Clinical Manifestations of Dermatomyositis and Polymyositis in Adults - UpToDate PDF

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Clinical manifestations of dermatomyositis and

polymyositis in adults
Authors: Marc L Miller, MD, Ruth Ann Vleugels, MD, MPH
Section Editors: Ira N Targoff, MD, Jeremy M Shefner, MD, PhD, Jeffrey Callen, MD, FACP, FAAD
Deputy Editors: Monica Ramirez Curtis, MD, MPH, Abena O Ofori, MD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jul 2019. | This topic last updated: Jan 27, 2019.
INTRODUCTION
Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies, characterized
by the shared features of proximal skeletal muscle weakness and by evidence of muscle
inflammation [1-5]. DM, unlike PM, is associated with a variety of characteristic skin manifestations. A
form of DM termed amyopathic DM (ADM, historically termed "dermatomyositis sine myositis") is a
condition in which patients have characteristic skin findings of DM without weakness or abnormal
muscle enzymes.
The clinical and serologic features of DM and PM vary among affected individuals and populations,
depending upon immunogenetic and possibly other genetic factors [6,7]. The immune mechanisms
and anatomic focus of injury within the muscle tissue in PM and DM appear distinct. The other major
type of idiopathic inflammatory myopathy is inclusion body myositis.
The clinical and laboratory manifestations of DM and PM in adults will be reviewed here. The
diagnosis, electrophysiologic and pathologic findings on diagnostic testing, differential diagnosis, and
treatment of these diseases and of the related disorders that occur in children (known as juvenile DM
and PM); the pathogenesis of inflammatory myopathies in adults and of juvenile DM and PM; the risk
for malignancy in patients with DM and PM; and the clinical manifestations, diagnosis, and treatment
of inclusion body myositis are discussed separately.
● (See "Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults".)
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● (See "Initial treatment of dermatomyositis and polymyositis in adults" and "Treatment of recurrent
and resistant dermatomyositis and polymyositis in adults" and "Juvenile dermatomyositis and
polymyositis: Treatment, complications, and prognosis".)
● (See "Initial management of cutaneous dermatomyositis in adults" and "Management of

refractory cutaneous dermatomyositis in adults".)
● (See "Pathogenesis of inflammatory myopathies" and "Juvenile dermatomyositis and

polymyositis: Epidemiology, pathogenesis, and clinical manifestations".)
● (See "Malignancy in dermatomyositis and polymyositis".)
● (See "Clinical manifestations and diagnosis of inclusion body myositis" and "Management of
inclusion body myositis".)
EPIDEMIOLOGY
The combined incidence of dermatomyositis (DM) and polymyositis (PM) has been estimated at 2 per
100,000 annually in the general population [8]. There is a female to male predominance of about two
to one. The peak incidence in adults occurs between the ages of 40 and 50, but individuals of any
age may be affected [9,10]. Estimates of prevalence range from 5 to 22 per 100,000 [11,12]. The
estimated annual incidence of the amyopathic subset of DM in the residents of Olmstead County in
Minnesota was 0.2 per 100,000 persons in a study in which the annual incidence of DM of all types
was approximately 1 per 100,000 [13].
CLINICAL MANIFESTATIONS
Dermatomyositis (DM) and polymyositis (PM) are both multisystem disorders with a wide variety of
clinical manifestations [9,10]. Most patients exhibit proximal skeletal muscle weakness. Several
characteristic skin eruptions are typical of DM, and a subset of patients with typical cutaneous
manifestations exhibit amyopathic DM (ADM), lacking evidence of muscle disease.
Interstitial pulmonary disease, dysphagia, and polyarthritis are also common in DM and PM, along
with constitutional symptoms; Raynaud phenomenon is present in some patients. Features that
overlap with other systemic rheumatic diseases, such as systemic lupus erythematosus (SLE) and
systemic sclerosis (SSc), may also be present. The risk of malignancy may be increased, particularly
in patients with DM. Each of the major clinical features is described in the sections below.
Muscle weakness — Muscle weakness is the most common feature of DM and PM; over 90 percent
of patients with PM present with muscle weakness [9]. However, cutaneous manifestations often
precede or accompany weakness, which is found at presentation in only 50 to 60 percent of patients
with DM [9,10]. Typically mild myalgias and muscle tenderness occur in 25 to 50 percent of cases.
(See 'Skin findings' below.)
The distribution of weakness is characteristically symmetric and proximal in both PM and DM.
Affected muscles typically include the deltoids and the hip flexors. Weakness of the neck flexors is
also common. Distal muscle weakness, if present, tends to be mild and usually does not cause
significant functional impairment. Rarely, patients present with focal myositis that usually but not
always progresses to the typical generalized form over time [14]. (See "Approach to the patient with
muscle weakness".)
Patients usually report a history of the insidious or subacute development of the muscle weakness,
with gradual worsening over a period of several months before medical attention is sought. However,
an acute onset of weakness is occasionally reported. Patients may describe increasing difficulty
climbing stairs, getting up from a chair, carrying heavy groceries, or picking up their children due to
the proximal muscle involvement. They may notice joint pain and swelling, if present, and they
occasionally mistakenly ascribe weakness to the joint involvement. Pain is mild, if present, and
stiffness is not a prominent complaint. (See 'Antisynthetase syndrome' below and 'Overlap
syndromes' below.)
Muscle atrophy is generally not seen in early cases, even in patients with marked weakness, but it
may occur in severe, longstanding disease.
Skin findings — Several distinct cutaneous eruptions, which are generally evident at the time of
clinical presentation, occur in DM but not in PM [15,16]. Other skin changes may occur in patients
with PM and in patients with DM and are not specific to either disorder. Dermatologic manifestations
may be prominent but can be quite subtle in some patients.
Characteristic dermatomyositis findings — Gottron's papules and the heliotrope eruption are
the hallmark and likely pathognomonic features of DM. Gottron's sign, photodistributed erythema,
poikiloderma, nailfold changes, scalp involvement, and calcinosis cutis are also characteristic and
useful in distinguishing DM from PM.
● Gottron's papules – Gottron's papules are erythematous to violaceous papules that occur
symmetrically over the extensor (dorsal) aspects of the metacarpophalangeal (MCP) and
interphalangeal (IP) joints (picture 1A-C). In addition, these lesions may involve the skin between
the MCP and IP joints, particularly when the eruption is prominent. Gottron's papules often have
associated scale and may ulcerate. When scaling is present, the lesions may mimic psoriasis or
lichen planus.
● Gottron's sign – Definitions used for Gottron's sign have varied in the literature. We define
Gottron's sign as the presence of erythematous to violaceous macules, patches, or papules on
the extensor surfaces of joints in sites other than the hands, particularly the elbows, knees, or
ankles. By contrast, some authors have used the term Gottron's papules to refer to papules in
these areas, reserving Gottron's sign for macular or patch-like lesions (picture 2) [17].
● Heliotrope eruption – The heliotrope eruption is an erythematous to violaceous eruption on the

