AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) with characteristic cutaneous findings. In 1975, Bohan and Peter first suggested a set of criteria to aid in the diagnosis and classification of dermatomyositis and polymyositis (PM).^{1, 2} Four of the 5 criteria are related to the muscle disease, as follows: progressive proximal symmetrical weakness, elevated muscle enzymes, an abnormal finding on electromyogram, and an abnormal finding on the muscle biopsy sample. The fifth criterion was compatible cutaneous disease.

The association between dermatomyositis (and possibly polymyositis) and malignancy has been recognized for a long time. Dermatomyositis is a systemic disorder that frequently affects the esophagus and lungs and, less commonly, may affect the heart. Calcinosis is a complication that is observed most often in children or adolescents. Therapy of the muscle component involves the use of corticosteroids with or without an immunosuppressive agent. The skin disease is treated with sun avoidance, sunscreens, topical corticosteroids, antimalarial agents, methotrexate, mycophenolate mofetil and/or intravenous immune globulin. Some of the newer biologic agents may prove useful for either component of the disease or for both components. The prognosis depends on the severity of the myopathy, the presence of a malignancy, and/or the presence of cardiopulmonary involvement.

Pathophysiology

The pathogenesis of the cutaneous disease is poorly understood. Bohan and Peter (1975) suggested 5 subsets of myositis, as follows: dermatomyositis, polymyositis, myositis with malignancy, childhood dermatomyositis/polymyositis, and myositis overlapping with another collagen-vascular disorder. In a subsequent publication, Bohan et al (1975) noted that cutaneous disease may precede the development of the myopathy. The existence of another subset of patients with disease that only affects the skin (called amyopathic dermatomyositis [ADM] or dermatomyositis-sine myositis) was only recently recognized. Lastly, a group of patients exists whose myopathy is controlled but who continue to have severe and sometimes debilitating skin disease; these patients' conditions have been termed postmyopathic dermatomyositis.

Recent studies of the pathogenesis of the myopathy have been controversial. Some suggest that the myopathy in dermatomyositis and polymyositis is pathogenetically different. Dermatomyositis is probably caused by complement-mediated (terminal attack complex) vascular inflammation, while polymyositis is caused by the direct cytotoxic effect of CD8⁺ lymphocytes on muscle. However, other studies of cytokines suggest that some of the inflammatory processes may be similar. A recent report has linked tumor necrosis factor (TNF) abnormalities with dermatomyositis.

Frequency

United States

The incidence of dermatomyositis/polymyositis has been estimated at 5.5 cases per million people, and the incidence is apparently increasing.

Mortality/Morbidity

• Dermatomyositis may cause death because of muscle weakness or cardiopulmonary involvement. Patients with an associated malignancy may die from the malignancy.
• Most patients with dermatomyositis survive, in which case they may develop residual weakness and disability. In children with severe disease, contractures can develop.
• Calcinosis may complicate dermatomyositis. It is very rare in adults, but is more common in children and has been linked to delay in diagnosis and to less aggressive therapy.

Race

No racial predilection exists for dermatomyositis or polymyositis.

Sex

Women are affected twice as often as men.

Age

Dermatomyositis can occur in people of any age. Two peak ages of onset exist. In adults, the peak age of onset is approximately 50 years, and, in children, the peak age is approximately 5-10 years.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

• Patients often present with skin disease as one of the initial manifestations. Sometimes, perhaps in as many as 40% of the patients, the skin disease may be the sole manifestation at the onset. Muscle disease may occur concurrently, may precede the skin disease, or may follow the skin disease by weeks to years.
• Patients often notice an eruption on exposed surfaces. The rash is often pruritic, and intense pruritus may disturb sleep patterns. Patients may also report a scaly scalp or diffuse hair loss.
• Muscle involvement is manifested by 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 tenderness is not a regular feature of the disease.
• Systemic manifestations may occur; therefore, the review of systems should assess for the presence of arthralgia, arthritis, dyspnea, dysphagia, arrhythmia, and dysphonia.
• Malignancy is possible in any patient with dermatomyositis, but it occurs more frequently in adults older than 60 years. Only a few children with dermatomyositis and malignancy have been reported. The history should include a thorough review of systems and an assessment for previous malignancy.
• Children with dermatomyositis may have an insidious onset that defies diagnosis until the dermatologic disease is clearly observed and diagnosed. Calcinosis is a complication of juvenile dermatomyositis but is rarely observed at the onset of disease. Ask questions about hard nodules of the skin during the initial examination.

