Jeffrey P. Callen
Introduction
Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies (IIM) (Callen 2000; Plotz et al. 1995; Targoff 1991). In 1975, Bohan and Peter pub- lished a classical article that first suggested a set of criteria to aid in the diag- nosis and classification of DM and polymyositis (PM). Four of the five criteria related to the muscle disease: 1) progressive proximal symmetrical weak- ness 2) elevated muscles enzymes, 3) an abnormal electromyogram and 4) an abnormal muscle biopsy, while the fifth was the presence of compatible cutaneous disease. It was felt that DM differed from PM only by the presence of cutaneous disease. Recent studies of the pathogenesis of the myopathy have been controversial, some suggesting that the myopathies in DM and PM are pathogenetically different with DM being due to a vascular inflamma- tion (Kuru et al. 2000) while other studies of cytokines suggest that the pro- cesses are similar (Wanchu et al. 1999; Shimizu et al. 2000; Sugiura et al. 2000;
Nyberg et al. 2000). There has been a renewed interest in the pathogenetic mechanisms involved in the myopathy with recent studies revealing abnor- mal levels of nitric oxide, elevation of circulating tumor necrosis factor recep- tors, elevated soluble CD40 expression, and increased expression of interleukin 1-alpha within the muscle. The pathogenesis of the cutaneous disease is poorly understood.
Classification and Clinical Appearance
Classification
Bohan and Peter (1975) suggested five subsets of myositis – DM, PM, myositis
with cancer, childhood DM/PM, and myositis overlapping with another col-
lagen-vascular disorder. In a subsequent publication, Bohan et al. (1977) noted
that cutaneous disease may precede the development of the myopathy, how- ever it was only recently recognized that another subset of patients with dis- ease that only affects the skin (amyopathic dermatomyositis) [ADM] or DM-sine myositis may occur (Euwer and Sontheimer 1991). A seventh subset known as inclusion body myositis (IBM) has more recently been recognized (Sayers et al. 1992). Perhaps there is an eighth group in which characteristic cutaneous disease is drug-induced (Dourmishev and Dourmishev 1999). Finally, Son- theimer has proposed another subset that classifies patients as hypomyopa- thic dermatomyositis when skin disease is present with subtle muscle disease evident with studies other than enzymatic analysis and a post-myopathic DM for those patients in whom the myositic component of the disease resolves while the skin disease remains active (Sontheimer 2002).
Cutaneous Manifestations
The characteristic and possibly pathognomonic cutaneous features of DM are the heliotrope rash and Gottron’s papules. The heliotrope rash consists of a violaceous to dusky erythematous rash with or without edema in a symmetri- cal distribution involving periorbital skin (Fig. 1). Sometimes this sign is quite subtle and may involve only a mild discoloration along the eyelid margin. A heliotrope rash is rarely observed in lupus erythematosus (LE) and sclero- derma. Occasionally patients may be felt to have angioedema or dermatitis.
Gottron’s papules are found over bony prominences, particularly the meta- carpophalangeal joints, the proximal interphalangeal joints, and/or the distal interphalangeal joints (Fig. 2). They may also be found overlying the elbows, knees and/or feet. The lesions consist of slightly elevated, violaceous papules and plaques. There may be a slight scale and on some occasions there is a
Fig. 1.Facial erythema with periorbital edema are present in this woman with dermatomyositis.
The periorbital changes represent the heliotrope eruption
thick psoriasiform scale. Within the lesions, there is often telangiectasia. These lesions may be clinically confused with lesions of LE, or, at times, with papu- losquamous disorders, such as psoriasis or lichen planus. Routine histopa- thological evaluation will aid in the differentiation from psoriasis of lichen planus, but cannot reliably distinguish the cutaneous lesions of DM from those of LE.
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. Nailfold changes consist of periungual telangiectasia and/or a characteristic cuticular change with hypertrophy of the cuticle and small, hemorrhagic infarcts with this hypertrophic area. Peri- ungual telangiectasia may be clinically apparent or may be appreciated only by capillary microscopy. Poikiloderma (the combination of atrophy, dyspig- mentation and telangiectasia) may occur on exposed skin such as the extensor surfaces of the arm, the ‘V’ of the neck (Fig. 3) or the upper back (Shawl sign).