upper eyelids, sometimes accompanied by eyelid edema, which, at times, may be quite marked
(picture 3A-D).
● Facial erythema – Patients may have midfacial erythema that can mimic the malar erythema
seen in SLE (picture 3A, 3E). In contrast to those with SLE, patients with DM will often have
involvement of the nasolabial fold, which can be helpful in distinguishing these two
photosensitive midfacial eruptions.
● Photodistributed poikiloderma (including the shawl and V signs) – Poikiloderma refers to

skin that demonstrates both hyperpigmentation and hypopigmentation, as well as telangiectasias
and epidermal atrophy. In DM, patients may demonstrate poikiloderma in any photo-exposed
site; however, classic areas of involvement are the upper back (shawl sign) (picture 4A-B) and
the V of the neck and upper chest. The poikiloderma in DM often presents with a violaceous hue.
Early in the course of cutaneous disease, these areas may demonstrate only erythema rather
than well-developed poikiloderma (picture 5). The erythema may be macular (nonpalpable) or
papular. In rare patients, these lesions become thickened and resemble papular mucinosis. The
cutaneous eruption of DM is often associated with significant pruritus, which may assist in
distinguishing its photo-exacerbated eruption from that of lupus erythematosus (LE).
● Holster sign – Patients with DM may also have poikiloderma on the lateral aspects of the thighs,
referred to as the "Holster sign" (picture 6A-B). It is unclear why this cutaneous manifestation
occurs on this classically photo-protected site.
● Generalized erythroderma – In rare patients, erythroderma may occur, which involves

extensive cutaneous surface area, including areas that are less exposed to ultraviolet light.
● Periungual abnormalities – The capillary nail beds in DM may be erythematous and may show
vascular changes similar to those observed in other systemic rheumatic diseases (eg,
scleroderma and SLE). Abnormal capillary nail bed loops may be evident, with alternating areas
of dilatation and dropout and with periungual erythema (picture 7A-B). In addition, cuticular
overgrowth, sometimes termed "ragged cuticles," is characteristic and may be associated with
hemorrhagic infarcts within the hypertrophic area (picture 7A). The degree of cuticular
involvement is thought to reflect ongoing cutaneous disease activity, representing active
vasculopathy [18].
● Psoriasiform changes in scalp – Changes in the scalp resembling seborrheic dermatitis or

psoriasis occur in a high percentage of patients with DM (picture 8). The scalp involvement in DM
is diffuse, often associated with poikilodermatous changes and with prominent scaling. Scalp
involvement may result in severe burning, pruritus, and/or sleep disturbance. In addition, severe
pruritus may occur in patients without visible disease.
● Calcinosis cutis – The deposition of calcium within the skin, a finding known as calcinosis cutis,
occurs commonly in juvenile DM. It is infrequent in adult DM. In children, calcinosis has been
associated with a delay in treatment with glucocorticoids and/or immunosuppressive therapy.
Calcinosis cutis, which is known to be very challenging to treat, may be seen in a variety of
conditions, including SSc, particularly limited cutaneous SSc; SLE (rarely); and overlap
connective tissue disorders. It may be more common in patients with DM with the
anti-p140/anti-MJ autoantibody [19,20]. (See 'Other myositis-specific autoantibodies' below.)
Skin findings in antisynthetase syndrome — Patients with either DM or PM (classically those

with the antisynthetase syndrome) may have "mechanic's hands," which present with hyperkeratotic,
fissured skin on the palmar and lateral aspects of the fingers. Occasionally, these changes result in
irregular, dirty-appearing horizontal lines that resemble those of a manual laborer (picture 9) [21].
(See 'Antisynthetase syndrome' below.)
Skin findings in MDA-5-associated dermatomyositis — Patients with melanoma differentiation-

