JEADV

DOI: 10.1111/jdv.13516

ORIGINAL ARTICLE

Dermatomyositis: factors predicting relapse

V. Vuong,1 T.A. Duong,1 J. Aouizerate,2,3 F.J. Authier,2,3 S. Ingen-Housz-Oro,1,4 L. Valeyrie-Allanore,1,4
N. Ortonne,2 P. Wolkenstein,1,4, R.K. Gherardi,2,3, O. Chosidow,1,4,5, A. Cosnes,1, E. Sbidian1,4,5,*
^pitaux universitaires Henri Mondor, Cre
teil, France
AP-HP, DHU-VIC, Service de Dermatologie, Ho
partement de Pathologie, Centre de refe
rence des pathologies neuromusculaires, Ho
^pitaux universitaires Henri Mondor,
AP-HP, De
teil, France
Cre
3
 Paris Est, IMRB, INSERM U955-Unite
 10, Cre
teil, France
Universite
4
 Paris Est, IMRB, EA 7379 EpiDermE, Cre
teil, France
Universite
5
 Paris Est, INSERM, CIC 1430, Cre
teil, France
Universite
*Correspondence: E. Sbidian. E-mail: emilie.sbidian@hmn.aphp.fr
1
2

Abstract
Background The course of dermatomyositis (DM) can be chronic with relapses, which are associated with major
morbidity.
Objective The aim of this study was to identify presentation features that predict DM relapses.
Methods We retrospectively reviewed data of patients with DM recorded from 1990 to 2011, including muscle biopsy
results. Characteristics of patients with and without relapses were compared. Hazard ratios (HRs) were estimated using
a Cox model.
Results We identied 34 patients, with a mean age of 46  17 years (range, 1877) and 24 (71%) women. The muscle
and skin abnormalities relapsed in 21 (61%) patients. By univariate analysis, two presentation features were signicantly
associated with a subsequently relapsing course, namely, dysphonia [HR = 3.2 (1.28.5)] and greater skin lesion severity
dened as a Cutaneous Disease Area Severity Index [CDASI] > 20 [HR = 3.5 (1.27.9)].
Conclusion Dysphonia and skin lesion severity at disease onset must be recorded, as they signicantly predict a
relapsing disease course.
Received: 8 June 2015; Accepted: 2 October 2015

Conicts of interest
None.

Funding source
None.

Introduction
Dermatomyositis (DM) is a rare idiopathic inflammatory
myopathy.1 The first diagnostic criteria, developed by Bohan
and Peter in 1975,2,3 were a specific skin rash (heliotrope rash,
Gottrons sign, and Gottrons papules), symmetrical muscle
weakness of the proximal limbs, muscle enzyme elevation, characteristic electromyogram (EMG) abnormalities and specific
muscle biopsy alterations. The diagnosis of DM is definite with
four criteria, probable with three and possible with two including the specific skin rash.
The course of DM may follow any of three patterns,
monophasic, chronic or polyphasic. In monophasic disease,
patients experience a single flare and then have no symptoms for

Equal contributors.

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at least 24 months after the diagnosis. Chronic DM is defined as

persistent clinical and/or laboratory abnormalities that require
continuous treatment for at least 24 months after the diagnosis.
Polyphasic DM runs an undulating course with relapses separated by remissions.4
No consensus exists regarding the definition of a DM
relapse.47 In practice, a relapse is diagnosed when the symptoms recur or worsen after first improving under first-line treatment. Several factors are known to predict a relapsing course or
poor outcomes including death, refractoriness to treatment and
disease progression: they consist of older age, a long time from
symptom onset to treatment, cardiac or pulmonary involvement, underlying neoplasia, dysphonia and dysphagia.4,7,8 However, these predictors were identified in studies of heterogeneous
populations including patients with paraneoplastic DM or

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Vuong et al.

814

polymyositis, juvenile dermatomyositis and overlap syndromes.

Moreover, the studies focussed on predictors not only of relapse,
but also of other outcomes such as death or refractoriness to
treatment.
The aim of this study was to identify features at presentation that predict a relapsing course of non-paraneoplastic
DM.

Histological and immunohistochemical analysis

Patients and methods

Outsourced muscle biopsy specimens were retrieved. All muscle

biopsies were reviewed by three pathologists (JA, FJA and RKG).
Unfixed 7-lm cryosections were routinely stained with hematein-eosin (HE) and with Gomori or Masson trichrome.
Immunostaining was achieved using a Bond-III automated stainer to detect major histocompatibility complex (MHC) class-I
antigens, membrane attack complex (MAC/C5b-9), regenerating
myofibres, clone CD564, macrophages and T lymphocytes.