Patients rarely complain of photosensitivity, despite the prominent photodis-
Fig. 2.Gottron’s papules. Erythematous scaly plaques are present on the dorsal hands, particularly over the bonfy prominences (MCP, PIP and DIP joints). There is also marked
periungual telangiectasia and cuticular overgrowth
tribution of the rash. This photosensitive poikilodermatous eruption may be difficult to differentiate from LE. Facial erythema may also occur in DM. This change must be differentiated from LE, rosacea, seborrheic dermatitis or atopic dermatitis. Scalp involvement in dermatomyositis is relatively common and is manifest by an erythematous to violaceous, psoriasiform dermatitis (Kasteler and Callen 1994). Clinical distinction from seborrheic dermatitis or psoriasis is occasionally difficult, but histopathologic evaluation is helpful. Nonscar- ring alopecia may occur in some patients and often follows a flare of the sys- temic disease. Lastly, recent reports have detailed the finding of gingival telangiectasia (Ghali et al. 1999) and angiokeratomas (Shannon and Ford 1999) in children with DM.
DM-sine myositis, also known as amyopathic DM (ADM), is diagnosed in patients with typical cutaneous disease in whom there is no evidence of mus- cle weakness and who repeatedly have normal serum muscle enzyme levels (Bohan et al. 1977; Rockerbie et al. 1989; Stonecipher 1993; Cosnes et al. 1995).
Some patients with ‘ADM’ when studied will have an abnormal ultrasound, magnetic resonance imaging or muscle biopsy. These patients have muscle involvement, and may be better classified as hypomyopathic DM (Sonthei- mer 2002). Since many of the ADM patients are not evaluated beyond clini- cal and enzymatic studies, many feel that the ADM patient represents a systemic process requiring systemic therapies. There are also a fair number of patients whose myositis resolves following therapy, but whose skin disease
Fig. 3.Poikiloderma of the upper chest in a patient with dermatomyositis
remains as an active, important feature of the disease. These patients are called Post-myopathic DM, and the skin is their major and often only mani- festation of the disease. There is also a small subset of patients who never develop myositis, despite having prominent cutaneous changes, and it is these patients who can be classified as amyopathic DM assuming that they have not received systemic corticosteroids or immunosuppressive agents.
Patients with DM are at times difficult to distinguish from patients with subacute cutaneous lupus erythematosus (SCLE). The lesions of DM differ slightly in their distribution, occurring more over bony prominences, and they are frequently accompanied by severe pruritus; whereas LE lesions tend to occur between the knuckles and are usually asymptomatic. Routine skin bi- opsy is not helpful in the distinction between LE and DM. Immunofluores- cence microscopy (IF) should be negative in DM and positive in LE, however, about 50% of SCLE patients have a negative IF, and IF may be falsely posi- tive on sun-exposed skin. Serologic testing is also imperfect, since only 25%
of DM patients are Mi2 positive, and on a single testing only 60–70% of SCLE patients are Ro (SS-A) antibody positive.
The skin lesions of DM are probably photoaggravated despite the lack of symptoms suggestive of photosensitivity reported by patients (Cheong et al.
1994). Clinical observations suggest that not only is the skin disease exacer- bated by light, but muscle disease may be worsened after sun exposure (Woo et al. 1985; Callen 1993; Zuber et al. 1996; Callen 1999). However, phototesting has not been able to reliably reproduce the skin lesions, thus, the wave- length of light that is responsible for the clinical manifestations (action spec- trum) is not known.
Rare cutaneous manifestations include vesiculobullous lesions (McCollough and Cockerell 1998), an eruption that simulates pityriasis rubra pilaris (Requena et al. 1997), a flagellate erythema (Nousari et al. 1999), vasculitis, erosive le- sions, as well as an exfoliative erythroderma. In small case series it has been suggested that some of these cutaneous manifestations may be more com- mon in patients with an associated malignancy.
Skin lesions of DM may precede the development of myopathy and may persist well after the control and quiescence of the myositis. Patients skin le- sions may flare with sun exposure, but only some of these patients will have a flare of their muscle involvement. Thus, in many instances the course of the skin lesions does not parallel that of the muscle disease.
A variety of other cutaneous lesions have been described in patients with
DM or PM that do not reflect the interface changes observed histopathologi-
cally with the pathognomonic or characteristic lesions. These include pannicu-
litis, plaque-like mucinosis (Fig. 4) (Abernethy et al. 1996; Kaufman et al. 1998),
a flagellate eruption (Nousari et al. 1999), urticaria, as well as changes of hyper-
keratosis of the palms known as mechanics hands. Lastly, children with DM
may develop calcinosis, but in addition insulin-resistance and lipodystrophy
has recently been reported as a relatively common complication despite ade-
quate therapy (Heumer et al 2001).