associated gene 5 (MDA-5) antibodies are now known to have a characteristic phenotype with classic
findings that include cutaneous ulceration involving the Gottron's papules, elbows, digital pulp, and
nailfolds; erythematous, painful palmar macules and papules; alopecia; oral ulcers; arthritis; and a
lower incidence of myositis [22]. In addition, patients with MDA-5 antibodies are at a higher risk for
interstitial lung disease (ILD), including a rapidly progressive presentation with high mortality.
Recognizing the cutaneous features are thus imperative in order to allow the clinician to closely
monitor the patient's pulmonary status, particularly in the absence of widely available autoantibody
testing [23].
Rare cutaneous findings — Rarely reported cutaneous findings in patients with DM have
included ichthyosis, panniculitis, cutaneous vasculitis, lichen planus, porcelain white atrophic scars,
vesicle and bullae formation, follicular hyperkeratosis, malakoplakia, papular mucinosis, and flagellate
erythema [15,16,24-26]. Diffuse non-pitting edema is another rare manifestation of DM, and may be a
marker of more aggressive disease [27].
Lung disease — ILD is an important complication in at least 10 percent of cases of DM and PM. In
DM, it can be observed in patients with either classic or amyopathic disease. The occurrence of ILD
may be associated with rapidly progressive pulmonary failure and death. In the immune-mediated
myopathies, ILD often occurs in the context of antisynthetase antibodies and the antisynthetase
syndrome. In addition to ILD, respiratory insufficiency in the immune-mediated myopathies may result
from diaphragmatic and chest wall muscle weakness. These issues are discussed in detail
separately. (See "Interstitial lung disease in dermatomyositis and polymyositis: Clinical manifestations
and diagnosis" and 'Myositis-specific autoantibodies' below and 'Antisynthetase syndrome' below.)
Malignancy — An increased rate of malignancy has been described, with a greater risk in patients
with DM. The spectrum of malignancies generally parallels the distribution in the general population
with a few possible exceptions. This issue is discussed separately. (See "Malignancy in
dermatomyositis and polymyositis".)
Esophageal disease — Weakness of the striated muscle of the upper one-third of the esophagus
(and/or the oropharyngeal muscles) contributes to dysphagia, nasal regurgitation, and/or aspiration
[28]. Esophageal involvement is more common in older patients and may underlie the increased
incidence of bacterial pneumonia [29].
Cardiac disease — Cardiac involvement with histologic evidence of myocarditis is well-described in

DM and PM, and subclinical manifestations are frequent, including conduction abnormalities and
arrhythmia detected by electrocardiographic studies [30,31]. Symptomatic cardiac disease, such as
congestive heart failure, is less common [32]. However, patients with DM and PM are also at
increased risk for myocardial infarction [33,34]. A large, retrospective, population-based study found a
nearly three- and fourfold increased risk of MI among 350 and 424 patients with incident DM and PM,
respectively, as compared with those without an inflammatory myopathy, after controlling for relevant
risk factors such as age, sex, glucocorticoids, and nonsteroidal antiinflammatory drugs (NSAIDs) [34].
The use of serum creatine kinase (CK), the CK-MB/total CK ratio, and cardiac troponin T in
evaluating patients for cardiac involvement is problematic in patients with inflammatory myositis, and
additional testing may be required in patients in whom a myocardial infarction is suspected clinically
[35-38]. (See 'Cardiac enzymes and troponins' below.)
Antisynthetase syndrome — Up to 30 percent of patients with DM or PM have a constellation of

clinical findings termed the "antisynthetase syndrome" [39-41]. These findings include relatively acute
disease onset, constitutional symptoms (eg, fever and weight loss), myositis, the Raynaud
phenomenon, mechanic's hands, arthritis that is generally nonerosive, and ILD [42]. Affected patients
have antibodies to aminoacyl-transfer ribonucleic acid (tRNA) synthetase enzymes; the presence of
one of these antibodies is highly specific for DM, PM, or ILD [43-45]. (See 'Myositis-specific
autoantibodies' below and "Interstitial lung disease in dermatomyositis and polymyositis: Clinical
manifestations and diagnosis" and 'Antisynthetase antibodies' below.)
This syndrome can be further characterized as follows:
● Not all patients with antisynthetase antibodies or even those classified as having the
antisynthetase syndrome have all manifestations of this syndrome. The syndrome is generally
considered present in patients with an antisynthetase antibody plus two of the following features,
which are elements of the syndrome: ILD, inflammatory myopathy, and inflammatory polyarthritis.
● This group of clinical findings or this general clinical picture is not specific for antisynthetase
antibodies. Patients with other types of autoantibodies (eg, anti-PM-Scl or anti-U1
ribonucleoprotein [RNP] antibodies) can also present with these types of features. However,
patients with antisynthetase antibodies generally have more prominent or severe myositis and
ILD, and they usually lack some of the other clinical features seen in patients with these other
autoantibodies.
● Some patients with antisynthetase antibodies have relatively little or no myositis, while ILD or
other features are more prominent. The absence of myositis is seen more often with some
antisynthetase antibodies than with others.
Amyopathic dermatomyositis — A distinct group of patients with "clinically amyopathic

dermatomyositis" (CADM), historically called "dermatomyositis sine myositis," have classic cutaneous
findings of DM without clinical evidence of muscle weakness [46-49]. There are two subsets within
this group. One subset, referred to as "hypomyopathic dermatomyositis" (HDM), has subclinical
evidence of myositis upon investigation by laboratory, electromyography, muscle biopsy, or imaging
despite a lack of clinical muscle weakness; the other subset, classified as "amyopathic
dermatomyositis" (ADM), includes patients without clinical weakness and without either laboratory or
muscle study abnormalities.
ADM is considered "provisional" after six months and "confirmed" after two years [50,51]. A definite
diagnosis of ADM is further supported by such patients having neither received systemic
immunosuppressive therapy for more than two consecutive months within the first six months of skin
disease onset nor taken drugs known to induce DM-like skin changes [51].
CADM comprises 10 to 30 percent of DM cases [13,52]. Patients with CADM also appear to be at
increased risk for internal malignancy, although this risk may be less than for patients with DM
[13,52,53]. In addition, some evidence suggests that the presence of anti-MDA-5 antibodies,
previously referred to as anti-CADM-140 autoantibodies, suggests that the patient is likely to have
amyopathic disease and characteristic cutaneous findings [22,54]. (See 'Skin findings in MDA-5-
associated dermatomyositis' above and 'Other myositis-specific autoantibodies' below.)
Overlap syndromes — DM and PM may overlap with features of other connective tissue diseases,
particularly scleroderma, SLE, and mixed connective tissue disease, as well as (less often)
rheumatoid arthritis and Sjögren's syndrome. The myopathy associated with the other connective
tissue diseases varies from clinically insignificant (with minimal muscle enzyme elevations and
minimal inflammatory changes on muscle biopsy) to typically severe DM or PM in which myopathy
dominates the clinical picture [9,10,55]. (See "Overview of the clinical manifestations of systemic
sclerosis (scleroderma) in adults" and "Overview of the clinical manifestations of systemic lupus
erythematosus in adults" and "Clinical manifestations of mixed connective tissue disease".)
LABORATORY FINDINGS
Several laboratory findings are characteristic of dermatomyositis (DM) and polymyositis (PM). These
include:
● Elevated levels of muscle enzymes [9,56]
● Autoantibodies, including antinuclear antibodies, in up to 80 percent of patients with DM and PM

