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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.

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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. 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.

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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]

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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 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]

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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]

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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.

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]

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Consultations

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.

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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.

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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.

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

Acknowledgements

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.

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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.
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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