding sun exposure and by using sunscreens and photoprotective clothing, as well as with topical corticosteroids, antimalarial agents,[58] and immunomodulatory medications such as methotrexate, mycophenolate mofetil, or intravenous immunoglobulin.

Surgical care is usually unnecessary in the management of dermatomyositis. Some patients may benefit from surgical removal of focal areas of calcinosis, particularly those that are painful. Inpatient care is needed for patients with fulminant dermatomyositis with muscle and/or internal organ involvement.

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


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. Rehabilitative exercise is also recommended for both adult and pediatric patients in order to maintain muscle strength, even during the course of active muscle disease.

For patients with dysphagia and/or gastroesophageal reflux, elevation of the head of their bed and avoidance of eating prior to bedtime are helpful. These simple maneuvers may prevent aspiration pneumonitis. Occasionally, nasogastric tube feeding is needed to increase caloric input.


Treatment of Muscle Disease

The mainstay of therapy for the muscle disease is systemically administered corticosteroids.[59, 60, 61] 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, most authorities administer a steroid-sparing immunosuppressive or cytotoxic agent early in the course.[62] Drugs reported to be steroid-sparing in some patients or in small open-label studies have included the following[63, 64, 65, 66] :

  • Methotrexate
  • Azathioprine
  • Cyclophosphamide
  • Cyclosporine
  • Mycophenolate mofetil
  • Leflunomide
  • Chlorambucil
  • Tacrolimus

Generally, methotrexate, mycophenolate mofetil, or azathioprine are used first line as glucocorticoid-sparing agents for muscle involvement. Response rates to methotrexate have been reported to be approximately 70-80%.[67] In addition, one small, randomized trial supported the long-term benefits of azathioprine as compared with prednisone monotherapy.[68] Results with cyclophosphamide in severe cases have been disappointing.

For refractory cases, the use of monthly high-dose intravenous immune globulin (IVIG) for 6 months has proved beneficial in the short term.[69, 70] 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.[71]

Rituximab, a chimeric antibody directed against CD20+ B cells, may be effective, but results have been mixed.[18, 72] In a recent multicenter, randomized, double-blind placebo-controlled trial of 44 weeks of rituximab therapy in patients with dermatomyositis and polymyositis, most patients experienced improvement in muscle disease activity; however, no significant differences were noted between groups based on muscle parameters. The study had a delayed-start design, with rituximab started immediately in one arm and after 8 weeks in the second arm, which is speculated to have influenced the results.[72]

Recently, an analysis of 195 patients with polymyositis and dermatomyositis looked to determine predictors of response to rituximab, and found that antisynthetase and anti-Mi2 autoantibodies, as well as lower disease damage and juvenile-onset disease, were predictors of clinical improvement with rituximab.[73]

The calcineurin inhibitor tacrolimus appears to be effective, safe, and well tolerated in patients with dermatomyositis that is refractory to other treatments. In addition to improvement in muscle strength and physical function, amelioration of skin lesions and interstitial lung disease have been reported. However, randomized, controlled trials have yet to be conducted.[74]


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 and photoprotective clothing. 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;[75, 76] however, roughly 25-30% of patients with dermatomyositis who are treated with hydroxychloroquine develop a drug eruption, and patients should be counseled regarding this potential side effect.[75] Some patients who develop a drug eruption to hydroxychoroquine may go on to tolerate chloroquine.[75]

Methotrexate is often considered first-line systemic therapy if antimalarials fail or are contraindicated.[77] Mycophenolate mofetil has been reported to be useful as well.[63, 78, 79, 80, 81]

Azathioprine has been reported to be effective for muscle involvement, but there is a paucity of literature regarding cutaneous response. Azathioprine appears to be less effective for cutaneous disease. Sirolimus, tactrolimus, and dapsone inhibitors are among other immunomodulatory medications that may be of value in some patients.[82, 83, 84, 74] In addition, small case series or individual reports of successful management with leflunomide have also appeared in the literature.[66]