[9,10,56,57]; myositis-specific autoantibodies, in at least 30 to 40 percent of patients [39,58-60];
and myositis-associated autoantibodies, especially in patients with overlap syndromes
● Elevated levels of serum and urine myoglobin [61,62]
The erythrocyte sedimentation rate (ESR) is often normal or is only mildly elevated, even in patients
with active muscle disease [5].
Muscle enzymes — Creatine kinase (CK), lactate dehydrogenase (LD), aldolase, aspartate
aminotransferase (AST), and alanine aminotransferase (ALT) are all muscle enzymes that may be
elevated in patients with inflammatory myopathy and other muscle disorders. (See "Muscle enzymes
in the evaluation of neuromuscular diseases".)
At some point in the course of the disease, almost all patients with DM and PM, except those with
amyopathic DM (ADM), have an elevation in at least one muscle enzyme; most have elevations in all
enzymes. In a review of 153 patients with DM or PM, normal results were found for CK in 5 percent,
for aldolase in 4 percent, for LD in 9 percent, and for the aminotransferases in 15 to 17 percent [9].
However, these data may underestimate the frequency of normal CK concentrations because the
Bohan and Peter criteria employed in that study included muscle enzyme elevation as a disease
criterion. Additionally, the study was performed before many autoantibody tests and magnetic
resonance imaging (MRI) studies were available to facilitate diagnosis. (See "Diagnosis and
differential diagnosis of dermatomyositis and polymyositis in adults", section on 'Classification
criteria'.)
The level of serum CK can vary widely. In untreated patients with active muscle disease, it is usually
more than 10-fold the upper limit of normal (ie, at least 2000 to 3000 international units/L). In severe
cases, the serum CK concentration may be elevated more than 50-fold or even 100-fold (ie, up to
10,000 to 20,000 international units/L), and higher levels are sometimes seen.
A correlation between the severity of the weakness and the height of elevation in serum muscle
enzymes may be seen, although the degree of muscle dysfunction may be much greater than the
enzyme levels would suggest [10]. In some cases, there may be elevations in serum muscle enzymes
without discernible muscle weakness. This is particularly observed in patients with early disease.
The occurrence of muscle weakness with relatively normal enzyme levels is more likely to occur in
DM than PM. Other patients with no clinical muscle involvement, as in ADM, will also have normal
enzyme levels. Persistently low serum muscle enzyme levels in the setting of obvious muscle
weakness may also occur in patients with advanced disease and significant loss of muscle mass.
Cardiac enzymes and troponins — Patients with myositis may have an elevated serum CK-MB
fraction in the absence of myocarditis, which is usually attributed to increased expression in
regenerating skeletal muscle affected by the inflammatory disease or, less often, to involvement of
the myocardium by the myositis. Myocardial infarction may be suspected in these patients, who may
require additional testing. (See "Troponin testing: Clinical use" and "Clinical manifestations and
diagnosis of myocarditis in adults", section on 'Cardiac biomarkers'.)
Measurement of cardiac troponin I may be helpful in patients in whom it may be difficult clinically to
determine whether elevations in CK or other muscle enzymes are due to cardiac rather than skeletal
muscle disease. Increased levels of cardiac troponin I appear relatively specific for myocardial injury,
unlike elevations of total CK, CK-MB, and other muscle enzymes or of cardiac troponin T, all of which
may be seen both in skeletal muscle inflammatory myopathy and in cardiac disease [5,36-38,56].
(See 'Cardiac disease' above and "Troponin testing: Clinical use".)
Autoantibodies — Antinuclear antibodies detected by standard immunofluorescence methods may

be present in up to 80 percent of patients with DM or PM [39,57,63]. Testing for specific
autoantibodies may demonstrate either of the following:
● Myositis-specific autoantibodies, which are detected primarily in patients with inflammatory

myositis and which may offer information regarding prognosis and potential patterns of organ
involvement
● Myositis-associated autoantibodies, which are found with other autoimmune rheumatic diseases
that may be associated with myositis
Myositis-specific autoantibodies — Several categories of autoantibodies found primarily in

patients with myositis and directed against cytoplasmic proteins, ribonucleoproteins, and certain
nuclear antigens are termed myositis-specific autoantibodies [39,43,45,58,64]. These autoantibodies
occur in approximately 30 percent of patients with DM and PM.
Particular myositis-specific autoantibodies or classes of myositis–specific autoantibodies are

associated with particular clinical syndromes within the myositis spectrum and may be associated
with certain histopathologic findings [45,65-69]. It is not known whether such antibodies have a direct
role in disease pathogenesis, and this question remains an area of strong interest to investigators.
(See "Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults", section on
'MSA and histopathology' and "Pathogenesis of inflammatory myopathies".)
There are several categories of myositis-specific autoantibodies, including:
● Antibodies to aminoacyl-transfer (t)RNA synthetases (antisynthetase antibodies), including anti-