Study design

Histological scoring To quantify the histological lesions, we

In this single-centre retrospective study, we identified patients

with DM referred to the dermatology department of the Henri
Mondor university hospital, Creteil, France, between 1990 and
2011. To this end, we crossed the dermatology department database and the National French Hospital Database (PMSI), which
we queried using the following International Classification of
Disease-10th diagnostic codes: paraneoplastic DM; juvenile DM
(JDM); other DM; other dermatopolymyositis; and polymyositis. The data thus obtained were pooled, and the list of patients
thus obtained was checked to eliminate duplicates and to resolve
discrepancies.

retrospectively used the validated scoring tool developed by the

International JDM Biopsy Consensus group11,12 and based on
four domains: inflammatory, vascular, muscle fibre and connective tissue. In each domain, well-defined elementary changes are
scored. In addition, a visual analogue scale (VAS) is used to
assess overall severity.

Patients

We reviewed the medical records of all the patients on the list.

Inclusion criteria were a diagnosis of DM meeting Bohan and
Peter criteria2,3 and systemic corticosteroid treatment. We did
not include patients with paraneoplastic DM, defined as a diagnosis of cancer within 2 years of the diagnosis of DM,9 since the
course of this form of DM is usually closely dependent on the
effectiveness of the anti-cancer treatment. The other noninclusion criteria were DM not requiring corticosteroid therapy
(usually amyopathic or hypomyopathic DM), overlap syndrome
and JDM defined as age younger than 18 years at the diagnosis
of DM.
Data collection

We abstracted the following data from the medical records:

age, sex, comorbidities, initial clinical features and severe complications related to DM. We retrospectively graded skin
lesion severity using the Cutaneous Disease Area Severity
Index (CDASI).10 The CDASI can range from 0 to 132. It is
based on the location of the lesions, intensity of the rash,
presence of periungual manifestations and presence of Gottrons papules. We recorded the results of the following investigations: creatine kinase (CK) level, antinuclear antibodies,
EMG and thoracic computed tomography (CT) if available.
Finally, we collected data on the treatments (initial dosage
and mean duration of corticosteroid therapy; and second-line
treatments) and follow-up (relapse, time to relapse, complications, and death).

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Patient classication: identication of relapsers

We classified the patients into two groups based on whether they

experienced at least one relapse. A relapse was defined as recurrence of muscle or cutaneous manifestations after an improvement, leading to at least one of the following treatment changes:
50% increase in the corticosteroid dosage, addition of an
immunosuppressant or switch to a different immunosuppressant and addition of intravenous immunoglobulins (IVIg). Prescription of an immunosuppressant or IVIg only to achieve a
corticosteroid-sparing effect was not classified as a relapse.
Statistical analysis

Data were analysed using STATA software version 11 (Stata Inc.,

College Station, TX, USA). All tests were two-tailed and P values
not greater than 0.05 were considered significant, except when
multiple testing was performed (histological scores, four items),
in which case P values not greater than 0.012 were considered
significant. The characteristics of the population were described.
Quantitative variables were either reported as median  standard deviation (SD) or converted to categorical variables. Qualitative variables were reported as number and percentage.
Event-free survival, defined as the time from the diagnosis to
the first relapse or last follow-up visit, was estimated using the
KaplanMeier method. To look for factors associated with
event-free survival, we compared survival curves using the logrank test. A Cox model was built to compute hazard ratios
(HRs) with their 95% confidence intervals (95% CIs).

Results
The initial screen of the two databases identified 130 patients
(Fig. 1). Among them, 52 (40%) did not have DM. Of the 78
patients with DM, only 34 (43.6%) fulfilled our patient selection
criteria.

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Dermatomyositis: factors predicting relapse

815

Table 1 Initial characteristics of the study population (n = 34)

Patients, n = 130
Other diagnosis, n = 52
Patients, n = 78
Paraneoplastic DM, n = 27
Patients, n = 51
JDM, n = 3
Patients, n = 48
DM without systemic corticosteroid,
n = 14

Patients, n = 34
Figure 1 Flow chart diagram.