Intravenous immune globulin (IVIG) has benefited muscle involvement and cleared the skin lesions in the patients in whom it was used. A retrospective evaluation of 42 patients with dermatomyositis compared 24 patients treated with IVIG as an add-on to conventional immunosuppressive therapies to those treated with conventional immunosuppression alone. This study found that muscular and cutaneous involvement were significantly improved at 6 months in the IVIG-treated group, and modified Cutaneous Dermatomyositis Area and Severity Index (CDASI) scores were significantly improved over pretreatment scores during 4 years of follow-up, despite no significant difference in cutaneous remission rates and a high cutaneous relapse rate.[85]

Subcutaneous IgG has also been effective in dermatomyositis.[86] Rituximab has been used for skin disease, but the results are mixed.[19, 72]


Management of Calcinosis

Calcinosis, a complication of dermatomyositis, is particularly likely to affect children and adolescents. Some experts 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.[87] In addition, the use of an oral bisphosphonate might be helpful.[88] Intravenous (IV) pamidronate has been demonstrated in several cases to result in resolution of the calcinosis.[89] Colchicine, alendronate, and warfarin appear to be potentially beneficial for the resolution of calcinosis, although the data are not conclusive.

Some patients with localized areas of calcinosis may wish to have the calcinotic nodules surgically removed, particularly if they are tender. A retrospective study from the Mayo clinic of 78 patients with dystrophic calcinosis in the setting of autoimmune connective-tissue disease (of which dermatomyositis and systemic sclerosis were the most common underlying diseases) found that surgical excision and diltiazem produced the best results in diminishing calcinosis.[90]


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.

Sun avoidance and sun protection measures are recommended in patients with skin lesions. Patients with dermatomyositis should maintain activity as much as possible. Although vigorous physical training should be avoided when the myositis is active, a rehabilitative exercise regimen is typically still recommended during the course of active muscle disease. Exercises to maintain the patient's range of motion are also advised.

Resistance training and aerobic exercise may also be beneficial for muscle involvement, especially if instituted early. In a recent randomized controlled trial, 12-week endurance exercise yielded improvement in muscle performance, Vo2 max, and activities of daily living as compared with a non-exercising control group. In a 1-year open-label extension follow-up, improvement in muscle performance was sustained, but other assessments returned to baseline, emphasizing the need for a continued exercise program.[91]

Another recent randomized controlled trial of 19 patients compared patients with recent-onset polymyositis/dermatomyositis assigned to 12 weeks of resistive home exercise with telephone support, along with encouragement for another 12 weeks of home or gym exercise, with a control group assigned to 24 weeks of range of motion exercises. Both groups experienced improvement in muscle performance and aerobic capacity, but there were no significant differences between groups. In an open-label 2-year follow-up, the exercise group maintained significant improvement in muscle performance and aerobic capacity compared to baseline, suggesting that sustained exercise may be of some benefit.[92]



Consultations with the following specialists may be indicated:

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

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.


Long-Term Monitoring

Disease activity must be closely monitored. Repeat measurements of muscle enzymes and clinical assessment of patients' strength may facilitate assessment of the activity of the myositis. 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. The CDASI has been found to be valid and responsive for characterizing cutaneous dermatomyositis severity and detecting improvement in disease activity.[93] Further testing to compare the responsiveness of all three measures is necessary.[94]

Annual physical examinations are useful to monitor for potential toxicity due to therapy or for the presence of a malignancy.

Malignancy evaluations, including imaging studies as noted above, should be conducted annually 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, continued vigilance is warranted.[7]

Selection of testing should be based on the patient's age, sex, race, and other symptoms or findings. Typically, a workup more extensive than age-appropriate screening is recommended. The principal malignancies associated with dermatomyositis are ovarian cancer and breast cancer in females and lung cancer in males.[95] After 3-5 years, patients should be monitored in the same manner as any other person of their same age, race, and sex.


Further Reading

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

Contributor Information and Disclosures

Alisa N Femia, MD Assistant Professor, Ronald O Perelman Department of Dermatology, New York University Medical Center

Alisa N Femia, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Medical Dermatology Society, Rheumatologic Dermatology Society

Disclosure: Nothing to disclose.


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, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Ruth Ann Vleugels, MD, MPH Assistant Professor of Dermatology, Harvard Medical School; Associate Physician, Department of Dermatology, Brigham and Women's Hospital; Associate Physician, Department of Immunology and Allergy, Children's Hospital Boston

Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology Foundation

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.


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

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

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.

Jeffrey Meffert, MD Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at 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.