Physical

• Dermatomyositis is a disease that primarily affects the skin and the muscles but may affect other organ systems.
• The characteristic, and possibly pathognomonic, cutaneous features of dermatomyositis are heliotrope rash and Gottron papules. Several other cutaneous features, including malar erythema, poikiloderma (ie, variegated telangiectasia, hyperpigmentation) in a photosensitive distribution, violaceous erythema on the extensor surfaces, and periungual and cuticular changes, are characteristic of the disease even though they are not pathognomonic.
• Muscle findings typically include proximal weakness and, sometimes, tenderness. Other systemic features include joint swelling, changes associated with Raynaud phenomenon, and abnormalities on cardiopulmonary examination.
• The heliotrope rash consists of a violaceous-to-dusky erythematous rash with or without edema in a symmetrical distribution involving periorbital skin. Sometimes, this sign is subtle and may involve only a mild discoloration along the eyelid margin. A heliotrope rash is rarely observed in other disorders; thus, its presence is highly suggestive of dermatomyositis.
• The Gottron papules are found over bony prominences, particularly the metacarpophalangeal joints, the proximal interphalangeal joints, and/or the distal interphalangeal joints. Papules may also be found overlying the elbows, knees, and/or feet. The lesions consist of slightly elevated violaceous papules and plaques. A slight scale and, occasionally, a thick psoriasiform scale may be present. These lesions may resemble lesions of lupus erythematosus (LE), psoriasis, or lichen planus (LP).
• Nailfold changes consist of periungual telangiectases and/or a characteristic cuticular change with hypertrophy of the cuticle and small hemorrhagic infarcts with this hypertrophic area. Periungual telangiectases may be apparent clinically or may be visible only on capillary microscopy.
• Poikiloderma may occur on exposed skin, such as the extensor surfaces of the arm, and may appear in a V-shaped distribution over the anterior neck and upper chest and back (ie, shawl sign).
• With the exception of the heliotrope rash, the eruption of dermatomyositis is photodistributed and photoexacerbated. Despite the prominent photodistribution of the rash, patients rarely report photosensitivity.
• Facial erythema may also occur in dermatomyositis. This change must be differentiated from LE, rosacea, seborrheic dermatitis, or atopic dermatitis.
• Scalp involvement in dermatomyositis is relatively common and is manifested by an erythematous-to-violaceous psoriasiform dermatitis. Clinical distinction from seborrheic dermatitis or psoriasis is occasionally difficult. Nonscarring alopecia may occur in some patients and often follows a flare of systemic disease.
• Dermatomyositis-sine myositis, also known as ADM, is diagnosed in patients with typical cutaneous disease, no clinical evidence of muscle weakness, and normal serum muscle enzyme levels for at least 2 years. Patients who have undergone disease-modifying therapies such as corticosteroids or immunosuppressive agents are not classified as having ADM. Some patients with ADM have abnormal findings on sonogram, MRI, or muscle biopsy sample. These patients have subclinical muscle involvement, but their condition still may be classified as ADM. Because many ADM patients are not evaluated beyond clinical and enzymatic studies, many think that ADM represents a systemic process requiring systemic therapies.
• Some patients have myositis that resolves following therapy, but the skin disease remains an active important feature of the disease. Although the skin disease is the major, and often only, manifestation of the disease, these patients are not diagnosed with ADM. In addition, a small subset of patients never develop myositis despite having prominent cutaneous changes. Rare cutaneous manifestations include vesiculobullous erosive lesions and exfoliative erythroderma. Biopsy tissue samples from these patients reveal an interface dermatitis (ie, inflammation at the dermal-epidermal junction) similar to biopsy tissue samples from heliotrope rash, Gottron papules, poikiloderma, or scalp lesions. These cutaneous manifestations may be more common in patients with an associated malignancy.
• Various other cutaneous lesions have been described in patients with dermatomyositis or polymyositis that do not reflect the interface changes observed histopathologically with the pathognomonic or characteristic lesions. Panniculitis, urticaria, and hyperkeratosis of the palms (known as mechanic's hands) are examples of these cutaneous lesions. Other findings include cutaneous mucinosis, follicular hyperkeratosis, hyperpigmentation, ichthyosis, white plaques on the buccal mucosa, cutaneous vasculitis, and flagellate erythema.
• Muscle disease manifests as a proximal symmetrical muscle weakness. Patients may have difficulty rising from a chair or squatting and raising themselves from this position. In an effort to rise, patients sometimes 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 flexors. Distal strength is almost always maintained. Muscle tenderness is a variable finding.
• Calcinosis of the skin or muscle is unusual in adults but may occur in as many as 40% of children or adolescents with dermatomyositis. Calcinosis cutis is manifested by firm, yellow- or flesh-colored nodules, often over bony prominences. Occasionally, the nodules can extrude through the surface of the skin, in which case secondary infection may occur.
• Joint swelling occurs in some patients with dermatomyositis. The small joints of the hands are most frequently involved. The arthritis associated with dermatomyositis is not erosive or deforming.
• Patients with pulmonary disease may have abnormal breath sounds (crackles from interstitial fibrosis or pneumonitis).
• Patients with an associated malignancy may have physical findings relevant to the affected organs.