Jo-1
● Antibodies to signal recognition particle (SRP)
● Antibodies to Mi-2, a nuclear helicase
Other types of myositis-specific autoantibodies that do not fall into one of these categories have also
been described, as discussed below [69].
Antisynthetase antibodies — Anti-Jo-1 antibodies are the most common myositis-specific

autoantibody and the most frequently observed antisynthetase antibody. Anti-Jo-1 is seen in about 20
percent of patients with idiopathic inflammatory myopathy. Anti-Jo-1 antibodies are directed against
histidyl–tRNA synthetase, one of a group of enzymes that catalyze the attachment of specific amino
acids to their cognate tRNAs during the process of protein synthesis. These antibodies are strongly
associated with several clinical findings, including interstitial lung disease (ILD), the Raynaud
phenomenon, arthritis, and mechanic's hands (picture 9) [39,64]. (See 'Antisynthetase syndrome'
above and 'Skin findings in antisynthetase syndrome' above.)
In one study at an academic medical center, inflammatory myositis, often with the antisynthetase
syndrome, was present in 94 percent of 81 patients with anti-Jo-1 antibodies in whom testing had
been performed for suspected autoimmune disease [70]. DM or PM was present in 88 percent, and
an undifferentiated connective tissue disease or overlap syndrome was present in 12 percent of
patients, several of whom had systemic sclerosis (SSc). Other findings in the patients positive for Jo-
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1 included ILD (69 percent), arthritis (57 percent), the Raynaud phenomenon (53 percent),
mechanic's hands (17 percent), and sclerodactyly (12 percent).
Anti-Jo-1 and other antisynthetase antibodies, such as anti-PL-12, have been observed in some
patients with ILD who lack evidence of myositis [45]. Other antisynthetase antibodies include
antibodies to the OJ, EJ, PL-7, PL-12, KS, Zo, and Ha antigens [44,71,72]. In the aggregate, these
are found in 1 to 5 percent of idiopathic inflammatory myopathy patients. Patients with these other
antisynthetase antibodies may have clinical manifestations similar to those with anti-Jo-1-antibodies,
but differences have sometimes been noted, particularly in the frequency of myositis [45].
Anti-SRP antibodies — The signal recognition particle (SRP) is involved in the translocation of
newly synthesized proteins into the endoplasmic reticulum. Anti-SRP antibodies have been found in
about 5 percent of patients with inflammatory myopathy and were described almost exclusively in
patients diagnosed with PM [65,73]. Unlike other patients with PM, however, the muscle biopsies in
these patients typically show muscle fiber necrosis and endomysial fibrosis but show little or no
inflammatory cell infiltrate [65,74]. In one study of patients with such disease, termed necrotizing
myopathy, anti-SRP antibodies were present in 6 of 38 patients (16 percent) [75].
Anti-SRP antibodies are associated with severe myopathy and aggressive disease that may be
difficult to control, even with high-dose glucocorticoids and immunosuppressive agents. These
antibodies are not entirely specific for necrotizing myopathy or polymyositis, however, as they have
also been described in two patients each with limb girdle muscular dystrophy and SSc, in a patient
with the antisynthetase syndrome without myopathy, and in several patients with DM [5,74,76]. (See
"Initial treatment of dermatomyositis and polymyositis in adults".)
Anti-Mi-2 antibodies — Anti-Mi-2 antibodies are directed against a helicase involved in

transcriptional activation [77]. Among patients with DM, anti-Mi-2 antibodies are present in about 7
percent of Caucasians and in about 30 percent of those from Central America [6]. They are
associated with the relatively acute onset of DM, are traditionally associated with a classic shawl or V
sign, and may respond well to therapy [39]. (See 'Characteristic dermatomyositis findings' above.)
Other myositis-specific autoantibodies — A number of other myositis-specific autoantibodies