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

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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.

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

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

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

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

  5. Airio A, Pukkala E, Isomäki H. Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol. 1995 Jul. 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. 1995 Jan. 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. 2001 Jan 13. 357(9250):96-100. [Medline].

  8. 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. 1992 Feb 6. 326(6):363-7. [Medline].

  9. 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. 2009 Dec. 36(12):2704-10. [Medline].

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

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

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

  13. Sontheimer RD. 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. 2002 Apr. 46(4):626-36. [Medline].

  14. 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 Jan. 146(1):26-30. [Medline]. [Full Text].

  15. Klein RQ, Teal V, Taylor L, Troxel AB, Werth VP. Number, characteristics, and classification of patients with dermatomyositis seen by dermatology and rheumatology departments at a large tertiary medical center. J Am Acad Dermatol. 2007 Dec. 57(6):937-43. [Medline].

  16. Sun Y, Liu Y, Yan B, Shi G. Interstitial lung disease in clinically amyopathic dermatomyositis (CADM) patients: a retrospective study of 41 Chinese Han patients. Rheumatol Int. 2013 May. 33(5):1295-302. [Medline].

  17. 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. 2006 Apr. 54(4):597-613. [Medline].

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

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

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

  21. 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. 2002 Sep. 119(3):617-20. [Medline].

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

  23. 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. 2002 Jun. 103(3 Pt 1):260-3. [Medline].

  24. Chen S, Wang Q, Wu Z, Wu Q, Li P, Li Y, et al. Associations between TNF-a-308A/G Polymorphism and Susceptibility with Dermatomyositis: A Meta-Analysis. PLoS One. 2014. 9(8):e102841. [Medline]. [Full Text].

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

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

  27. 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. 2004 Nov. 50(11):3646-50. [Medline].

  28. Callen JP, Hyla JF, Bole GG Jr, Kay DR. The relationship of dermatomyositis and polymyositis to internal malignancy. Arch Dermatol. 1980 Mar. 116(3):295-8. [Medline].

  29. Buchbinder R, Forbes A, Hall S, Dennett X, Giles G. Incidence of malignant disease in biopsy-proven inflammatory myopathy. A population-based cohort study. Ann Intern Med. 2001 Jun 19. 134(12):1087-95. [Medline].

  30. 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. 1995 Jan. 6(1):9-13. [Medline].

  31. Limaye V, Luke C, Tucker G, Hill C, Lester S, Blumbergs P, et al. The incidence and associations of malignancy in a large cohort of patients with biopsy-determined idiopathic inflammatory myositis. Rheumatol Int. 2013 Apr. 33(4):965-71. [Medline].

  32. So MW, Koo BS, Kim YG, Lee CK, Yoo B. Idiopathic inflammatory myopathy associated with malignancy: a retrospective cohort of 151 Korean patients with dermatomyositis and polymyositis. J Rheumatol. 2011 Nov. 38(11):2432-5. [Medline].

  33. Zantos D, Zhang Y, Felson D. The overall and temporal association of cancer with polymyositis and dermatomyositis. J Rheumatol. 1994 Oct. 21(10):1855-9. [Medline].

  34. Stockton D, Doherty VR, Brewster DH. Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study. Br J Cancer. 2001 Jul 6. 85(1):41-5. [Medline]. [Full Text].

  35. Femia AN, Vleugels RA, Callen JP. Cutaneous dermatomyositis: an updated review of treatment options and internal associations. Am J Clin Dermatol. 2013 Aug. 14(4):291-313. [Medline].

  36. Huang YL, Chen YJ, Lin MW, Wu CY, Liu PC, Chen TJ, et al. Malignancies associated with dermatomyositis and polymyositis in Taiwan: a nationwide population-based study. Br J Dermatol. 2009 Oct. 161(4):854-60. [Medline].

  37. Kuo CF, See LC, Yu KH, Chou IJ, Chang HC, Chiou MJ, et al. Incidence, cancer risk and mortality of dermatomyositis and polymyositis in Taiwan: a nationwide population study. Br J Dermatol. 2011 Dec. 165(6):1273-9. [Medline].

  38. Liu WC, Ho M, Koh WP, Tan AW, Ng PP, Chua SH, et al. An 11-year review of dermatomyositis in Asian patients. Ann Acad Med Singapore. 2010 Nov. 39(11):843-7. [Medline].

