- Author: Alisa N Femia, MD; Chief Editor: Herbert S Diamond, MD more...
Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous findings that occur in children and adults (see the image below). This systemic disorder most frequently affects the skin and muscles but may also affect the joints; the esophagus; the lungs; and, less commonly, the heart.[1, 2] Dystrophic calcinosis may complicate dermatomyositis and is most often observed in children and adolescents.
Signs and symptoms
Persons with dermatomyositis often present with skin disease as one of the initial manifestations, and it may be the sole manifestation at onset in perhaps as many as 40% of individuals with this condition. Cutaneous involvement may manifest as follows:
Eruption predominantly on photo-exposed surfaces
Pruritus of skin lesions, sometimes intense enough to disturb sleep
Erythema of the mid-face
Eruption along the eyelid margins, with or without periorbital edema
Eruption on the dorsal hands, particularly over the knuckles
Changes in the nailfolds of the fingers
Eruption of the upper outer thighs
Scaly scalp or diffuse hair loss 
Muscle disease may occur concurrently, may precede the skin disease, or may follow the skin disease by weeks to years. Muscle involvement manifests as the following:
Proximal muscle weakness
Muscle fatigue/weakness when climbing stairs, walking, rising from a seated position, combing hair, or reaching for items above shoulders
Muscle tenderness: May occur, but not a typical feature of dermatomyositis
Systemic manifestations that may occur include the following:
General systemic disturbances, fever, arthralgia, malaise, weight loss, Raynaud phenomenon
Dysphagia due to esophageal skeletal muscle involvement
Gastroesophageal reflux due to esophageal smooth muscle involvement
Atrioventricular defects, tachyarrhythmias, dilated cardiomyopathies
Gastrointestinal ulcers and infections, more common in children
Pulmonary involvement due to weakness of thoracic muscles, interstitial lung disease
Subcutaneous calcification,  which may result in contracture of joints; more common in children
Children may also develop a tiptoe gait secondary to flexion contracture of the ankles in early childhood
Malignancy in adult patients
See Clinical Presentation for more detail.
Examination for cutaneous dermatomyositis may reveal the following findings:
Characteristic, possibly pathognomonic cutaneous features: Heliotrope, Gottron papules
Characteristic but not pathognomonic features: Malar erythema, violaceous erythema or poikiloderma in a photosensitive distribution, violaceous erythema on the extensor surfaces, and periungual and cuticular changes
- Violaceous erythema or poikiloderma involving the anterior chest is referred to as the “V-neck sign” whereas involvement of the upper back and shoulders is referred to as the “shawl sign”
Rare cutaneous manifestations include vesiculobullous erosive lesions and an exfoliative erythroderma, which may be more common in patients with an associated malignancy than in those without a malignancy; biopsy samples of these manifestations reveal an interface dermatitis similar to that seen in biopsy samples of heliotrope rash, Gottron papules, poikiloderma, or scalp lesions
Examination for muscle disease in dermatomyositis may demonstrate the following:
Quadriparesis involving proximal musculature
Difficulty rising from a seated or supine position without support
Extensor muscles often more affected than the flexor muscles
Neck flexor muscle weakness
Distal strength, sensation, and tendon reflexes maintained (unless the patient has severely weak and atrophic muscle)
Laboratory and other studies that may be helpful include the following:
Muscle enzyme levels (eg, creatine kinase, aldolase, aspartate aminotransferase, lactic dehydrogenase)
Antinuclear antibody levels
Pulmonary function studies with diffusion capacity
Colonoscopy to screen for underlying malignancy
Papanicolaou smear in women for malignancy screening
CA-125 and CA-19-9 for malignancy screening
The following imaging studies may be used in the evaluation of dermatomyositis:
MRI or ultrasonography of the muscles
Imaging to screen for underlying malignancy, including CT scanning of the chest, abdomen, and pelvis, as well as transvaginal ultrasound and mammography for women
The following procedures may be helpful in the evaluation of dermatomyositis:
Muscle biopsy (open or via a needle): Findings can be diagnostic (perivascular and interfascicular inflammatory infiltrates with adjoining groups of muscle fiber degeneration/regeneration)
See Workup for more detail.