Initial characteristics

Total

Relapse
(N = 21)

No relapse
(N = 13)

Men

6 (46)

10 (29)

4 (19)

Age, years [mean (SD)]

46 (17)

48 (19)

46 (15)

Time to diagnosis, months

[mean (SD)]

2 (2)

2 (10)

2 (15)

Bohan and Peter criteria, n (%)

Skin rash

34 (100)

21 (100)

13 (100)

Proximal and symmetrical

weakness

32 (94)

20 (95)

12 (92)

CK elevation

29 (85)

17 (81)

12 (92)

Electromyogram abnormalities
(NA, n = 3)

24 (71)

14 (67)

10 (77)

Specic muscle biopsy

(NA, n = 2)

28 (82)

17 (81)

11 (85)

Hypertension

1 (3)

1 (8)

Depressive symptoms

2 (6)

1 (5)

1 (8)

Obesity

1 (3)

1 (8)

Comorbidities, n (%)

Patients

At the diagnosis of DM, the 34 patients had a mean age of

46  17 years (range, 1877); 24 (71%) were women (Table 1).
The diagnosis of DM was definite in 28 (82%) patients, probable
in 3 (15%) and possible in 1 (3%). Table 1 reports the main
patient characteristics. Signs of severe muscle involvement consisted of dysphagia (n = 17, 50%) and dysphonia (n = 10,
30%). Three patients had pulmonary interstitial disease that did
not require specific treatment. None had cardiac involvement.
Anti-nuclear antibodies were positive (>1/80) in 17 (59%) of the
29 patients for whom this test was available. Ro-SSa antibodies
were positive in only 2 (7%) of the 30 patients with this information. Data on the DM-specific antibodies Anti-JO1 and antiMi2 were missing for 38% and 68% of patients, respectively,
precluding an evaluation of these markers by univariate analysis.
All patients received corticosteroid therapy in a dosage of 1
1.5 mg/kg/day; four patients also received methylprednisolone
bolus therapy (250500 mg/day) during the first month because
of dysphagia, impaired swallowing or dysphonia. Finally, 10
(29%) patients received hydroxychloroquine (400 mg/day) in
addition to corticosteroid therapy.
Histological scoring

An open deltoid or quadriceps muscle biopsy was performed

under local anaesthesia at the Henri Mondor university hospital
in 22 patients and at other centres in three additional patients.
Of these 25 biopsies, 18 were available for review (Table 2). The
International JDM Biopsy Consensus group score indicated severe typical DM features in all 18 patients. There was a typical
inflammatory infiltrate containing CD3+ and CD68+ cells, with
a median inflammatory domain score of 7.5 (range, 012). The
infiltrates were evenly distributed between the perimysial and
endomysial areas. Macrophages predominated in the perivascular infiltrates. Muscle fibre injuries included perifascicular and
non-perifascicular atrophy and regeneration/degeneration/

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Initial clinical characteristics, n (%)

Dermatological abnormalities
Skin necrosis*

12 (35)

7 (33)

5 (38)

Gottrons papules

10 (29)

6 (29)

4 (31)

Periungual involvement

26 (76)

15 (72)

11 (85)

Pruritus

21 (62)

14 (67)

7 (54)

CDASI > 20

14 (41)

9 (43)

5 (38)

Dysphagia

17 (50)

12 (57)

5 (38)

Swallowing disorders

10 (30)

7 (33)

3 (23)

Dysphonia

10 (30)

8 (38)

2 (15)

Soft palate paralysis

1 (3)

1 (5)

Arthralgia

10 (29)

4 (19)

6 (46)

Dyspnoea

5 (15)

4 (19)

1 (8)

Alopecia

3 (14)

Severity of muscle involvement

Other manifestations

Findings from initial investigations, n (%)

CK elevation

30 (88)

18 (86)

12 (92)

CK level >5000 IU

9 (26)

3 (14)

6 (46)

Interstitial syndrome

3 (9)

2 (10)

1 (8)

Electrocardiogram
abnormalities
(NA, n = 15)

*Skin necrosis was dened as necrosis visible to the naked eye or by microscopic examination of a biopsy specimen.
SD, standard deviation; CK, creatine kinase; NA, not available; CDASI, Cutaneous Disease Area Severity Index.

necrosis, with a score of 8 (range, 010). Fibre MHC class I reexpression was noted in all 18 patients. Ischaemia with infarcts
was observed in 10/18 patients. Microvascular involvement
included C5b-9 deposits on endomysial capillaries (16/18) and
capillary drop (3/18). A single patient had arterial abnormalities.
The median VAS score for overall severity was 7 (range, 010).