  39. Chen D, Yuan S, Wu X, Li H, Qiu Q, Zhan Z, et al. Incidence and predictive factors for malignancies with dermatomyositis: a cohort from southern China. Clin Exp Rheumatol. 2014 Jul 28. [Medline].

  40. Valenzuela A, Chung L, Casciola-Rosen L, Fiorentino D. Identification of clinical features and autoantibodies associated with calcinosis in dermatomyositis. JAMA Dermatol. 2014 Jul. 150(7):724-9. [Medline].

  41. Rai SK, Choi HK, Sayre EC, Aviña-Zubieta JA. Risk of myocardial infarction and ischaemic stroke in adults with polymyositis and dermatomyositis: a general population-based study. Rheumatology (Oxford). 2015 Sep 30. [Medline].

  42. Lee CW, Muo CH, Liang JA, Sung FC, Hsu CY, Kao CH. Increased osteoporosis risk in dermatomyositis or polymyositis independent of the treatments: a population-based cohort study with propensity score. Endocrine. 2015 Oct 1. [Medline].

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

  44. Labirua-Iturburu A, Selva-O'Callaghan A, Vincze M, Dankó K, Vencovsky J, Fisher B, et al. Anti-PL-7 (anti-threonyl-tRNA synthetase) antisynthetase syndrome: clinical manifestations in a series of patients from a European multicenter study (EUMYONET) and review of the literature. Medicine (Baltimore). 2012 Jul. 91(4):206-11. [Medline].

  45. 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). 2010 Jan. 89(1):47-52. [Medline].

  46. Trallero-Araguás E, Rodrigo-Pendás JÁ, Selva-O'Callaghan A, Martínez-Gómez X, Bosch X, Labrador-Horrillo M, et al. Usefulness of anti-p155 autoantibody for diagnosing cancer-associated dermatomyositis: a systematic review and meta-analysis. Arthritis Rheum. 2012 Feb. 64(2):523-32. [Medline].

  47. Hamaguchi Y, Kuwana M, Hoshino K, Hasegawa M, Kaji K, Matsushita T, et al. Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional study. Arch Dermatol. 2011 Apr. 147(4):391-8. [Medline].

  48. Fujimoto M, Hamaguchi Y, Kaji K, Matsushita T, Ichimura Y, Kodera M, et al. Myositis-specific anti-155/140 autoantibodies target transcription intermediary factor 1 family proteins. Arthritis Rheum. 2012 Feb. 64(2):513-22. [Medline].

  49. Sato S, Hirakata M, Kuwana M, Suwa A, Inada S, Mimori T, et al. Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum. 2005 May. 52(5):1571-6. [Medline].

  50. Sato S, Hoshino K, Satoh T, Fujita T, Kawakami Y, Fujita T, et al. RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive interstitial lung disease. Arthritis Rheum. 2009 Jul. 60(7):2193-200. [Medline].

  51. Hoshino K, Muro Y, Sugiura K, Tomita Y, Nakashima R, Mimori T. Anti-MDA5 and anti-TIF1-gamma antibodies have clinical significance for patients with dermatomyositis. Rheumatology (Oxford). 2010 Sep. 49(9):1726-33. [Medline].

  52. Chaisson NF, Paik J, Orbai AM, Casciola-Rosen L, Fiorentino D, Danoff S, et al. A novel dermato-pulmonary syndrome associated with MDA-5 antibodies: report of 2 cases and review of the literature. Medicine (Baltimore). 2012 Jul. 91(4):220-8. [Medline]. [Full Text].

  53. Cuesta-Mateos C, Colom-Fernández B, Portero-Sainz I, Tejedor R, García-García C, Concha-Garzón MJ, et al. Autoantibodies against TIF-1-? and CADM-140 in Spanish patients with clinically amyopathic dermatomyositis (CADM): clinical significance and diagnostic utility. J Eur Acad Dermatol Venereol. 2014 Jul 28. [Medline].

  54. Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol. 2011 Jul. 65(1):25-34. [Medline]. [Full Text].