Therapy for the muscle component of dermatomyositis involves the use of corticosteroids, typically with an immunosuppressive agent. Therapy for the skin disease includes the following, among other options:
Sunscreens and photoprotective clothing
Medications used in the management of dermatomyositis include the following:
Corticosteroids (eg, prednisone): Prednisone is a first-line therapy for muscle involvement in dermatomyositis
Immunosuppressive agents (eg, methotrexate, mycophenolate mofetil, azathioprine, rituximab, sirolimus)
Immune globulins (eg, intravenous or subcutaneous immunoglobulin)
Antimalarial agents (eg, hydroxychloroquine, chloroquine)
In addition to the medications listed above, diltiazem, colchicine, alendronate, and warfarin are among the medications that have shown potential benefit in treating calcinosis. Surgical excision of focal, tender calcinotic lesions is also considered a therapeutic option.
General therapeutic measures may include the following:
Physical therapy and rehabilitative measures
Sun-protection (eg, broad-spectrum sunscreens, sun protective clothing)
Elevation of head of bed
Avoidance of eating before bedtime
Surgical care is usually unnecessary in the management of dermatomyositis. However, some patients may benefit from surgical removal of localized areas of calcinosis, particularly those that are painful.
Dermatomyositis is an idiopathic inflammatory myopathy (IIM) with characteristic cutaneous findings. It is a systemic disorder that most frequently affects the skin and muscles, but may also affect the joints; the esophagus; the lungs; and, less commonly, the heart.[1, 2] Calcinosis is a complication of dermatomyositis that is observed most often in children and adolescents. An association between dermatomyositis and cancer has long been recognized in adult patients.[5, 6, 7, 8, 9, 10]
In 1975, Bohan and Peter first suggested a set of five criteria to aid in the diagnosis and classification of dermatomyositis.[11, 12] Four of the five criteria are related to the muscle disease, as follows:
Progressive proximal symmetrical weakness
Elevated levels of muscle enzymes
An abnormal finding on electromyography
An abnormal finding on muscle biopsy
The fifth criterion is compatible cutaneous disease.
In addition to dermatomyositis, Bohan and Peter suggested the following four subsets of myositis :
Myositis with malignancy
Myositis overlapping with another collagen-vascular disorder
In a subsequent publication, Bohan et al noted that cutaneous disease may precede the development of the myopathy in patients with dermatomyositis. In addition, the existence of another subset of patients with dermatomyositis that affects only the skin (ie, amyopathic dermatomyositis [ADM], or dermatomyositis sine myositis) has been recognized. Finally, another subset of patients with dermatomyositis are those with controlled myopathy who continue to have severe and sometimes debilitating skin disease (ie, postmyopathic dermatomyositis).
ADM is diagnosed in patients with typical cutaneous disease who show no evidence of muscle weakness and in whom serum muscle enzyme levels are repeatedly normal over a 2-year period in the absence of the use of disease-modifying therapies such as corticosteroids, immunosuppressive agents, or both for 2 months or longer.
When studied, some ADM patients may have abnormal findings on ultrasonography, electromyography, magnetic resonance imaging (MRI), magnetic resonance spectroscopy, or muscle biopsy. These patients are better classified as having hypomyopathic dermatomyositis. ADM or hypomyopathic DM may also be related to an underlying malignancy.
The term clinically amyopathic dermatomyositis (CADM) is often used to encompass patients with both amyopathic and hypomyopathic dermatomyositis. CADM is estimated to account for about 20% of patients with dermatomyositis, and one large review suggests that CADM is associated with malignancy and lung disease as frequently as classic dermatomyositis. In addition, some patients with CADM develop severe pulmonary disease, particularly persons from Asian countries.
Patients exist in whom myositis resolves after therapy but skin disease remains an active and important feature of the disorder. These patients are not classified as having ADM, even though by this point, the skin is the major and often only manifestation of the disease. Germani and colleagues have suggested the term postmyopathic dermatomyositis for these patients.
Therapy for the muscle component of dermatomyositis involves the use of systemic corticosteroids, with or without an immunomodulatory agent. The skin disease is treated with sun avoidance, sunscreens, photoprotective clothing, topical corticosteroids, antimalarial agents, methotrexate, mycophenolate mofetil, or intravenous immunoglobulin (IVIG). Systemic corticosteroids are generally not administered for cutaneous involvement. Rituximab may be useful in the treatment of muscle disease in dermatomyositis, and has had mixed results in treatment of skin disease.[18, 19]
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.