Causes

The cause of dermatomyositis is unknown; however, the following factors have been implicated:

• A genetic predisposition may exist. Dermatomyositis rarely occurs in multiple family members, but it may be linked to certain human leukocyte antigen (HLA) types (eg, DR3, DR5, DR7). In addition, polymorphisms of TNF-alpha have been linked to photosensitivity in patients with dermatomyositis.
• Immunological abnormalities are common in patients with dermatomyositis. Patients frequently have circulating autoantibodies. Abnormal T-cell activity may be involved in the pathogenesis of both the skin and the muscle disease. In addition, family members may have other diseases associated with autoimmunity.
• Autoantibodies to nuclear antigens (ANA) and cytoplasmic (ie, antitransfer RNA synthetases) antigens may be present. Although their presence may help to define subtypes of dermatomyositis and polymyositis, their role in pathogenesis is uncertain.
• Infectious agents, including viruses (eg, coxsackievirus, parvovirus, echovirus, human T-cell lymphotrophic virus type 1 [HTLV-1], HIV) and Toxoplasma and Borrelia species, have been suggested as possible triggers of the disease.
• Several cases of drug-induced disease have been reported. Dermatomyositislike skin changes have been reported with hydroxyurea in patients with chronic myelogenous leukemia or essential thrombocytosis. Other agents that may trigger the disease include penicillamine, statin drugs, quinidine, and phenylbutazone.
• Dermatomyositis may be initiated or exacerbated by silicon breast implants or collagen injections, but the evidence for this is anecdotal and has not been verified in case-control studies. A recent report detailed HLA differences among women in whom inflammatory myopathy develops after they received silicone implants.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia (CREST) syndrome
Inclusion body myositis
Lichen myxedematosus
Lichen planus
Lupus erythematosus (systemic)
Lupus erythematosus (discoid)
Lupus erythematosus (subacute cutaneous)
Morphea
Parapsoriasis
Pityriasis rubra pilaris
Polymorphous light eruption
Psoriasis (plaque)
Rosacea
Tinea corporis
Urticaria (chronic)