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816

Table 2 Muscle biopsy characteristics (n = 18) and their association with relapse
Relapse (N = 12)
Median (range)

No relapse (N = 6)
Median (range)

HR
(95% CI)*

P value*

7.5 (412)

7.5 (412)

8 (411)

0.9 (0.71.1)

0.4

0 (03)

0.5 (03)

0 (01)

1.3 (0.72.4)

0.4

Muscle Fibre Domain (010)

8 (210)

7.5 (410)

8.5 (210)

1.0 (0.81.2)

0.7

Connective Tissue Domain (02)

0 (02)

0 (02)

0 (01)

0.4 (0.21.2)

0.1

Visual Analogue Scale (010)

7 (18.5)

7 (18)

7 (18.5)

0.85 (0.71.1)

0.2

Total (037)

24 (9.532)

18.5 (1332)

18 (9.528.5)

1.0 (0.91.1)

0.4

Domain (extreme range)

Median
(range)

Inammatory Domain (012)

Vascular Domain (03)

*Hazard ratios (HR) and their two-sided 95% condence intervals (CIs) were estimated using a Cox model.
Values of P for trend 0.012 (four items) were considered signicant according to Bonferronis Correction.
Values of P 0.05 for the total histological score were considered signicant.

Factors predicting a relapsing course

Complications

Median follow-up was 3 years and 7 months (range, 3 months

31 years) (Tables 2 and 3). At least one relapse occurred in 21
patients, and mean time to the first relapse was 15  26 months
(range 3 months9 years). The number of relapses per patient
ranged from 1 to 8. At relapse onset, mean corticosteroid dosage
was 0.13 mg/kg/day  0.22 and six patients had been off corticosteroid treatment for more than 3 months. Treatments given
for the first relapse were IVIg (n = 3); methotrexate (n = 6);
azathioprine (n = 1); corticosteroids alone (n = 4); corticosteroids combined with methotrexate (n = 4) or cyclophosphamide (n = 1); corticosteroids combined with IVIg (n = 1);
and corticosteroids combined with both methotrexate and IVIg
(n = 1).
Two factors were significantly associated with relapse
(Table 3) by univariate analysis, namely, dysphonia [HR 3.2
(1.28.5)] and severe skin involvement defined as CDASI>20
[HR 3.5 (1.27.9)]. Factors showing a trend towards an association with relapse (P < 0.20 or, for multiple testing of histological
scores, P < 0.05) were arthralgia, dyspnoea, and alopecia.
Dysphagia, muscle weakness severity and the histological score
were not associated with relapse. Because of the small sample
size, multivariate analyses were not performed.

No patient died during follow-up, but 13 (38%) experienced

complications. At least one infection occurred in 7 (20%)
patients: tuberculosis (n = 3; involving the lungs, lymph nodes,
or bone), Pneumocystis jiroveci pneumonia (n = 2], bacterial
pneumonia (n = 2), cutaneous or gastrointestinal abscesses
(n = 3), arthritis (n = 1) and infective endocarditis (n = 1).
Bone complications developed in five patients, including three
with osteoporosis and two with avascular necrosis of the humeral and femoral head respectively.
During follow-up, diabetes mellitus was diagnosed in four
patients and hypertension in five patients.

Discussion
A cohort of 34 DM patients receiving corticosteroid therapy and
followed at a dermatology department between 1990 and 2011
was studied retrospectively. Among these patients, 21 (62%)
experienced at least one relapse defined as recurrent clinical
symptoms requiring a change in treatment. Dysphonia and severe skin involvement at presentation significantly predicted a
subsequently relapsing course. Non-significant trends linked
dyspnoea, arthralgia, and alopecia to subsequent relapses.
Internal validity

Table 3 Factors showing a signicant association or a trend

towards an association with relapse by univariate analysis (N = 34)
Factors associated with relapse by univariate analysis (N = 34)
Initial characteristics

HR*

95%CI

P value**

Dysphonia

3.2

1.28.5

0.02

CDASI 20

3.5

1.27.9

0.02

Arthralgia

0.99.6

0.06

Dyspnoea

2.5

0.87.6

0.12

Alopecia

2.5

0.78.9

0.16

*Hazard ratios (HR) and their two-sided 95% condence intervals (CIs) were
estimated using a Cox model.
**P value from the Cox model.
CDASI, Cutaneous Disease Area Severity Index.

