AUTHOR AND EDITOR INFORMATION

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• Follow-up
• Miscellaneous
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INTRODUCTION

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Background

Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) with characteristic cutaneous findings. DM is a systemic disorder that frequently affects the joints, the esophagus, the lungs, and, less commonly, the heart.

In 1975, Bohan and Peter¹ first suggested a set of criteria to aid in diagnosing and classifying DM and polymyositis (PM). Of the 5 criteria, 4 relate to the muscle disease; these include progressive proximal symmetrical weakness, elevated muscle enzyme levels, abnormal findings on electromyograms, and abnormal findings from muscle biopsy. The fifth criterion is compatible cutaneous disease.

Bohan and Peter suggested 5 subsets of myositis: DM, PM, myositis with cancer, childhood DM/PM, and myositis overlapping with another collagen vascular disorder. In a subsequent publication, Bohan and Peter² noted that cutaneous disease might precede the development of the myopathy; however, only recently was another possible subset of patients with disease that affects only the skin recognized; this condition is known as amyopathic dermatomyositis (ADM), or  DM sine myositis.³ The association between DM (and possibly PM) and cancer has long been recognized.^{4, 5, 6, 7}

DM sine myositis, also known as ADM, is diagnosed in patients with typical cutaneous disease in whom no evidence of muscle weakness exists and in whom serum muscle enzyme levels are repeatedly normal for a 2-year period in the absence of disease-modifying therapies such as corticosteroids, immunosuppressive agents, or both. When studied, some patients with ADM have abnormal ultrasound, MRI or magnetic resonance spectroscopy, or muscle biopsy findings. These patients have muscle involvement, and their condition may be better classified as hypomyopathic DM. Patients with these variations may also reflect an underlying malignancy, and some develop severe pulmonary disease, particularly persons from Asian countries.

Patients exist in whom myositis resolves following therapy but whose skin disease remains as an active, important feature of the disease. These patients are not classified as having ADM, despite the fact that, at this point in time, the skin is the major and often only manifestation of the disease. Sontheimer⁸ has suggested the term postmyopathic DM for these patients.

Rare cutaneous manifestations include vesiculobullous, erosive lesions, and an exfoliative erythroderma. Biopsy samples from patients reveal an interface dermatitis similar to that of biopsy samples of heliotrope rash, Gottron papules, poikiloderma, or scalp lesions. These cutaneous manifestations may be more common in patients with an associated malignancy than in those without a malignancy.

Pathophysiology

Recent studies of the pathogenesis of the myopathy have been controversial. Some suggest that the myopathy in DM and PM is pathogenetically different from DM due to a vascular inflammation, while other studies of cytokines suggest that the processes are similar. The pathogenesis of the cutaneous disease is poorly understood.

Frequency

United States

The estimated incidence of DM/PM is 5.5 cases per million population. However, the incidence appears to be increasing.

Mortality/Morbidity

• DM may cause death because of muscle weakness or cardiopulmonary involvement. Patients with an associated cancer may die from the malignancy.
• Most patients with DM survive, in which case they may develop residual weakness and disability. In children with severe disease, contractures can develop if they do not receive physical therapy.
• Calcinosis may be a complication, particularly in children.

Race

Whites are more frequently affected. However, the rise in the incidence in African Americans is greater than that in whites.

Sex

Women are affected twice as often as men.

Age

DM can occur in persons of any age, but the most common age at onset is in the fifth and sixth decades of life.