Muscle Disease
Clinical and laboratory abnormalities suggestive of muscle disease are char- acteristic features of DM (Wortmann 2000). The myopathy primarily affects the proximal muscles, is usually symmetrical and is slowly progressive over a period of weeks to months. Initial complaints including myalgias, fatigue, or weakness manifest as an inability to climb stairs, to raise the arms for actions like hair grooming or shaving, to rise from a squatting or sitting position, or a combination of these features. Tenderness upon palpation of the muscles is variable. An inability to swallow and symptoms of aspiration may reflect the involvement of striated muscle of the pharynx or upper esophagus. Dysphagia or dysphonia generally signifies a rapidly progressive course and may be as- sociated with poor prognosis.
Systemic Features
Dermatomyositis is a multisystem disorder (Spiera and Kagen 1998). Arthral- gias and/or arthritis may be present in up to one fourth of patients with in- flammatory myopathy. The usual picture is one of generalized arthralgias
Fig. 4.Papular mucinosis-like lesions in a patient with dermatomyositis. Prominent papular lesions are present within the poikilodermatous eruption. Histopathology revealed deposition
of massive amounts of mucin in the dermis
accompanied by morning stiffness. The small joints of the hands, wrists, and ankles may be involved with a symmetrical nondeforming arthritis.
Esophageal disease as manifested by dysphagia is estimated to be pres- ent in 15% to 50% of patients with inflammatory myopathy. The dysphagia can be of two types: proximal dysphagia or distal dysphagia. Proximal dyspha- gia is caused by involvement of striated muscle in the pharynx or proximal esophagus. This involvement correlates well with the severity of the muscle disease and is steroid-responsive. Distal dysphagia is related to involvement of nonstriated muscle and appears to be more frequent in patients who have an overlap with scleroderma or another collagen vascular disorder. Dyspha- gia is associated with a poor prognosis and correlates with the presence of pulmonary involvement.
Pulmonary disease occurs in dermatomyositis and polymyositis in ap- proximately 15% to 30% of patients (Marie et al. 1998). Interstitial pneumo- nitis is a primary process observed in DM/PM. It is more frequent in patients with esophageal involvement. Lung disease may also occur as a direct com- plication of the muscle disease, such as hypoventilation or aspiration in pa- tients with dysphagia, or may be a result of treatment, as with opportunistic infections or drug-induced hypersensitivity pneumonitis. In a retrospective review of 70 patients with myositis associated interstitial lung disease seen at Mayo Clinic between 1990 and 1998, most of the patients presented with ei- ther symptoms of lung disease or symptoms of myositis alone, with only 15 in whom the involvement occurred simultaneously (Douglas et al 2001). In gen- eral, the lung disease was at first felt to be a pneumonitis that was antibiotic resistant. Biopsy of the lung revealed non-specific interstitial pneumonitis or diffuse alveolar damage in a majority of those who were biopsied. Only two patients had bronchiolitis obliterans organizing pneumonia (BOOP). It is un- clear how many of the patients had dermatomyositis, but perhaps between 8 and 12. Therapy included corticosteroids with or without an immunosup- pressive agent and the prognosis is poorer for these patients than unselected patients with myositis as demonstrated by a 5-year survival of 60.4%. Pa- tients with Jo-1 antibodies (19 of 50 who were tested) had roughly the same features and prognosis as those who did not have this antibody.
Clinically symptomatic cardiac involvement in patients with DM or PM is uncommon, but when present it is associated with a poor prognosis (Gonzalez- Lopez et al. 1996). Various abnormalities have been described which include conduction defects, and rhythm disturbances primarily. Although congestive heart failure, pericarditis, and valvular disease may occur, they are much less frequent. Depending on the report, cardiac manifestations may occur in up to 50% of patients, but only a small proportion of these patients manifest symptoms. It is not known whether the identification of asymptomatic abnor- malities has an effect on long-term outcome, or even if the findings are more prevalent in DM/PM than in an age-matched control group.