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Muscle enzyme levels are often abnormal during the course of dermatomyositis, except in patients with the amyopathic variant. The most sensitive/specific enzyme is an elevated creatine kinase (CK), but aldolase levels and other tests (eg, aspartate aminotransferase [AST], lactic dehydrogenase [LDH]) may also yield abnormal results. At times, the elevation of the enzymes precedes clinical evidence of myositis. Thus, if a patient who is presumably stable develops an elevation of an enzyme previously within the reference range, the clinician should assess the possibility of a flare of the muscle disease.
• Several serologic abnormalities have been identified and may be helpful in the classification of subtypes for prognosis, but they are not used for routine diagnosis. As a group, these antibodies have been termed myositis-specific antibodies (MSAs). These autoantibodies occur in about 30% of all patients with dermatomyositis or polymyositis.
• • A positive ANA finding is common in patients with dermatomyositis.
  • Anti–Mi-2 antibodies are highly specific for dermatomyositis but lack sensitivity because only 25% of the patients with dermatomyositis demonstrate them. They are associated with acute-onset classic dermatomyositis with the V-shaped and shawl rash (poikiloderma) and a relatively good prognosis.
  • Anti–Jo-1 (antihistidyl transfer RNA [t-RNA] synthetase) is more frequent in patients with polymyositis than in patients with dermatomyositis. It is associated with pulmonary involvement (interstitial lung disease), Raynaud phenomenon, arthritis, and mechanic's hands.
  • Other MSAs include antisignal recognition protein (anti-SRP), associated with severe polymyositis, and anti–PM-Scl and anti-Ku, which are associated with overlapping features of myositis and scleroderma.

Imaging Studies

• Magnetic resonance imaging
• • MRI may be useful for assessing the presence of an inflammatory myopathy in patients without weakness.
  • MRI is useful in differentiating a steroid myopathy from continued inflammation.
  • MRI imaging may serve as a guide in selecting a muscle biopsy site.
• Chest radiographs should be obtained at the time of diagnosis and when symptoms develop.
• A barium swallow allows evaluation of esophageal dysmotility.
• Ultrasonography of the muscles has been suggested for evaluation, but it has not been widely accepted.
• Electromyography is a means of detecting muscle inflammation and damage and, at times, has been useful in selecting a muscle biopsy site. This test is obtained less commonly since the introduction of MRI of muscle.
• CT scans are useful in the evaluation of potential malignancy that might be associated with inflammatory myopathy.

Other Tests

• Pulmonary function studies
• Electrocardiography
• Esophageal manometry - May be obtained in select patients

Procedures

• A muscle biopsy sample, obtained either open or by a needle, may enhance the clinician's ability to diagnose dermatomyositis. The results of this biopsy may be useful in differentiating steroid myopathy from active inflammatory myopathy when patients have been on corticosteroid therapy but are still weak.

Histologic Findings

A skin biopsy sample reveals an interface dermatitis that is difficult to differentiate from LE. Vacuolar changes of the columnar epithelium and lymphocytic inflammatory infiltrates at the dermal-epidermal junction basement membrane can occur.

Muscle biopsy samples in patients with dermatomyositis reveal perivascular and interfascicular inflammatory infiltrates with adjoining groups of muscle fiber degeneration/regeneration. This contrasts with polymyositis infiltrates, which are mainly intrafascicular (endomysial inflammation) with scattered individual muscle fiber necrosis.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

The therapy of dermatomyositis involves general measures and measures to control both the muscle disease and the skin disease. In addition, some patients may need treatment for other systemic manifestations or complications.