CLINICAL

Section 3 of 11  

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History

• Patients often present with skin disease as one of the initial manifestations. In as many as 40% of patients, the skin disease may be the sole manifestation at the onset. Muscle disease may occur concurrently, it may precede the skin disease, or it may follow the skin disease by weeks to years.
• Patients often notice an eruption on exposed surfaces. The disease is often pruritic, and, sometimes, intense pruritus may disturb sleep patterns. Patients may also complain of a scaly scalp or diffuse hair loss (see Media File 5).
• Muscle involvement manifests as proximal muscle weakness. Patients often begin to note fatigue of their muscles or weakness when climbing stairs, walking, rising from a sitting position, combing their hair, or reaching for items in cabinets that are above their shoulders. Muscle tenderness may occur, but it is not a regular feature of the disease.
• Systemic manifestations may occur; therefore, a review of systems should assess for the presence of arthralgias, arthritis, dyspnea, dysphagia, arrhythmias, and dysphonia.
• Malignancy is possible in any patient with DM, but it is much more common in adults older than 60 years. Only a handful of children with DM and malignancy have been reported. The history should include a thorough review of systems, as well as an assessment for previous malignancy.
• Children with DM may have an insidious onset that hides the true diagnosis until the dermatologic disease is clearly observed and diagnosed. Calcinosis is a complication of juvenile DM, but it is rarely observed at the onset of disease. African Americans and patients in lower socioeconomic groups are more likely to have a delay in their diagnosis. The prognosis in children with DM is worse in those in whom diagnosis is delayed.

Physical

DM is a disease that primarily affects the skin and the muscles, but it might also affect other organ systems.

• The characteristic and possibly pathognomonic cutaneous features of DM are the heliotrope rash and Gottron papules (see Media File 2). Several other cutaneous features are characteristic of the disease despite not being pathognomonic. They include malar erythema, poikiloderma in a photosensitive distribution, violaceous erythema on the extensor surfaces, and periungual and cuticular changes.
• • The heliotrope rash consists of a violaceous to dusky erythematous rash with or without edema in a symmetrical distribution involving the periorbital skin. Sometimes, this sign is subtle and may consist of only a mild discoloration along the eyelid margin. Similar to other areas, scale may be present on the eyelids. A heliotrope rash is rarely observed in other disorders; therefore, its presence is highly suggestive of DM.
  • Gottron papules are found over bony prominences, particularly the metacarpophalangeal joints, the proximal interphalangeal joints, and/or the distal interphalangeal joints. They may also be found overlying the elbows, the knees, and/or the feet. The lesions consist of slightly elevated, violaceous papules and plaques. A slight scale may be present, and, occasionally, a thick psoriasiform scale is observed. These lesions may resemble lesions of lupus erythematosus, psoriasis, or lichen planus (LP).
• Nail fold changes consist of periungual telangiectases and/or a characteristic cuticular change with hypertrophy of the cuticle and small, hemorrhagic infarcts in this hypertrophic area (see Media File 3). Periungual telangiectases may be clinically apparent, or they may be appreciated only by capillary microscopy.
• Poikiloderma may occur on exposed skin, such as the extensor surfaces of the arm, the V of the neck (see Media File 6), or the upper part of the back (Shawl sign).
• With the exception of the heliotrope rash, the eruption of DM is photodistributed and photoexacerbated. Patients rarely complain of photosensitivity, despite the prominent photodistribution of the rash.
• Facial erythema may also occur in DM. This change must be differentiated from lupus erythematosus, rosacea, seborrheic dermatitis, or atopic dermatitis.
• Scalp involvement in DM is relatively common and manifests as an erythematous to violaceous, psoriasiform dermatitis. Clinical distinction from seborrheic dermatitis or psoriasis is occasionally difficult. In some patients, nonscarring alopecia may occur and often follows a flare of systemic disease.
• Other cutaneous lesions have been described in patients with DM or PM that do not reflect the interface changes observed at histopathologic examination with pathognomonic or characteristic lesions. These include panniculitis (see Media File 9) and urticaria, as well as changes of hyperkeratosis of the palms known as mechanic's hands. Other findings include cutaneous mucinosis, follicular hyperkeratosis, hyperpigmentation, ichthyosis, white plaques on the buccal mucosa, cutaneous vasculitis, and a flagellate erythema.
• Calcinosis of the skin or the muscle is unusual in adults, but it may occur in as many as 40% of children or adolescents with DM. Calcinosis cutis manifests as firm, yellow or flesh-colored nodules, often over bony prominences. Occasionally, these nodules can extrude through the surface of the skin, in which case, secondary infection may occur.⁹
• Muscle findings include weakness and, sometimes, tenderness.
• • Muscle disease manifests as a proximal symmetrical muscle weakness. Patients may have difficulty rising from a chair or squatting and then raising themselves from this position. Sometimes, in an effort to rise, patients use other muscles that are not as affected. The careful examiner may note this finding.
  • Testing of the muscle strength is part of each assessment of the patient. Often, the extensor muscles of the arms are more affected than the flexor muscles.
  • Distal strength is almost always maintained. Muscle tenderness is a variable finding.
• Other systemic features include joint swelling, changes associated with Raynaud phenomenon, and abnormal findings on cardiopulmonary examination.
• • Joint swelling occurs in some patients with DM. The small joints of the hands are the most frequently involved. The arthritis associated with DM is nondeforming.
  • Patients with pulmonary disease may have abnormal breath sounds.
  • Patients with an associated malignancy may have physical findings relevant to the affected organs.