Calcinosis of the skin or muscle is unusual in adults but may occur in up to
40% of children or adolescents with DM. Calcinosis cutis is manifested by firm,
yellow or flesh-colored nodules, often over bony prominences. Occasionally, these nodules can extrude through the surface of the skin, in which case sec- ondary infection may occur. Calcification of the muscles is often asymptom- atic and may be seen only on radiological examination. In severe forms, the calcinosis can cause loss of function, and, rarely bone formation is possible.
Myositis and Malignancy
The relationship of DM-polymyositis to malignancy has been recently clari- fied (Hill et al. 2001). The reported frequency of malignancy in dermatomyo- sitis has varied from 6% to 60% with most large population-based cohort studies revealing a frequency of about 20–25%.
Several Scandinavian studies have documented the increased frequency of malignancy in DM over the general population (Siguregeirsson et al. 1992;
Chow et al. 1995; Ario et al. 1995; Hill et al. 2001). While polymyositis pa- tients had a slight increase in cancer frequency; it was not highly significant and could be explained by a more aggressive cancer search (known as diag- nostic suspicion bias). These studies have not dealt with the amyopathic DM subset, but data from Mayo Clinic suggests that these patients may also have an associated malignancy (el-Azhary and Pakzad 2002). Malignancies may occur prior to the onset of myositis, concurrently with myositis, or after the onset of DM. In addition, the myositis may follow the course of the malignancy (a paraneoplastic course) or may follow its own course independent of the treatment of the malignancy. Studies demonstrating benefits of cancer sur- gery on myositis as well as those showing no relationship of the myositis to the malignancy have been reported.
A wide variety of malignancies have been reported in patients with DM.
Gynecologic malignancy, in particular ovarian carcinoma may be overrepre- sented in DM (Whitmore et al. 1994; Hill et al. 2001). Asians with DM are of- ten found to have nasopharyngeal cancer (Peng et al. 1995). In the recent analysis of combined data from Scandinavia, Hill et al. (2001) again noted the increased association of ovarian cancer, but also noted increases in lung, pancreatic, stomach, colorectal cancer and non-Hodgkin’s lymphoma. Ma- lignancy is more common in older patients (> 50 years) (Marie et al. 1998;
Pautas et al. 2000), but reports of young adults and rarely even children with
DM have appeared, suggesting that age alone should not dissuade the phy-
sician from a careful evaluation (see below). The site of malignancy can be
predicted by the patient’s age (e.g., malignancy in a young man is more of-
ten testicular cancer, whereas, in an elderly male, colon or prostate cancer
would be more common). In the past, there was concern about whether the
use of immunosuppressive therapies would predispose the patient to an ex-
cess cancer risk. This has not proven to be the case with most cancers being
reported within the first three years following diagnosis.
Juvenile (Childhood) Dermatomyositis (JDMS)
DM is much more common than polymyositis in children and adolescents (Pachman 1995). A fulminant course may be present, but most often the on- set is indolent and children are first thought to have viral infections or der- matitis. Delayed diagnosis is more common in the non-white population. Often JDMS is characterized as a vasculitis, but the major difference of JDMS from adult DM is the greater potential for calcinosis. A recent report detailed the chronic nature of this disease in children, with many patients requiring ther- apy to suppress their disease activity more than three years after diagnosis (Huber et al. 2000). In addition, it was noted that the development of calcinosis was not related to initial therapy, but was associated with a lower score on an assessment instrument of physical function. Pachman et al. (2000) linked the presence of calcinosis and a prolonged course of disease with TNF-308A al- lele in their patients with juvenile DM.
Drug-Induced Dermatomyositis
The etiology of most cases of DM is unknown, however, in a small number of patients, the cutaneous manifestations are due to, or are exacerbated by drugs.
This has been best documented for hydroxyurea in which de-challenges and re-challenges have been performed (Daoud et al. 1997; Marie et al. 2000, Dacey and Callen 2003). However, quinidine, non-steroidal anti-inflammatory drugs, D-penicillamine, and HMG-CoA-reductase inhibitors have also been linked on occasion to DM (Dourmishev and Dourmishev 1999).
Diagnosis and Evaluation of the Patient with Dermatomyositis
The diagnosis of DM is suspected in patients with clinically compatible cuta- neous findings. Exclusion of other possible cutaneous conditions is aided by skin biopsy and the recognition of muscle involvement. In the absence of iden- tifiable myopathy, the differentiation from cutaneous LE may be difficult.
Muscle weakness may be caused by many other disorders including toxins, infections, metabolic abnormalities, and neurologic disorders (Wortmann 2000).