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One strength of this work is that the diagnosis of DM was definite in most of the patients. The distribution of the clinical patterns of DM in our department (classical, paraneoplastic, and
amyopathic/hypomyopathic; Fig. 1) were similar to those
reported previously,9,13 indicating that any selection bias was
probably minimal. As we intended to compare the response to
treatment between patients with and without relapses, we
included only patients who were prescribed corticosteroid therapy at the diagnosis of their disease. Therefore, patients with
amyopathic or hypomyopathic DM were not included. We did
not include patients with paraneoplastic DM, a disease whose
course is governed by the effectiveness of the anti-cancer treatment. The small sample size enabled only univariate analyses,
and an influence of confounding factors therefore remains possi-

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Dermatomyositis: factors predicting relapse

ble. Each of the categories of inflammatory myositis (e.g. classical DM, paraneoplastic DM, and polymyositis) has a specific disease course and specific treatment requirements. This
heterogeneity complicates the interpretation of risk factor data
obtained from studies including all categories. We therefore constituted a uniform population of patients with classical DM,
although this decision limited our sample size and therefore the
statistical power of our study. Also, to minimize bias due to the
retrospective design, we reviewed the muscle biopsy specimens
using a validated scoring tool.

817

Other factors reported to be associated with a poor outcome

of DM are older age, male sex, neoplasia, cardiac or pulmonary
involvement, dysphagia, anti-JO1 or Ac anti-SRP antibodies,
vasculitis lesions, and a long time from symptom onset to treatment.4,7,8,19 These risk factors were identified in heterogeneous
populations including patients with paraneoplastic DM,
polymyositis, JDM and overlap syndrome. Furthermore, they
were not specifically associated with relapse but, instead, with a
poor outcome defined as relapse, disease progression or death.
The results of these earlier studies cannot therefore be meaningfully compared to our findings.

External validity

The 62% relapse rate in our study is consistent with the rates of
3865% reported by others.5,7 Time from diagnosis to first
relapse was 15 months, and most relapses occurred during the
corticosteroid taper, also in keeping with previous publications.5,14 This last finding emphasises the need for close monitoring during the corticosteroid taper.
Dysphonia at presentation significantly predicted a relapsing
course by univariate analysis. Dysphonia is a well-known indicator of severe DM that is routinely sought during the initial evaluation. In a multicentre cohort of 490 patients with JDM,
dysphonia/dysphagia at onset was significantly associated with
persistent muscle weakness.15 In contrast, in 77 adults with
polymyositis or DM, dysphonia/dysphagia at onset was not associated with subsequent outcomes.4
Greater severity of the initial skin involvement also significantly predicted a relapsing course: CDASI10 values 20 at onset,
as assessed retrospectively, were associated with a subsequent
relapse. The retrospective determination of the CDASI is an
important limitation of our study, but nevertheless enabled a
standardised assessment of skin lesion severity, as the dermatological features in each patient were recorded in the same way by
a single dermatologist (AC) throughout the study period. This
method minimised classification bias. In a study of 32 adults
with DM, extensive skin lesions over the torso were associated
with a poor outcome16 and, in children with JDM, persistent
periungual erythema or Gottrons papules were associated with a
longer time to remission.17 These findings support the need for
a standardized assessment of the skin involvement with the goal
of optimising the treatment and follow-up.
The initial muscle-biopsy findings assessed using a validated
scoring tool were not associated with a relapsing course. In a
study of 72 muscle biopsies from patients with JDM, extensive
myopathic changes and central nuclei without basophilia were
associated with a poor outcome, defined as a variety of clinical
events and not only as a relapse.18 In our cohort, the availability
of initial muscle biopsies for only 18/34 patients limited the statistical power of our study to detect associations with histological features. Further studies of these features in larger cohorts
are needed.

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Conclusion
Factors associated with a relapsing course were assessed in a uniform cohort of adults with classical DM (as opposed to paraneoplastic or amyopathic/hypomyopathic DM). Dysphonia and
severe skin involvement at presentation significantly predicted a
relapsing course by univariate analysis. We are currently conducting a larger multicentre cohort study to further evaluate the
predictors of relapse in adults with classical DM. Should this
new study confirm the present findings, there would be a rationale for evaluating the appropriateness of greater treatment
intensity in patients at high risk for relapse and of lower
corticosteroid dosages in patients at low risk for relapse.

Acknowledgements
We thank A. Wolfe, MD, for revising the manuscript.

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