Causes

• The cause of DM is unknown. Factors that have been implicated are listed below.
• • A genetic predisposition may exist. Rarely, DM manifests in multiple family members. However, a link to certain HLA types may exist. Polymorphisms of tumor necrosis factor may be involved; specifically, the presence of the -308A allele is linked to photosensitivity in adults and calcinosis in children.
  • Immunologic abnormalities are common in patients with DM. Patients frequently have circulating autoantibodies. Abnormal T-cell activity may be involved in the pathogenesis of both the skin disease and the muscle disease. In addition, family members may manifest other diseases associated with autoimmunity.
  • Infectious agents, including viruses (particularly coxsackievirus, echovirus, human T-lymphotropic virus 1 [HTLV-1], and human immunodeficiency virus [HIV]), Toxoplasma species, and Borrelia species, have been suggested as possible triggers of the disease.
  • Several cases of drug-induced disease have been reported. DM-like skin changes have been reported with hydroxyurea in patients with chronic myelogenous leukemia or essential thrombocytosis.¹⁰ Other agents that may trigger the disease include penicillamine, statin drugs, quinidine, and phenylbutazone.
  • DM may be initiated or exacerbated by silicon breast implants or collagen injections. However, this evidence is anecdotal and has not been verified in case-control studies.

DIFFERENTIALS

Section 4 of 11  

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WORKUP

Section 5 of 11  

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Lab Studies

• Muscle enzyme levels are often abnormal at some time in patients with DM, except in those with the amyopathic variant. The most common enzyme level to obtain is the creatine kinase level. However, an aldolase level test and other tests, such as aspartate aminotransferase and lactate dehydrogenase tests, may also yield abnormal results. At times, the elevation of the enzyme levels precedes clinical evidence of myositis. Therefore, if a patient who is presumably stable develops an elevation in an enzyme level that was previously normal, the clinician should assess the possibility of a flare of the muscle disease.
• Several serologic abnormalities have been identified, but their routine use has not yet been delineated. As a group, these antibodies have been termed myositis-specific antibodies (MSAs).
• • A positive antinuclear antibody result is common in patients with DM.
  • Anti-Mi-2 is highly specific for DM, but it lacks sensitivity because only 25% of patients with DM demonstrate this abnormality.
  • Anti-Jo-1 and other antisynthetase antibodies are associated with pulmonary involvement, but it is more common in patients with PM than DM.
  • Other MSAs include antisignal recognition protein and anti-Ku.

Imaging Studies

• MRI with T2 weighting or magnetic resonance spectroscopy may be useful for assessing the presence of an inflammatory myopathy in patients without weakness. It is also useful in differentiating a steroid myopathy from a continued inflammation. Lastly, it may serve as a guide for selection of a site for muscle biopsy.
• Chest radiography should be obtained at the time of diagnosis and when symptoms develop.
• The barium swallow test allows evaluation of esophageal dysmotility.
• Ultrasonography of the muscles has been suggested for evaluation, but its use has not been widely accepted.

Other Tests

• Pulmonary function studies and electrocardiography may be performed.
• Esophageal manometry or other esophageal studies may be performed in selected patients.
• Electromyography is a means of detecting inflammation of the muscles. At times, it has been useful for the selection of a site for muscle biopsy. This test is less commonly obtained now by dermatologists caring for patients with typical skin lesions.