The prognosis of dermatomyositis depends on the severity of the myopathy, the presence of malignancy, and/or the presence of esophageal and/or cardiopulmonary involvement. Residual weakness is common, even in patients who fully recover.
For discussion of dermatomyositis in pediatric patients, see Juvenile Dermatomyositis.
Dermatomyositis is considered to be the result of a humoral attack against the muscle capillaries and small arterioles (endothelium of the endomysial blood vessels). Since 1966, there has been evidence supporting an ongoing microangiopathy.
The disease starts when putative antibodies or other factors activate C3, forming C3b and C4b fragments that lead to formation of C3bNEO and membrane attack complex (MAC), which are deposited in the endomysial vasculature. Complement C5b-9 MAC is deposited and is needed in preparing the cell for destruction in antibody-mediated disease. B cells and CD4 (helper) cells are also present in abundance in the inflammatory reaction associated with the blood vessels.
As the disease progresses, the capillaries are destroyed, and the muscles undergo microinfarction. Perifascicular atrophy occurs in the beginning; however, as the disease advances, necrotic and degenerative fibers are present throughout the muscle.
The pathogenesis of the cutaneous component of dermatomyositis is poorly understood, but is thought to be similar to that of muscle involvement.
Studies on the pathogenesis of the muscle component have been controversial. Some suggest that the myopathy in dermatomyositis is pathogenetically different from that in polymyositis. The former is probably caused by complement-mediated (terminal attack complex) vascular inflammation, the latter by the direct cytotoxic effect of CD8+ lymphocytes on muscle. However, other cytokine studies suggest that some of the inflammatory processes may be similar. One report has linked tumor necrosis factor (TNF) abnormalities with dermatomyositis.
The cause of dermatomyositis is unknown. However, genetic, immunologic, infectious, and environmental factors have been implicated.
A genetic component may predispose to dermatomyositis. Dermatomyositis rarely occurs in multiple family members, but a link to certain human leukocyte antigen (HLA) types (eg, DR3, DR5, DR7) may exist.
Polymorphisms of tumor necrosis factor (TNF) may be involved; specifically, the presence of the -308A allele is linked to photosensitivity in adults and calcinosis in children.[21, 22, 23] A meta-analysis demonstrated that the TNF-α-308A/G polymorphism might contribute to dermatomyositis susceptibility, especially in a European population.
Immunologic 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 disease and the muscle disease. In addition, family members may manifest other diseases associated with autoimmunity.
Antinuclear antibodies (ANAs) and antibodies to cytoplasmic antigens (ie, antitransfer RNA synthetases) may be present. Although their presence may help to define subtypes of dermatomyositis and polymyositis, their role in pathogenesis is uncertain.
Infectious agents have been suggested as possible triggers of dermatomyositis. These include the following:
Cases of drug-induced dermatomyositis have been reported. Dermatomyositis-like skin changes have been reported with hydroxyurea in patients with chronic myelogenous leukemia or essential thrombocytosis.[25, 26] Other agents that may trigger the disease include the following:
Anti–tumor necrosis factor drugs
Bacillus Calmette-Guérin (BCG) vaccine
Dermatomyositis may be initiated or exacerbated by silicone breast implants or collagen injections, but the evidence for this is anecdotal and has not been verified in case-control studies. One report detailed HLA differences among women in whom inflammatory myopathy developed after silicone implants.
The estimated incidence of dermatomyositis is 9.63 cases per million population. The estimated incidence of AMD is 2.08 cases per million.
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, whereas in children, the peak age is approximately 5-10 years. Dermatomyositis and polymyositis are twice as common in women as in men. Neither condition shows any racial predilection.
Most patients with dermatomyositis survive, in which case they may develop residual weakness and disability. Children with severe dermatomyositis may develop contractures. The disease may spontaneously remit in as many as 20% of affected patients. About 5% of patients have a fulminant progressive course with eventual death. Therefore, many patients require long-term therapy.