• General treatments
• • Bedrest is often valuable for those patients with severe muscle inflammation.
  • In patients with muscle weakness, especially children, a program of physical therapy is useful to help prevent contractures that can complicate the disease when patients do not fully move their joints.
  • For patients with dysphagia, recommending elevating the head of their bed and not eating before bedtime is useful, possibly preventing aspiration pneumonitis. Occasionally, nasogastric tube feeding may be needed to increase caloric input.
• Treatments for muscle disease
• • The mainstay of therapy for the muscle disease is systemically administered corticosteroids. Traditionally, prednisone (1-2 mg/kg/d) is administered as initial therapy. Taper slowly to avoid relapse of the disease.
  • Because most patients develop steroid-related toxic effects, many authorities begin an immunosuppressive or cytotoxic agent early in the course.
  • Most clinical and published experience is with the use of methotrexate as a steroid-sparing agent, but azathioprine and mycophenolate mofetil have been used. Results with cyclophosphamide in severe cases have been disappointing.
  • In patients for whom these measures fail, the use of monthly high-dose intravenous immunoglobulin (IVIG) for 6 months has proved beneficial in the short term.
  • A recent report has suggested that rituximab, a chimeric antibody directed against CD20⁺ B cells, may be effective.³ However, other reports have failed to produce positive results.
• Treatments for skin disease
• • Therapy of the cutaneous disease is often difficult.
  • Some patients present primarily with skin disease (ie, amyopathic dermatomyositis), while other patients 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 protective measures, including broad-spectrum sunscreens.
  • Hydroxychloroquine and chloroquine have been beneficial in small, open-label case studies; however, roughly 20% of patients with dermatomyositis who are treated with an antimalarial agent develop a drug eruption.
  • Methotrexate is useful. Recently, mycophenolate mofetil has been reported to be useful. Sirolimus may also be of use in some patients.⁴
  • IVIG not only benefits the muscle but also clears the skin lesions.
• Calcinosis
• • This complication of the disease affects children and adolescents.
  • Some believe that aggressive early treatment of the myositis may aid in preventing this complication.
  • Once established, the process is debilitating in many patients. Although spontaneous remission is a possibility, 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.

Surgical Care

• Surgical care is usually not necessary.
• Some patients may request surgical removal of local areas of calcinosis.

Consultations

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

Diet

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

Activity

• Activity should be maintained as much as possible; however, avoid vigorous physical training when the myositis is active. Use exercises to maintain the patient's range of motion.
• Recommend sun avoidance and sun protective measures in patients with skin lesions.

MEDICATION

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Drug Category: Glucocorticoids

These agents may be used topically for cutaneous disease. The mainstay of therapy for patients with dermatomyositis and muscle involvement is systemic corticosteroids. Cutaneous disease has a variable response to systemic corticosteroids. Disease with pulmonary or cardiac involvement may respond, whereas disease involving esophageal dysfunction usually does not respond.

Drug Category: Immunosuppressive agents

These agents are used early in the course as steroid-sparing agents. Lowers risk of steroid-related complications.

Drug Category: Immune globulins

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

Drug Category: Calcium channel blockers

These agents may be useful to treat calcinosis.

Drug Category: Antimalarial agents

These agents may be used as steroid-sparing agents to treat skin disease. Hydroxychloroquine is preferred; chloroquine and quinacrine (100 mg/d) are second-line agents. Quinacrine may suppress bone marrow and is distributed by the Centers for Disease Control and Prevention (CDC); blood counts should be obtained regularly.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Inpatient Care

• Inpatient care is needed for patients with fulminant disease.

Further Outpatient Care

• Monitoring disease activity is necessary at least on a monthly basis. Repeat measurements of muscle enzymes may aid in assessment of the activity of the myositis, along with clinical assessment of patients' strength. Machines are available that can aid in the quantification of strength, but they are not used widely.
• Skin disease is assessed with physical examination in conjunction with history. A system for judging the activity and damage associated with dermatomyositis is being developed.
• Annual physical examinations are useful to monitor for potential toxicity from therapy or for the presence of a malignancy.
• Malignancy evaluations should be conducted for at least the first 3 years following diagnosis. A report by Hill et al (2001) suggested that the risk of malignancy never returns to baseline, even after 3 years; thus, vigilance is still warranted.⁵ The testing selected should be chosen based upon the patient's age, sex, race, and other symptoms or findings. Female patients should be screened for ovarian cancer. After 3 years, patients should be monitored as any other person of their same age, race, and sex.