However, the presence of characteristic skin lesions allows the diagnosis to be more firmly established.
Muscle involvement is suspected clinically. Enzymatic testing will reveal
elevations of creatine kinase, aldolase, lactic dehydrogenase or ALT. The CK
seems most specific and most widely available and is a useful test for follow-
ing response to therapy. Additional testing including electromyography (EMG),
muscle biopsy, ultrasound or MR imaging may be ordered in patients in whom other tests are inconclusive (Garcia 2000).
Serologic tests are often ordered, but their clinical application is at best controversial. Antinuclear antibody testing is frequently positive in DM pa- tients. Several myositis-specific antibodies (MSAs) have been recognized and correlate with certain subsets (Love et al. 1991). Most MSAs are described in PM patients and will not be further discussed here. Anti-Jo-1 antibody is predictive of pulmonary involvement, but rarely occurs in DM patients.
Anti-Mi-2 occurs in roughly 25% of DM patients and although specific for DM, it is not sensitive. Recently Targoff et al. (2000) described a new anti- body to a 155 kd antigen or Se antigen (90–95 kd) that appears to be a marker of amyopathic DM (16 of 18 patients studied). The anti-155 kd antibody may also be associated with juvenile DM and might predict a chronic course.
Anti-Ro (SS-A) antibody may occur rarely. Perhaps as further studies are performed, serologic testing will become clinically useful. Until then, these tests are primarily reserved for investigation.
Once the diagnosis is confirmed, the patient should have a thorough eval- uation. Evaluation has several purposes: assessment of severity, prediction of prognosis and identification of associated disorders. The severity of the myositis often correlates with enzyme levels and degree of weakness. Pa- tients should be assessed for esophageal, pulmonary, and cardiac involvement with tests such as a barium swallow and/or esophageal motility studies, chest x-ray, pulmonary function studies including diffusion studies, and an elec- trocardiogram.
An evaluation of malignancy should be considered in all adult patients with DM (Callen 1982, Callen 2002). The type of evaluation is selected based upon the patient’s age and sex. The likelihood of malignancy increases with age and the sites vary depending on the patient’s age. Malignancy evaluation is repeated with new symptoms or annually for the first three-years following diagnosis. The over-representation of cancer in these patients seemingly ap- proaches normal levels after three-years (Hill et al. 2001), thus, age-specific malignancy screening, along with evaluation of any abnormal symptoms or findings, are recommended for following patients more than three years af- ter the initial diagnosis.
Course and Therapy
Several general measures are helpful in treating patients with DM. Bedrest
is often valuable in the individual with progressive weakness; however, this
must be combined with a range-of-motion exercise program to prevent con-
tractures. Patients who have evidence of dysphagia should have the head of
their bed elevated and should avoid eating meals immediately before retiring.
Corticosteroids and Immunosuppressive Agents
The mainstay of therapy for DM is the use of systemic corticosteroids. Tradi- tionally, prednisone is given at a dose of 0.5 to 1 mg/kg daily as initial ther- apy. The treatment should continue for at least one month after the myositis has become clinically and enzymatically inactive. At this point, the dose is slowly tapered, generally over a period lasting one and a half to two times as long as the period of active treatment. Approximately 25% of patients with dermatomyositis will not respond to systemic corticosteroids, another 25–50%
will develop significant steroid-related side effects. Therefore, early inter- vention with steroid-sparing agents primarily immunosuppressive agents, such as methotrexate, azathioprine, cyclophosphamide, mycophenolate mofetil, chlo- rambucil, or cyclosporin may be an effective means of inducing or maintain- ing a remission (Sinoway and Callen 1993; Villalba and Adams 1996; Gelber et al. 2000; Vencovsky et al. 2000). Roughly one-half to three fourths of pa- tients treated with an immunosuppressive agent will respond with an in- crease in strength, a decrease in enzyme levels, or a reduction in corticosteroid dosage. However, there few double-blind, placebo-controlled studies that demonstrate the effectiveness of any of these agents.
Additional Therapeutic Options
Patients who fail to respond to these immunosuppressives may respond to pulse methylprednisolone therapy (Callen et al. 1994; Sawhney et al. 2000;
Al-Mayouf et al. 2000a) combination immunosuppressive therapy (Villalba et al. 1998), etanercept (Saadeh 2000), infliximab (Hengstman et al. 2000) or total body irradiation. Early enthusiasm for plasmapheresis was followed by a placebo-controlled study that failed to demonstrate effectiveness. (Miller et al. 1992). However, in a double-blind, placebo-controlled study, Dalakas and co-workers (1993) demonstrated the benefits of high dose intravenous immune globulin for recalcitrant DM. Further open-label studies have dem- onstrated similar results (Cherin et al 2002).