have been described in different populations of patients with myositis. The clinical usefulness of these
autoantibodies in patients suspected of having DM or PM is not well-established:
● Antibodies directed against hPMS-1, a deoxyribonucleic acid (DNA) mismatch repair enzyme,
are myositis-specific autoantibodies reported to occur in 7.5 percent of patients with myositis
[78].
● Antibodies that have high specificity for ADM were first reported in Japanese patients [79-81].
These antibodies recognize a protein involved in innate immune responses, called clinically ADM
(CADM)-140, a 140kD polypeptide. This protein is the RNA helicase encoded by the melanoma
differentiation-associated gene 5 (MDA-5), and is now most often referred to as the anti-MDA-5
antibody [80,81]. In reports from two different groups in Japan, the presence of anti-MDA-5
antibodies was also strongly associated with the development of rapidly progressive ILD [79-81].
Similarly, in another cohort of 64 Chinese patients with DM or PM, anti-MDA-5 antibodies were
strongly associated with rapidly progressive ILD. These patients, however, when compared with
Japanese cohorts in a meta-analysis, demonstrated a significantly lower frequency of CADM
[82]. (See 'Amyopathic dermatomyositis' above.)
Anti-MDA-5 has also been described in a small cohort of patients in the United States with DM
but little or no myositis, with increased risk of ILD, and with vasculopathy affecting the skin [22].
In addition to the typical cutaneous signs of dermatomyositis, anti-MDA-5 patients
characteristically develop cutaneous ulceration involving Gottron's papules and Gottron's sign,
digital pulp, and nailfolds, as well as erythematous, painful palmar papules and macules,
alopecia, and oral ulcers. These patients also frequently have arthritis and amyopathic disease.
Anti-MDA-5 antibodies are strongly associated with ILD with a rapidly progressive course and
poor overall survival related to pulmonary complications [83]. In a large case-control study
investigating the rates of mortality among DM patients with various myositis-associated
antibodies, those with anti-MDA-5 antibodies displayed the lowest survival rates, even when
compared with individuals with malignancy-associated DM [84]. Further study in other
populations will help to clarify the diagnostic and prognostic utility of this antibody. Evidence
suggests that patients with MDA-5 antibodies should be monitored closely for lung involvement.
(See 'Skin findings in MDA-5-associated dermatomyositis' above.)
● Anti-p140, also termed anti-MJ, is an antibody against a 140-kD protein that is distinct from the
anti-CADM-140 antibodies. This antibody has been studied primarily in patients with juvenile DM,
in whom it is associated with calcinosis [19]. This association has also been described in adults
with DM [20]. The antibody is directed against nuclear matrix protein 2 (NXP2), which has a role
in transcriptional regulation. It has also been found in some adults with DM and ILD [72].
● Antibodies to a 155-kD protein were found in sera of 51 of 244 patients with myositis (21
percent); most of the positive patients had a form of DM. Antibodies were present in only 1 of 108
patients with other connective tissue diseases and in no normal controls [85]. Caucasian patients
with this autoantibody had a unique human leukocyte antigen (HLA) risk factor, HLA-DQA1*0301,
and had an increased frequency of the V sign rash. Patients with this autoantibody were clinically
distinct from those with autoantibodies to aminoacyl-transfer RNA synthetases.
This myositis-specific autoantibody, known as the anti-p155/140 antibody, has reactivity with the
155-kD nuclear protein transcriptional intermediary factor (TIF)-1gamma and often also with the
140-kD TIF-1alpha [72,85,86]. Reaction with TIF-1beta may also occur [86,87]. It has also been
described in Japanese patients [88]. The anti-155/140 antibody was detected in 7 of 52 patients
(13 percent) with DM but in none of the disease controls with PM, systemic lupus erythematosus
(SLE), SSc, or idiopathic interstitial pneumonia. Clinical associations of the anti-155/140 antibody
include strong associations with adult and juvenile DM, rather than PM, and with cancer-
associated myositis. Flagellate erythema and the conventional cutaneous DM findings of
Gottron's papules and a heliotrope eruption may be present [88,89]. The anti-p155/140 antibody
is associated with an increased risk for cancer in patients with DM, with a sensitivity approaching
70 percent and specificity approaching 90 percent [90]. (See "Malignancy in dermatomyositis and
polymyositis".)
● Myositis-specific autoantibodies that target the small ubiquitin-like modifier activating enzyme
(SAE) were found in 8 percent of 266 patients with adult DM but were not found in 250 patients
with other connective tissue diseases or in 50 healthy controls [91]. Most of the patients with anti-
SAE antibodies presented with skin disease, including heliotrope eruptions and Gottron's lesions,
before progressing to myositis with systemic manifestations, including dysphagia. The
autoantibody was strongly associated with the HLA-DQB1*03, HLA-DRB1*04, and HLA-
DQA1*03 haplotypes.
Autoantibodies and overlap syndromes — The detection of anti-Ro, anti-La, anti-Sm, or anti-
ribonucleoprotein (RNP) antibodies in a patient with myositis suggests a diagnosis of myositis
associated or overlapping with another systemic rheumatic disease [57]. Anti-Ro52 antibodies are
common in patients with antisynthetase antibodies, and anti-Ro60 and anti-La may be seen in a
smaller number of such patients and in those with other myositis-specific antibodies. (See "The anti-
Ro/SSA and anti-La/SSB antigen-antibody systems" and "Antibodies to double-stranded (ds)DNA,
Sm, and U1 RNP".)
The presence of anti-Ro52 without anti-Ro60 is more common in myositis than in other conditions
and is more common in patients with the antisynthetase syndrome than in others with myositis. In
general, anti-Ro, anti-La, and anti-U1 RNP can be seen in some patients who also have myositis-
specific autoantibodies, but myositis-specific autoantibodies tend to be mutually exclusive with each
other. High titers of anti-RNP antibodies are associated with mixed connective tissue disease, the
overlap syndrome of myositis with features of scleroderma and SLE [92]. (See "The anti-Ro/SSA and
anti-La/SSB antigen-antibody systems", section on 'Anti-Ro60 versus anti-Ro52 antibodies' and
"Definition and diagnosis of mixed connective tissue disease".)
Anti-PM-Scl and anti-Ku antibodies have been identified in patients with overlapping features of
myositis and scleroderma [59,66]. Many patients with these antibodies, however, do not have
myositis. (See "Diagnosis and differential diagnosis of systemic sclerosis (scleroderma) in adults",
section on 'Laboratory testing'.)
The precise diagnosis of an underlying connective tissue disease is critical to patient management
because of the prognostic and treatment implications of specific diagnoses. (See "Antibodies to
double-stranded (ds)DNA, Sm, and U1 RNP" and "The anti-Ro/SSA and anti-La/SSB antigen-
antibody systems".)
HISTOPATHOLOGY
Dermatomyositis (DM) and polymyositis (PM) can be distinguished from each other and from other
forms of myopathy by their histopathologic findings. In patients with DM, characteristic findings may
also be seen on skin biopsy, although these findings are very similar on light microscopy to changes
that can be seen in systemic lupus erythematosus (SLE). The histologic features of DM and PM are
described in detail separately. (See "Diagnosis and differential diagnosis of dermatomyositis and
polymyositis in adults", section on 'Muscle histopathology' and "Diagnosis and differential diagnosis of
dermatomyositis and polymyositis in adults", section on 'Skin histopathology'.)
ELECTROMYOGRAPHY
Characteristic electrophysiologic abnormalities on electromyography (EMG) are often seen in