  55. Muro Y, Sugiura K, Hoshino K, Akiyama M. Disappearance of anti-MDA-5 autoantibodies in clinically amyopathic DM/interstitial lung disease during disease remission. Rheumatology (Oxford). 2012 May. 51(5):800-4. [Medline].

  56. Tansley SL, Betteridge ZE, Gunawardena H, Jacques TS, Owens CM, Pilkington C, et al. Anti-MDA5 autoantibodies in juvenile dermatomyositis identify a distinct clinical phenotype: a prospective cohort study. Arthritis Res Ther. 2014 Jul 2. 16(4):R138. [Medline].

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

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

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

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

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

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

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

  64. 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. 1997 Jan. 36(1):67-71. [Medline].

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

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

  67. Newman ED, Scott DW. The Use of Low-dose Oral Methotrexate in the Treatment of Polymyositis and Dermatomyositis. J Clin Rheumatol. 1995 Apr. 1(2):99-102. [Medline].

  68. Bunch TW. Prednisone and azathioprine for polymyositis: long-term followup. Arthritis Rheum. 1981 Jan. 24(1):45-8. [Medline].

  69. 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. 1993 Dec 30. 329(27):1993-2000. [Medline].

  70. 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. 2009 Dec. 9(2):124-7. [Medline].

  71. 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). 2010 Dec. 62(12):1748-55. [Medline].

  72. Oddis CV, Reed AM, Aggarwal R, Rider LG, Ascherman DP, Levesque MC, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum. 2013 Feb. 65(2):314-24. [Medline]. [Full Text].

  73. Aggarwal R, Bandos A, Reed AM, Ascherman DP, Barohn RJ, Feldman BM, et al. Predictors of clinical improvement in rituximab-treated refractory adult and juvenile dermatomyositis and adult polymyositis. Arthritis Rheumatol. 2014 Mar. 66(3):740-9. [Medline]. [Full Text].

  74. Ge Y, Zhou H, Shi J, Ye B, Peng Q, Lu X, et al. The efficacy of tacrolimus in patients with refractory dermatomyositis/polymyositis: a systematic review. Clin Rheumatol. 2015 Sep 2. [Medline].

  75. 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. 2002 Sep. 138(9):1231-3; discussion 1233. [Medline].

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

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

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

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

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

  81. 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. 2004 Aug. 51(2 Suppl):S124-30. [Medline].

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

  83. Cohen JB. Cutaneous involvement of dermatomyositis can respond to Dapsone therapy. Int J Dermatol. 2002 Mar. 41(3):182-4. [Medline].

  84. Shimojima Y, Ishii W, Kato T, Hoshi K, Matsuda M, Hashimoto T, et al. Intractable skin necrosis and interstitial pneumonia in amyopathic dermatomyositis, successfully treated with cyclosporin A. Intern Med. 2003 Dec. 42(12):1253-8. [Medline].

  85. Kampylafka EI, Kosmidis ML, Panagiotakos DB, Dalakas M, Moutsopoulos HM, Tzioufas AG. The effect of intravenous immunoglobulin (IVIG) treatment on patients with dermatomyositis: a 4-year follow-up study. Clin Exp Rheumatol. 2012 May-Jun. 30(3):397-401. [Medline].

  86. Danieli MG, Moretti R, Gambini S, Paolini L, Gabrielli A. Open-label study on treatment with 20 % subcutaneous IgG administration in polymyositis and dermatomyositis. Clin Rheumatol. 2014 Apr. 33(4):531-6. [Medline].

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

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

  89. 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. 2010 Jan. 77(1):70-2. [Medline].

  90. Balin SJ, Wetter DA, Andersen LK, Davis MD. Calcinosis cutis occurring in association with autoimmune connective tissue disease: the Mayo Clinic experience with 78 patients, 1996-2009. Arch Dermatol. 2012 Apr. 148(4):455-62. [Medline].

  91. Alemo Munters L, Dastmalchi M, Andgren V, Emilson C, Bergegård J, Regardt M, et al. Improvement in health and possible reduction in disease activity using endurance exercise in patients with established polymyositis and dermatomyositis: a multicenter randomized controlled trial with a 1-year open extension followup. Arthritis Care Res (Hoboken). 2013 Dec. 65(12):1959-68. [Medline].