In/Out Patient Meds

• Prednisone is a first-line therapy. The dose is altered depending on the response of the patient's condition.
• Immunosuppressive/cytotoxic agents are used as steroid-sparing agents for the muscle disease. Methotrexate has been demonstrated to be useful for the skin disease even in the absence of significant muscle disease. Mycophenolate mofetil may also be useful for cutaneous disease.
• Antimalarials, particularly hydroxychloroquine, may be useful for the cutaneous disease. An increased risk of drug eruptions may exist in patients with dermatomyositis.
• Intravenous immunoglobulin is used in patients for whom therapy with corticosteroids and immunosuppressives fails. This agent is useful for both the skin and the muscles.
• Biologic therapies including rituximab and TNF antagonists might be useful. However, their widespread use is discouraged until adequate, preferably controlled studies demonstrate efficacy and safety.

Transfer

• Patients 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.

Deterrence/Prevention

• Skin disease is exacerbated by sunlight and other sources of ultraviolet light. In addition, the muscle disease may be exacerbated. Sunscreens and sun protective measures are helpful in avoiding flares of the disease.

Complications

• Calcinosis may complicate dermatomyositis in children and adolescents.
• Contractures can occur if the patient is immobile.

Prognosis

• Patients with dermatomyositis who have malignancy, cardiac involvement, or pulmonary involvement or who are elderly (ie, >60 y) have a poorer prognosis.
• The disease may spontaneously remit in as many as 20% of the 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.
• Patients may visit The Myositis Association Web site for more information.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Failure to diagnose: The skin disease may be misdiagnosed as eczema, psoriasis, or LE early in the disease course.
• Failure to recognize an associated malignancy
• Failure to inform the patient about the potential toxicity of therapies

Special Concerns

• In older patients, the potential for an associated malignancy increases. Assessment for malignancy should be performed regularly, and the frequency should be based on the patient's sex, age, and race.
• Children and adolescents are much more prone to the development of calcinosis. Aggressive and early treatment may prevent this complication.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  The heliotrope flower from which the manifestation of dermatomyositis is named.
	View Full Size Image
Media type:  Photo

Media file 2:  Heliotrope rash in a woman with dermatomyositis.
	View Full Size Image
Media type:  Photo

Media file 3:  Gottron papules and nailfold telangiectasia are present in this patient with dermatomyositis.
	View Full Size Image
Media type:  Photo

Media file 4:  These lesions on the dorsal hands demonstrate the photodistribution of dermatomyositis. Note the sparing of the interdigital web spaces.
	View Full Size Image
Media type:  Photo

Media file 5:  A diffuse alopecia with a scaly scalp dermatosis is common in patients with dermatomyositis.
	View Full Size Image
Media type:  Photo

Media file 6:  Dermatomyositis is often associated with a poikiloderma in a photodistribution.
	View Full Size Image
Media type:  Photo

Media file 7:  The histopathology of dermatomyositis is an interface dermatitis.
	View Full Size Image
Media type:  Photo

Media file 8:  Calcinosis caused by dermatomyositis (DM) in childhood can be observed in a patient who had active DM 15 years before the time of this photograph.
	View Full Size Image
Media type:  Photo

Media file 9:  Histopathology of dermatomyositis showing inflammatory myopathic changes with a predominantly perivascular chronic inflammatory infiltrate.
	View Full Size Image
Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

1. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. Feb 20 1975;292(8):403-7. [Medline].
2. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. Feb 13 1975;292(7):344-7. [Medline].
3. Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum. 2005;52:601-7. [Medline].
4. Nadiminti U, Arbiser JL. Rapamycin (sirolimus) as a steroid-sparing agent in dermatomyositis. J Am Acad Dermatol. Feb 2005;52(2 Suppl 1):17-9. [Medline].
5. Hill CL, Zhang Y, Sigurgeirsson B, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. Jan 13 2001;357(9250):96-100. [Medline].
6. Airio A, Pukkala E, Isomaki H. Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol. Jul 1995;22(7):1300-3. [Medline].
7. Callen JP. Dermatomyositis. Lancet. Jan 1 2000;355(9197):53-7. [Medline].
8. Callen JP. Relation between dermatomyositis and polymyositis and cancer. Lancet. Jan 13 2001;357(9250):85-6. [Medline].
9. Callen JP. The value of malignancy evaluation in patients with dermatomyositis. J Am Acad Dermatol. Feb 1982;6(2):253-9. [Medline].
10. Callen JP. When and how should the patient with dermatomyositis or amyopathic dermatomyositis be assessed for possible cancer?. Arch Dermatol. Jul 2002;138(7):969-71. [Medline].
11. Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. Sep-Oct 2006;24(5):363-73. [Medline].
12. Chow WH, Gridley G, Mellemkjaer L, et al. Cancer risk following polymyositis and dermatomyositis: a nationwide cohort study in Denmark. Cancer Causes Control. Jan 1995;6(1):9-13. [Medline].
13. Dalakas MC. Molecular immunology and genetics of inflammatory muscle diseases. Arch Neurol. Dec 1998;55(12):1509-12. [Medline].
14. Dalakas MC, Illa I, Dambrosia JM, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med. Dec 30 1993;329(27):1993-2000. [Medline].
15. Daoud MS, Gibson LE, Pittelkow MR. Hydroxyurea dermopathy: a unique lichenoid eruption complicating long-term therapy with hydroxyurea. J Am Acad Dermatol. Feb 1997;36(2 Pt 1):178-82. [Medline].
16. Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Arch Dermatol. Jan 2006;142(1):65-9. [Medline].
17. Efthimiou P. Tumor necrosis factor-alpha in inflammatory myopathies: pathophysiology and therapeutic implications. Semin Arthritis Rheum. Dec 2006;36(3):168-72. [Medline].
18. el-Azhary RA, Pakzad SY. Amyopathic dermatomyositis: retrospective review of 37 cases. J Am Acad Dermatol. 2002;46:560-5. [Medline].
19. Euwer RL, Sontheimer RD. Amyopathic dermatomyositis (dermatomyositis sine myositis). Presentation of six new cases and review of the literature. J Am Acad Dermatol. Jun 1991;24(6 Pt 1):959-66. [Medline].
20. Fujisawa T, Suda T, Nakamura Y, et al. Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis. J Rheumatol. Jan 2005;32(1):58-64. [Medline].
21. Hengstman GJ, van den Hoogen FH, van Engelen BG. Treatment of dermatomyositis and polymyositis with anti-tumor necrosis factor-alpha: long-term follow-up. Eur Neurol. 2004;52(1):61-3. [Medline].
22. Kasteler JS, Callen JP. Low-dose methotrexate administered weekly is an effective corticosteroid-sparing agent for the treatment of the cutaneous manifestations of dermatomyositis. J Am Acad Dermatol. Jan 1997;36(1):67-71. [Medline].
23. Kasteler JS, Callen JP. Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed. JAMA. Dec 28 1994;272(24):1939-41. [Medline].
24. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology (Oxford). Mar 2005;44(3):386-9. [Medline].
25. Marie I, Hatron PY, Levesque H, et al. Influence of age on characteristics of polymyositis and dermatomyositis in adults. Medicine (Baltimore). May 1999;78(3):139-47. [Medline].