inflammatory myopathy. However, such changes are not specific for the diagnoses of
dermatomyositis (DM) or polymyositis (PM), and the EMG is normal in approximately 10 percent of
patients. Similar findings may occur in various infectious, toxic, or metabolic myopathies. The EMG
abnormalities seen in DM and PM are described in detail separately. (See "Diagnosis and differential
diagnosis of dermatomyositis and polymyositis in adults", section on 'EMG findings'.)
MAGNETIC RESONANCE IMAGING
Magnetic resonance imaging (MRI) of skeletal muscles is a noninvasive sensitive but nonspecific
modality for detecting areas of muscle inflammation and edema with active myositis, fibrosis, and
calcification. (image 1) [93]. Findings on MRI in dermatomyositis (DM) and polymyositis (PM) are
described separately. (See "Diagnosis and differential diagnosis of dermatomyositis and polymyositis
in adults", section on 'MRI'.)
SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions around
the world are provided separately. (See "Society guideline links: Dermatomyositis and polymyositis".)
INFORMATION FOR PATIENTS
UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The
Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and
they answer the four or five key questions a patient might have about a given condition. These
articles are best for patients who want a general overview and who prefer short, easy-to-read
materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more
detailed. These articles are written at the 10th to 12th grade reading level and are best for patients
who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-
mail these topics to your patients. (You can also locate patient education articles on a variety of
subjects by searching on "patient info" and the keyword(s) of interest.)
● Basics topics (see "Patient education: Dermatomyositis (The Basics)" and "Patient education:
Polymyositis (The Basics)")
● Beyond the Basics topics (see "Patient education: Polymyositis, dermatomyositis, and other
forms of idiopathic inflammatory myopathy (Beyond the Basics)")
SUMMARY
● Dermatomyositis (DM) and polymyositis (PM) are both multisystem disorders with a wide variety
of clinical manifestations. Most patients exhibit proximal skeletal muscle weakness and evidence
of muscle inflammation, but the immune mechanisms and the anatomic focus of injury within the
muscle tissue in DM and PM appear distinct. The muscle weakness usually develops in an
insidious or subacute fashion, with gradual worsening over a period of several months. Muscle
atrophy may be present in severe, longstanding disease. Oropharyngeal and upper esophageal
muscle involvement may lead to dysphagia, nasal regurgitation, or aspiration. Respiratory failure
may result from weakness of the diaphragm and chest wall muscles. (See 'Clinical
manifestations' above and 'Muscle weakness' above and 'Lung disease' above and 'Esophageal
disease' above.)
● Several characteristic skin eruptions are typical of DM, including Gottron's papules and the
heliotrope eruption (picture 1A, 3A); these changes are considered pathognomonic for DM.
Photodistributed erythema and poikiloderma, as well as nailfold changes, are also characteristic
and useful in distinguishing DM from PM. A form of DM, termed amyopathic DM (ADM,
historically known as "dermatomyositis sine myositis"), is a condition in which patients have
characteristic skin findings of DM without weakness, abnormal muscle enzymes, or other
abnormal muscle studies. (See 'Clinical manifestations' above and 'Skin findings' above and
'Amyopathic dermatomyositis' above.)
● Interstitial lung disease (ILD) may occur with DM (including ADM), PM, and overlap myositis.
Dysphagia and polyarthritis are also common in DM and PM, along with constitutional symptoms;
the Raynaud phenomenon is present in some patients. Features that overlap with other systemic
rheumatic diseases, such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc),
may also be present. The risk of malignancy may be increased, particularly in patients with DM.
(See 'Clinical manifestations' above and 'Lung disease' above and 'Esophageal disease' above
and 'Antisynthetase syndrome' above and 'Overlap syndromes' above and 'Malignancy' above.)
● Elevations in serum creatine kinase (CK), lactate dehydrogenase, aldolase, and

aminotransferases occur in most patients. Muscle enzyme levels are useful in making the
diagnosis and in following disease activity. Although CK-MB elevation is not unusual, a common
source is skeletal muscle, and symptomatic cardiac muscle involvement is uncommon. (See
'Muscle enzymes' above and 'Cardiac disease' above.)
● Autoantibodies are found in a majority of patients. Among the myositis-specific autoantibodies,

antibodies against aminoacyl transfer ribonucleic acid (tRNA) synthetases, particularly anti-
histidyl-tRNA synthetase antibodies (anti-Jo-1), have been associated with ILD, the Raynaud
phenomenon, arthritis, and mechanic's hands, known collectively as the antisynthetase
syndrome. Additional myositis-specific autoantibodies include other antisynthetase antibodies,
anti-signal recognition particle antibodies, anti-Mi-2 antibodies, and others. (See 'Autoantibodies'
above and 'Antisynthetase syndrome' above.)
Use of UpToDate is subject to the Subscription and License Agreement.
Topic 5159 Version 18.0
GRAPHICS
Gottron's papules in dermatomyositis
Multiple violaceous, scaly papules are present overlying the joints on the dorsal
hand.
Reproduced with permission from: www.visualdx.com. Copyright VisualDx. All rights

reserved.
Graphic 63383 Version 5.0
Multiple erythematous to violaceous papules on the metacarpophalangeal joints and the

interphalangeal joints in a patient with dermatomyositis. The eruption extends onto the skin between
the joints.
Courtesy of Ruth Ann Vleugels, MD, MPH.
Erythematous to violaceous papules over the extensor joints of the hand in a patient with
dermatomyositis.
Reproduced with permission from: www.visualdx.com. Copyright VisualDx. All rights reserved.
Gottron's sign in dermatomyositis
Erythematous to violaceous patches with overlying scale are present on the extensor
surfaces of both knees in this child with dermatomyositis.
Copyright © 2017 American College of Rheumatology. Used with permission.
Heliotrope eruption in dermatomyositis
Violaceous erythema on the upper lids in a patient with dermatomyositis. Mid-facial erythema that
does not spare the nasolabial folds is also present.
Courtesy of Jeffrey Callen, MD, FACP, FAAD.
Heliotrope euption in dermatomyositis
A reddish-purple eruption on the upper eyelid (the heliotrope eruption),

accompanied by swelling of the eyelid in a patient with dermatomyositis (DM).
This is the most specific cutaneous eruption in DM, although it is only present in
a minority of patients.
Courtesy of John H Stone, MD, MPH.
Heliotrope eruption in dermatomyositis
Courtesy of John H Stone, MD, MPH.
Eyelid edema in dermatomyositis
Edematous upper and lower eyelids in a patient with dermatomyositis.
Acute cutaneous lupus erythematosus
An erythematous, edematous eruption is present on the malar area. Note the sparing of
the nasolabial folds.
Shawl sign in dermatomyositis
Poikilodermatous eruption on the upper back in a patient wtih dermatomyositis.
Shawl sign in dermatomyositis
Poikilodermatous patches are present on the upper back of this patient with
dermatomyositis.