Patients with dermatomyositis who have an associated malignancy; those with cardiac, pulmonary, or esophageal involvement; and those who are elderly (ie, >60 years) have a poorer prognosis. Dermatomyositis may cause death because of muscle weakness or cardiopulmonary involvement. Patients with an associated cancer may die of the malignancy.
The association between malignancy and dermatomyositis has long been recognized. An estimated 25% of patients with dermatomyositis have or will develop an associated malignancy, and the risk appears to remain elevated for 3-5 years.[28, 29, 30] Strong data from Scandinavia, Australia, North America, and Asia continue to confirm this association with malignancy, and existing data supports that patients with CADM have a similar malignancy risk to those with classic dermatomyositis.[17, 29, 30, 31, 32, 33, 34]
Ovarian cancer is clearly over-represented in patients with dermatomyositis; however, any malignancy may occur. Reported maligancies include lung, colon, prostate, breast, pancreatic, cervical, and hematologic malignancies. Predilection for certain types of malignancy may be more common in specific populations. For example, nasopharyngeal carcinoma appears to be over-represented in certain Asian populations.[36, 37, 38]
In a recently published, approximately 10-year retrospective study from southern China, 60 of 246 dermatomyositis patients developed malignancies. The risk of malignancy was highest in the first year after diagnosis of dermatomyositis, and nasopharyngeal carcinoma and ovarian carcinoma were the most common malignancies. Male gender, dysphagia and elevated erythrocyte sedimentation rate were risk factors for malignancy, whereas the presence of interstitial lung disease appeared to reduce the risk of malignancy. Older age appears to be the strongest predictor of malignancy in patients with dermatomyositis.
Calcinosis may also complicate dermatomyositis. It is rare in adults but is more common in children and has been linked to delay in diagnosis and to less-aggressive therapy. Contractures can occur if the patient is immobile.
Population-based studies from British Columbia concluded that patients with dermatomyositis (or polymyositis) are at increased risk for venous thromboembolism (deep venous thrombosis or pulmonary embolism) and myocardial infarction, especially in the first year after diagnosis. However, dermatomyositis was not associated with an increased risk of ischemic stroke.
A study from Taiwan reported that the risk of osteoporosis in persons with dermatomyositis (or polymyositis) was 2.99 times higher than in those without these disorders. This risk was independent of treatment with corticosteroids and immunosuppressant drugs.
African Americans and patients in lower socioeconomic groups are more likely to experience a delay in diagnosis. The prognosis in children with dermatomyositis is worse in those in whom diagnosis is delayed.
Callen JP. Dermatomyositis. Lancet. 2000 Jan 1. 355(9197):53-7. [Medline].
Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. 2006 Sep-Oct. 24(5):363-73. [Medline].
Kasteler JS, Callen JP. Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed. JAMA. 1994 Dec 28. 272(24):1939-41. [Medline].
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].
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].
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].
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].
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].
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].
Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975 Feb 20. 292(8):403-7. [Medline].
Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975 Feb 13. 292(7):344-7. [Medline].
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].
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].
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].
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].
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].
Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum. 2005 Feb. 52(2):601-7. [Medline].
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].
Banker BQ, Victor M. Dermatomyositis (systemic angiopathy) of childhood. Medicine (Baltimore). 1966 Jul. 45(4):261-89. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Ang GC, Werth VP. Combination antimalarials in the treatment of cutaneous dermatomyositis: a retrospective study. Arch Dermatol. 2005 Jul. 141(7):855-9. [Medline].
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].
Chérin P. [Current therapy for polymyositis and dermatomyositis]. Rev Med Interne. 2008 Jun. 29 Spec No 2:9-14. [Medline].
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].
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].
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].
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].
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].
Boswell JS, Costner MI. Leflunomide as adjuvant treatment of dermatomyositis. J Am Acad Dermatol. 2008 Mar. 58(3):403-6. [Medline].
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].
Bunch TW. Prednisone and azathioprine for polymyositis: long-term followup. Arthritis Rheum. 1981 Jan. 24(1):45-8. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology (Oxford). 2005 Mar. 44(3):386-9. [Medline].
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].
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].
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].
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].
Cohen JB. Cutaneous involvement of dermatomyositis can respond to Dapsone therapy. Int J Dermatol. 2002 Mar. 41(3):182-4. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].