Procedures

• An age-appropriate evaluation for a possible malignancy should be performed at the time of diagnosis and then annually for the first 3 years. Female patients should be carefully screened for ovarian cancer.¹¹ After that time, patients should be evaluated for malignancy at intervals similar to any other person of the same age and sex.
• Muscle biopsy, either open biopsy or needle biopsy, may enhance the ability of the clinician to diagnose DM. It is also sometimes useful in differentiating steroid myopathy from active inflammatory myopathy when patients have been on corticosteroid therapy but are still weak.

Histologic Findings

Skin biopsy samples reveal an interface dermatitis that is difficult to differentiate from lupus erythematosus. Often, the histologic features of DM and lupus erythematosus are identical. Both may contain excessive mucin, and both demonstrate an interface vacuolar dermatitis. Well-formed lesions of discoid lupus erythematosus differ from those of DM, but often DM and subacute cutaneous lupus erythematosus cannot be differentiated.

TREATMENT

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Medical Care

The therapy for DM involves general measures, measures to control the muscle disease, and measures to control the skin disease. In addition, in some patients, treating other systemic manifestations or complications may be necessary.

Therapy of the muscle component involves the use of corticosteroids with or without an immunosuppressive agent. The skin disease is treated by avoiding sun exposure and by using sunscreens, topical corticosteroids, antimalarial agents, and/or methotrexate or mycophenolate mofetil.

• Several general measures are helpful in the care of patients with DM. Bedrest is often valuable for those with severe inflammation of the muscles. In patients with muscle weakness, a program of physical therapy is useful in preventing contractures that can be a complication 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.
• The mainstay of therapy for the muscle disease is systemically administered corticosteroids. Traditionally, prednisone (0.5-1 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. Use of drugs, such as methotrexate, azathioprine, cyclophosphamide, cyclosporin, mycophenolate mofetil, and chlorambucil, has been reported to be steroid sparing in some patients or in small open-label studies.^{12, 13} For conditions that do not improve, the use of monthly high-dose intravenous immune globulin has proved to be beneficial.¹⁴
• Therapy for the cutaneous disease is often difficult. Patients who present primarily with skin disease (ADM) and those in whom the muscle component is controlled but who still have significant skin disease exist. The first-line of therapy is recognizing that the patient is photosensitive and advising the patient to avoid sun exposure and to use sun protective measures, including broad-spectrum sunscreens. Hydroxychloroquine and chloroquine have been beneficial in small open-label case studies.¹⁵ Methotrexate is also useful.¹⁶ Recently, mycophenolate mofetil has been reported to be useful.¹⁷ Intravenous immune globulin not only benefited the muscle but also cleared the skin lesions in the patients in whom it was used.
• Aggressive early treatment of the myositis may aid in the prevention of calcinosis. Once established, the process is debilitating in many patients. Although spontaneous remission is possible, it often occurs after many years. 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.¹⁸ In addition, the use of an oral bisphosphonate might be helpful.¹⁹

Surgical Care

• Surgical care is usually not necessary.
• Some patients with local areas of calcinosis may wish to have them surgically removed.

Consultations

• Rheumatologist
• Internal medicine or pediatric specialist, depending on the patient's age
• Neurologist
• Oncologist, medical or surgical, for patients with cancer

Diet

• A well-balanced diet is helpful. Patients with severe inflammation of the muscles may need extra protein to balance their loss.
• Feeding should be avoided prior to bedtime in patients with dysphagia.

Activity

• Activity should be maintained as best as possible. Vigorous physical training should be avoided. Exercises should be used to maintain the patient's range of motion.
• Patients with skin lesions should avoid sun exposure and use sun protective measures.

MEDICATION

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The mainstay of therapy for patients with DM is systemic corticosteroids.