26. Mosca M, Strigini F, Carmignani A, et al. Pregnant patient with dermatomyositis successfully treated with intravenous immunoglobulin therapy. Arthritis Rheum. Feb 15 2005;53(1):119-21. [Medline].
27. O'Hanlon T, Koneru B, Bayat E, Love L, Targoff I, Malley J. Immunogenetic differences between Caucasian women with and those without silicone implants in whom myositis develops. Arthritis Rheum. Nov 2004;50(11):3646-50. [Medline].
28. Oliveri MB, Palermo R, Mautalen C, Hubscher O. Regression of calcinosis during diltiazem treatment in juvenile dermatomyositis. J Rheumatol. Dec 1996;23(12):2152-5. [Medline].
29. Pelle MT, Callen JP. Adverse cutaneous reactions to hydroxychloroquine are more common in patients with dermatomyositis than in patients with cutaneous lupus erythematosus. Arch Dermatol. Sep 2002;138(9):1231-3; discussion 1233. [Medline].
30. Pisoni CN, Cuadrado MJ, Khamashta MA, Hughes GR, D'Cruz DP. Mycophenolate mofetil treatment in resistant myositis. Rheumatology (Oxford). Mar 2007;46(3):516-8. [Medline].
31. Rowin J, Amato AA, Deisher N, Cursio J, Meriggioli MN. Mycophenolate mofetil in dermatomyositis: is it safe?. Neurology. Apr 25 2006;66(8):1245-7. [Medline].
32. Sigurgeirsson B, Lindelof B, Edhag O, Allander E. Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study. N Engl J Med. Feb 6 1992;326(6):363-7. [Medline].
33. Sontheimer RD. Skin manifestations of systemic autoimmune connective tissue disease: diagnostics and therapeutics. Best Pract Res Clin Rheumatol. Jun 2004;18(3):429-62. [Medline].
34. Sontheimer RD. The management of dermatomyositis: current treatment options. Expert Opin Pharmacother. May 2004;5(5):1083-99. [Medline].
35. Targoff IN. Dermatomyositis and polymyositis. Curr Probl Dermatol. 1991;3:131-180.
36. Villalba L, Hicks JE, Adams EM, et al. Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum. Mar 1998;41(3):392-9. [Medline].
37. Waldman MA, Callen JP. Self-resolution of Epstein-Barr virus-associated B-cell lymphoma in a patient with dermatomyositis following withdrawal of mycophenolate mofetil and methotrexate. J Am Acad Dermatol. Aug 2004;51(2 Suppl):S124-30. [Medline].
38. Weinel S, Callen JP. Calcinosis cutis complicating adult-onset dermatomyositis. Arch Dermatol. 2004;140:365-6. [Medline].
39. Werth VP, Callen JP, Ang G, Sullivan KE. Associations of tumor necrosis factor alpha and HLA polymorphisms with adult dermatomyositis: implications for a unique pathogenesis. J Invest Dermatol. Sep 2002;119(3):617-20. [Medline].
40. Wetter DA, Davis MD, Yiannias JA, et al. Effectiveness of intravenous immunoglobulin therapy for skin disease other than toxic epidermal necrolysis: a retrospective review of Mayo Clinic experience. Mayo Clin Proc. Jan 2005;80(1):41-7. [Medline].
41. Whitmore SE, Rosenshein NB, Provost TT. Ovarian cancer in patients with dermatomyositis. Medicine (Baltimore). May 1994;73(3):153-60. [Medline].
42. Woo TY, Callen JP, Voorhees JJ, et al. Cutaneous lesions of dermatomyositis are improved by hydroxychloroquine. J Am Acad Dermatol. Apr 1984;10(4):592-600. [Medline].
43. Yokoyama T, Sakamoto T, Shida N, et al. Fatal rapidly progressive interstitial pneumonitis associated with amyopathic dermatomyositis and CD8 T lymphocytes. J Intensive Care Med. May-Jun 2005;20(3):160-3. [Medline].
44. Zieglschmid-Adams ME, Pandya AG, Cohen SB, Sontheimer RD. Treatment of dermatomyositis with methotrexate. J Am Acad Dermatol. May 1995;32(5 Pt 1):754-7. [Medline].

Article Last Updated: Feb 14, 2008