Therapy of cutaneous disease in patients with DM is often difficult be-
cause even though the myositis may respond to treatment with corticosteroids
and/or immunosuppressive drugs, the cutaneous lesions often persist. Al-
though cutaneous disease may be of minor importance in patients with seri-
ous fulminant myositis, in many patients, cutaneous disease becomes the
most important aspect of their disorder. Most patients with cutaneous lesions
are photosensitive; thus, the daily use of a broad-spectrum sunscreen with a
high sun protective factor is recommended. Topical therapy with an appro-
priately selected corticosteroid or a non-steroidal immunomodulators such
as tacrolimus or pimecrolimus may be useful adjunctive therapy (Hollar and
Jorizzo 2004). Hydroxychloroquine HCL in dosages of 200 mg to 400 mg per
day is effective in approximately 80% of patients treated as a steroid-sparing
agent (Woo et al. 1984). Patients who do not respond well to hydroxychloro- quine can be switched to chloroquine phosphate 250 to 500 mg per day or can have quinacrine HCL 100 mg twice daily added to the regimen. Patients on continuous antimalarial therapy should have periodic ophthalmologic ex- aminations and blood counts. It appears that patients with DM have a greater frequency of drug eruptions from antimalarials, thus patients should be warned about this possibility (Pelle and Callen 2002).
Methotrexate in doses of 15–35 mg per week has been reported to be use- ful for skin lesions of DM (Zieglschmid et al. 1995; Kasteler and Callen 1997).
These studies, however, are uncontrolled, open-label observations. The need for routine liver biopsy in the DM patient treated with methotrexate is con- troversial, but patients who are obese, diabetic, or have abnormal liver func- tion tests should probably have periodic liver biopsies.
Other immunosuppressive agents have not been systematically studied as treatment of cutaneous lesions of DM, but some anecdotes suggest that mycophenolate mofetil might be beneficial. Lastly, intravenous immune glob- ulin administered monthly can result in clearing of patients with cutaneous lesions.
Calcinosis is a complication of disease in children and adolescents. This process may be prevented by aggressive early treatment. Preliminary analy- sis of the use of intravenous methylprednisolone suggested that this therapy might lessen the frequency and severity of this process (Callen et al. 1994).
Others have suggested that immunosuppressives may similarly reduce the chance of calcinosis (Al-Mayouf et al. 2000b). Once established, calcinosis is difficult to treat. Although possible, spontaneous regression is unusual. Indi- vidual patients have been treated with low-dose warfarin or oral aluminum hydroxide, however, no studies have documented the usefulness in larger groups of patients. Recent reports of long-term administration of diltiazem are promising (Oliveri et al. 1996).
The prognosis of dermatomyositis varies greatly, depending on the series of patients studied. Factors that affect prognosis include the patient’s age, the severity of myositis, the presence of dysphagia, the presence of cardio- pulmonary involvement, the presence of an associated malignancy, and the response to corticosteroid therapy (Marie et al. 1999). It seems to be well established by retrospective reports that the use of corticosteroids and/or immu- nosuppressive therapies improves the prognosis.
Summary
DM is a condition primarily of the skin and muscles, but other systemic fea-
tures may occur. DM appears to be the predominant myopathy in children,
whereas in adults many patients without skin disease occur (polymyositis or
inclusion body myositis). The pathogenesis of the muscle disease is becoming
better understood, but the cutaneous disease mechanisms remain enigmatic.
DM in adults is associated with malignancy and thus a careful evaluation of each patient should be part of their initial and followup assessments. Patients should also be evaluated for the presence of esophageal, pulmonary and car- diac disease. Calcinosis is more frequent in children with DM, and early ag- gressive therapy may limit the chance of this complication. Corticosteroids, immunosuppressives, biologic agents and/or immune globulin are effective therapies for the myopathy of DM, whereas the skin disease is best mana- ged with sunprotection, topical corticosteroids, antimalarials, methotrexate and/or immune globulin. The prognosis is good except for patients with ma- lignancy, those with severe weakness and those with cardiopulmonary dys- function.
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