  92. Alexanderson H, Munters LA, Dastmalchi M, Loell I, Heimbürger M, Opava CH, et al. Resistive home exercise in patients with recent-onset polymyositis and dermatomyositis -- a randomized controlled single-blinded study with a 2-year followup. J Rheumatol. 2014 Jun. 41(6):1124-32. [Medline].

  93. Anyanwu CO, Fiorentino DF, Chung L, Dzuong C, Wang Y, Okawa J, et al. Validation of the Cutaneous Dermatomyositis Disease Area and Severity Index: characterizing disease severity and assessing responsiveness to clinical change. Br J Dermatol. 2015 Oct. 173 (4):969-74. [Medline].

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

  95. Di Rollo D, Abeni D, Tracanna M, Capo A, Amerio P. Cancer risk in dermatomyositis: a systematic review of the literature. G Ital Dermatol Venereol. 2014 Jun 30. [Medline].

  96. Metzger AL, Bohan A, Goldberg LS, Bluestone R, Pearson CM. Polymyositis and dermatomyositis: combined methotrexate and corticosteroid therapy. Ann Intern Med. 1974 Aug. 81(2):182-9. [Medline].

  97. Marie I, Hachulla E, Hatron PY, Hellot MF, Levesque H, Devulder B, et al. Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis. J Rheumatol. 2001 Oct. 28(10):2230-7. [Medline].

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

  99. Efthimiou P, Schwartzman S, Kagen LJ. Possible role for tumour necrosis factor inhibitors in the treatment of resistant dermatomyositis and polymyositis: a retrospective study of eight patients. Ann Rheum Dis. 2006 Sep. 65(9):1233-6. [Medline]. [Full Text].

  100. Iannone F, Scioscia C, Falappone PC, Covelli M, Lapadula G. Use of etanercept in the treatment of dermatomyositis: a case series. J Rheumatol. 2006 Sep. 33(9):1802-4. [Medline].

  101. Dastmalchi M, Grundtman C, Alexanderson H, Mavragani CP, Einarsdottir H, Helmers SB, et al. A high incidence of disease flares in an open pilot study of infliximab in patients with refractory inflammatory myopathies. Ann Rheum Dis. 2008 Dec. 67(12):1670-7. [Medline].

  102. Hengstman GJ, De Bleecker JL, Feist E, Vissing J, Denton CP, Manoussakis MN, et al. Open-label trial of anti-TNF-alpha in dermato- and polymyositis treated concomitantly with methotrexate. Eur Neurol. 2008. 59(3-4):159-63. [Medline].

  103. Liu SW, Velez NF, Lam C, Femia A, Granter SR, Townsend HB, et al. Dermatomyositis induced by anti-tumor necrosis factor in a patient with juvenile idiopathic arthritis. JAMA Dermatol. 2013 Oct. 149(10):1204-8. [Medline].

  104. Fathi M, Vikgren J, Boijsen M, Tylen U, Jorfeldt L, Tornling G, et al. Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology. Arthritis Rheum. 2008 May 15. 59(5):677-85. [Medline].

  105. Linos E, Fiorentino D, Lingala B, Krishnan E, Chung L. Atherosclerotic cardiovascular disease and dermatomyositis: an analysis of the Nationwide Inpatient Sample survey. Arthritis Res Ther. 2013 Jan 8. 15(1):R7. [Medline].

  106. Carruthers EC, Choi HK, Sayre EC, Aviña-Zubieta JA. Risk of deep venous thrombosis and pulmonary embolism in individuals with polymyositis and dermatomyositis: a general population-based study. Ann Rheum Dis. 2014 Sep 5. [Medline].

  107. Shimojima Y, Ishii W, Matsuda M, Tazawa K, Ikeda S. Coadministration of tacrolimus with corticosteroid accelerates recovery in refractory patients with polymyositis/ dermatomyositis: a retrospective study. BMC Musculoskelet Disord. 2012 Nov 22. 13:228. [Medline].

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.
A 47-year-old woman presented with a pruritic, diffuse rash across her upper hands and face that is worsened with sun exposure. ANA testing by outside providers was negative. Her rash was not responsive to topical steroids, and improved with oral prednisone but recurred with tapers beyond 15 mg daily. Diagnosis was dermatomyositis sine myositis. Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD, Division of Rheumatology, University of Colorado Denver School of Medicine.
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