Drug Category: Corticosteroids

These agents may be used topically for cutaneous disease. Systemic corticosteroids are a mainstay of primary therapy for patients with muscle involvement. Patients with cutaneous disease have a variable response to systemic corticosteroids. Patients with pulmonary involvement may respond, but those with esophageal dysfunction do not respond. Patients with cardiac involvement may respond.

Drug Category: Immunosuppressives

These agents should be used early in the course as steroid-sparing agents. They lower the risk of steroid-related complications.

Drug Category: Antimalarials

These agents may be used as a steroid-sparing agent for the treatment of the skin disease. Hydroxychloroquine is preferred; chloroquine and quinacrine (100 mg/d) are second-line agents. However, antimalarial-induced drug eruptions are reportedly more common in patients with DM than in patients with LE. Quinacrine may suppress bone marrow and is distributed by the Centers for Disease Control and Prevention; blood cell counts should be regularly obtained.

Drug Category: Immune globulins

High-dose IV immunoglobulin has been reported to be useful for patients with recalcitrant DM.

FOLLOW-UP

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Further Inpatient Care

• Inpatient care is needed for patients with fulminant disease.

Further Outpatient Care

• Monitoring of the activity of the disease is necessary on at least a monthly basis. Repeat measurements of muscle enzyme levels may aid in the assessment of the activity of the myositis, along with clinical assessment of strength. Machines are available that can aid in the quantification of the strength, but they are not widely used.
• An assessment of the skin disease is by a physical examination in conjunction with a history.
• Annual physical examinations are useful to monitor for potential toxicity from therapy or for a malignancy.
• • Malignancy evaluations should be conducted for at least the first 3 years following diagnosis and at other times if symptoms develop or the patient's disease is poorly responsive to therapy. The testing selected should be chosen based on the patient's age, sex, race, and other symptoms or findings. After 3 years, patients should be monitored as any other person of the same age, race, and sex.
  • Women should be screened for ovarian cancer.¹¹

Complications

• Calcinosis may be a complication in children and adolescents with DM (see Media File 8). Its presence has been linked to a delay in diagnosis and to therapy that is less aggressive.
• Contractures can occur if the patient is immobile.

Prognosis

• The prognosis depends on the severity of the myopathy, the presence of a malignancy, and/or the presence of cardiopulmonary involvement.
• • Patients with DM who have malignancy, cardiac involvement, or pulmonary involvement and those with DM who are elderly (>60 y) have a poorer prognosis.
  • The disease may spontaneously remit in as many as 20% of patients.
  • About 5% of patients have a fulminant progressive course, with eventual death. Therefore, many patients require long-term therapy.

Patient Education

• Physical therapy and rehabilitative measures are necessary in selected patients.
• Sun protective measures are necessary for patients with skin disease.
• The Myositis Association of America is a resource on inflammatory myopathies for patients and the medical community.

MISCELLANEOUS

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Medical/Legal Pitfalls

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

MULTIMEDIA

Section 10 of 11  

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Media file 1:  The heliotrope rash is a characteristic and possibly pathognomonic cutaneous feature of dermatomyositis. The heliotrope flower from which the manifestation is named is pictured.
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Media file 2:  Heliotrope rash in a woman with dermatomyositis.
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Media file 3:  Gottron papules and nail fold telangiectasia are present in this patient.
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Media file 4:  The lesions on the dorsal aspect of the hand demonstrate the photodistribution of dermatomyositis. Note the sparing of the interdigital web spaces.
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Media file 5:  A diffuse alopecia with a scaly scalp dermatosis is common in dermatomyositis.
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Media file 6:  Dermatomyositis is often associated with a poikiloderma in a photodistribution.
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Media file 7:  The histopathologic feature of dermatomyositis is an interface dermatitis.
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Media file 8:  Calcinosis due to dermatomyositis in childhood can be seen in this patient who had active dermatomyositis 15 years prior to the time of this photograph.
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Media file 9:  Calcifying panniculitis in a patient with dermatomyositis.
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REFERENCES

Section 11 of 11

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31. Targoff IN. Dermatomyositis and polymyositis. Curr Prob Dermatol. 1991;3:131-80.
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Article Last Updated: Feb 6, 2007