reserved.
Dermatomyositis
Confluent areas of violaceous erythema are present on the neck, chest, and
upper arms of this patient with dermatomyositis.

reserved.
Holster sign
Courtesy of Jeffrey P Callen, MD.
Holster sign in dermatomyositis
Poikilodermatous changes on the lateral aspect of the thigh.
Nailfold changes in dermatomyositis
Dilated capillary loops at the proximal nailfold, cuticular overgrowth, and periungual erythema.
Nail changes in dermatomyositis
Dilated capillary loops at the proximal nailfold visualized with dermoscopy.
Dermatomyositis
Psoriasiform changes on the scalp in dermatomyositis.
Mechanic's hands in a patient with polymyositis, and the

anti-synthetase syndrome
Courtesy of John H. Stone, MD, MPH.
56-year-old man with suspected polymyositis
Whole-body turbo short tau inversion recovery MRI (TR/TE, 4000/30; inversion
time, 160 msec) of posterior aspect of upper torso shows extensive
inflammation in deltoid, trapezius, triceps, and latissimus dorsi muscles
bilaterally (straight arrows) and further muscle edema and inflammation in
vastus medialis and lateralis muscles of left thigh (curved arrow). Note
susceptibility artifact at site of right hip prosthesis, limiting assessment of
muscles adjacent to this site.
MRI: magnetic resonance imaging.
O'Connell MJ, Powell T, Brennan D, et al. Whole-body MR imaging in the dianosis of

polymyositis. Am J Roentgenol 2002; 179:967. Reprinted with permission from the
American Journal of Roentgenology. Copyright © 2002 American Roentgen Ray
Society.

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14/8/2019 Clinical manifestations of dermatomyositis and polymyositis in adults - UpToDate

Official reprint from UpToDate®

Clinical manifestations of dermatomyositis and

● (See "Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults".)

● (See "Initial management of cutaneous dermatomyositis in adults" and "Management of

● (See "Pathogenesis of inflammatory myopathies" and "Juvenile dermatomyositis and

● (See "Malignancy in dermatomyositis and polymyositis".)

● Heliotrope eruption – The heliotrope eruption is an erythematous to violaceous eruption on the

● Photodistributed poikiloderma (including the shawl and V signs) – Poikiloderma refers to

● Generalized erythroderma – In rare patients, erythroderma may occur, which involves

● Psoriasiform changes in scalp – Changes in the scalp resembling seborrheic dermatitis or

Skin findings in antisynthetase syndrome — Patients with either DM or PM (classically those

Skin findings in MDA-5-associated dermatomyositis — Patients with melanoma differentiation-

Cardiac disease — Cardiac involvement with histologic evidence of myocarditis is well-described in

Antisynthetase syndrome — Up to 30 percent of patients with DM or PM have a constellation of

This syndrome can be further characterized as follows:

Amyopathic dermatomyositis — A distinct group of patients with "clinically amyopathic

● Elevated levels of muscle enzymes [9,56]

● Autoantibodies, including antinuclear antibodies, in up to 80 percent of patients with DM and PM

● Elevated levels of serum and urine myoglobin [61,62]

Autoantibodies — Antinuclear antibodies detected by standard immunofluorescence methods may

● Myositis-specific autoantibodies, which are detected primarily in patients with inflammatory

Myositis-specific autoantibodies — Several categories of autoantibodies found primarily in

Particular myositis-specific autoantibodies or classes of myositis–specific autoantibodies are

There are several categories of myositis-specific autoantibodies, including:

● Antibodies to aminoacyl-transfer (t)RNA synthetases (antisynthetase antibodies), including anti-

Antisynthetase antibodies — Anti-Jo-1 antibodies are the most common myositis-specific

Anti-Mi-2 antibodies — Anti-Mi-2 antibodies are directed against a helicase involved in

Other myositis-specific autoantibodies — A number of other myositis-specific autoantibodies

Characteristic electrophysiologic abnormalities on electromyography (EMG) are often seen in

MAGNETIC RESONANCE IMAGING

SOCIETY GUIDELINE LINKS

INFORMATION FOR PATIENTS

● Elevations in serum creatine kinase (CK), lactate dehydrogenase, aldolase, and

● Autoantibodies are found in a majority of patients. Among the myositis-specific autoantibodies,

Use of UpToDate is subject to the Subscription and License Agreement.

Topic 5159 Version 18.0

Gottron's papules in dermatomyositis

Reproduced with permission from: www.visualdx.com. Copyright VisualDx. All rights

Graphic 63383 Version 5.0

Gottron's papules in dermatomyositis

Multiple erythematous to violaceous papules on the metacarpophalangeal joints and the

Courtesy of Ruth Ann Vleugels, MD, MPH.

Graphic 89229 Version 1.0

Gottron's papules in dermatomyositis

Graphic 89230 Version 2.0

Gottron's sign in dermatomyositis

Copyright © 2017 American College of Rheumatology. Used with permission.

Graphic 50909 Version 15.0

Heliotrope eruption in dermatomyositis

Courtesy of Jeffrey Callen, MD, FACP, FAAD.

Graphic 89101 Version 2.0

Heliotrope euption in dermatomyositis

A reddish-purple eruption on the upper eyelid (the heliotrope eruption),

Courtesy of John H Stone, MD, MPH.

Graphic 73090 Version 4.0

Heliotrope eruption in dermatomyositis

Courtesy of John H Stone, MD, MPH.