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Case Based Review
Published: 23 December 2019
Dilemma in management of
hemorrhagic myositis in
dermatomyositis
Julia M. Chandler
Irene L. Wapnir
, Yoo Jung Kim, Justin L. Bauer &
Rheumatology International 40, 331–336 (2020)
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Abstract
Dermatomyositis (DM) is a rare inflammatory
disorder affecting the muscle and skin. DM
patients can present with spontaneous muscle
hemorrhage, a potentially fatal complication.
The best practice for management of
hemorrhagic myositis in these patients
remains unclear. Here we discuss the case of a
patient who presented with progressive muscle
weakness and intermittent rash that was
diagnosed with dermatomyositis. During
admission, she developed spontaneous
hemorrhagic myositis of the right pectoralis
Log in
major treated with surgical evacuation. She
also developed a spontaneous left anterior
thigh hematoma which was treated
conservatively. She recovered and showed no
evidence of recurrent bleeding at either
location. We performed a literature review and
identified ten cases of spontaneous
hemorrhage in DM patients, with a 60%
mortality rate among reported cases. Given the
high mortality rate associated with
spontaneous hemorrhage in DM patients, it is
important for physicians to be aware of the
diagnosis, workup, and management
strategies.
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Introduction
Dermatomyositis (DM) is a rare systemic
autoimmune disease of skin and muscle
inflammation that affects 3 to 23 in 100,000
people [1, 2]. Patients present with weakness
of the proximal muscles, pink papules
overlying the dorsal interphalangeal joints
(Gottron’s papules), periorbital rash
(Heliotrope sign), and nailfold telangiectasias.
DM is associated with an increased incidence
of cancer, esophageal motility disorders,
calcinosis, and interstitial lung disease [3].
Spontaneous hemorrhagic myositis is a rare
complication of DM that can be fatal
[4,5,6,7,8]. The pathophysiology has not been
clearly elucidated; previous case reports
describe a possible association with deep vein
thrombosis (DVT) prophylaxis medications
such as heparin and dalteparin, although cases
of idiopathic hemorrhagic myositis also exist
[5, 9].
We describe a case of recurrent hemorrhage in
a patient with DM. A review of the literature of
DM-related spontaneous muscular
hematomas and their management and
prognosis is included.
Case presentation
A 64-year-old female with no significant past
medical history and no current medications
presented to the emergency department with a
2-month history of progressive weakness, a
5-month history of intermittent rash of the
face and hands, and a 1-week history of
progressive hoarseness and dysphagia. The
generalized muscle weakness was severe as
she was unable to stand from a chair or lift her
head while supine. Other notable features of
the physical exam were Gottron's papules on
the bilateral fingers, violaceous rash or Holster
sign on the thighs, and non-pitting edema in
both legs. Muscle strength was 3/5 in the
proximal upper extremities, hip flexors, and
hip extensors. Labs were consistent with DM,
including elevated aspartate aminotransferase
(337 U/L; reference: < 40 U/L), alanine
aminotransferase (276 U/L; reference: < 60
U/L), creatinine kinase (4812 ng/mL;
reference: < 60 ng/mL), c-reactive protein
(1.9; reference: < 0.9 mg/dL), and lactate
dehydrogenase (2268 U/L; reference: < 340
U/L). Coagulation labs were notable for an
elevated activated partial thromboplastic time
(54.1 s; reference: 25.1–36.6 s); however, these
labs were drawn after initiation of routine
prophylactic subcutaneous heparin, and a
subsequent PTT inhibitor screen had
indeterminate results due to the presence of
anticoagulant in the sample. She tested weakly
positive for anti-Mi-2, but other DM-
associated antibodies were negative. Skin
biopsy showed “vacuolar interface dermatitis
with mucin,” compatible with DM. The patient
had no known risk factors for malignancy, and
an extensive screening workup for cancer was
performed with studies for breast, colon, and
cervical cancers all negative.
On admission, the patient was started on
methylprednisolone 1 g for 3 days, intravenous
immunoglobulin 2 g/kg over 2 days, and
mycophenolate mofetil 500 mg twice daily,
resulting in prompt improvement in strength
and swallowing (Fig. 1). Subcutaneous heparin
for DVT prophylaxis was also initiated.
Fig. 1
Timeline of significant events and medication
regimen for a case of hemorrhagic myositis in a
dermatomyositis patient
Two days after admission, she developed rapid
enlargement of the right breast (Fig. 2)
associated with hypotension of 60/36 and a
drop in hemoglobin from 12.0 to 9.7 g/dL.
Two units of fresh frozen plasma were
administered, and a computed tomography
(CT) angiogram of the chest revealed active
arterial extravasation within a 13 cm
hematoma located in the right pectoralis
major muscle (Fig. 3). Given the hemodynamic
instability and further decrease in hemoglobin
to 7.9 g/dL over the ensuing hours, the patient
was taken to the operating room for
hematoma evacuation and exploration for the
source of bleeding. The muscle fibers were
splayed, containing a 400 mL hematoma that
extended medially to the sternal border,
superiorly to the clavicle, and laterally along
the mid-axillary line down to the
inframammary fold. However, no significant
arterial or venous bleeding vessels were
identified. Numerous bleeding small
intramuscular vessels were cauterized or
ligated. Two units of packed red blood cells
and one unit of cryoprecipitate were
administered intraoperatively, and a JacksonPratt drain was placed. A muscle biopsy was
sent as part of the surgical pathology and was
notable for “extensive hemorrhage” in the
setting of a “chronic myopathic process.”
Fig. 2
Top–down comparison between the normal left
breast and the swollen right breast in a
dermatomyositis patient with hemorrhagic
myositis of the right pectoralis major. The right
breast demonstrates tense fullness superiorly
and laterally
Fig. 3
Computed tomography (CT) angiogram
demonstrating large right pectoralis major
hematoma in a dermatomyositis patient with
hemorrhagic myositis. There is active
extravasation within the hematoma, surrounding
fat stranding, and edema involving the serratus
anterior. a Arterial phase CT with black arrow
pointing to a focus of active extravasation within
the hematoma, b venous phase CT with a black
arrow pointing to further pooling of extravasated
contrast
The postoperative recovery was uneventful.
However, on the second day, the patient
experienced increasing pain and swelling in
the anterior left thigh. An ultrasound showed a
massive 30.5 cm hematoma occupying the
area. This represented a second episode of
hemorrhagic myositis. A decision was made to
treat this event conservatively with a pressure
dressing, because the patient was otherwise
asymptomatic and blood counts remained
stable. There was minimal drainage from the
chest wall drain, and she was discharged to a
skilled nursing facility on oral prednisone
60 mg daily and mycophenolate mofetil
1500 mg twice daily.
Ten months after discharge, the patient’s
overall muscle strength has improved except
for the left lower extremity where the
hematoma was not drained. Bilateral manual
muscle testing 8 (MMT8) scores have been
largely stable to improving, with scores of 128
when first performed 6 months after
admission and 133 another 6 months later
(maximum score possible: 150). Significantly,
the characteristic skin findings, including
Holster sign and pink erythema of face, neck,
hands, elbows, arms, and knees, have
persisted, but with gradual improvement over
time; scores on the Cutaneous
Dermatomyositis Disease Area and Severity
Index (CDASI) decreased from 16 to 9 in the
3 months following admission. She remains
compliant with a regimen of mycophenolate
mofetil 1500 mg twice daily and prednisone
5 mg daily, which is being tapered.
Search strategy
We used MEDLINE, EMBASE, Scopus, and
Web of Science to find studies describing
patients with spontaneous hemorrhagic
myositis in the context of a previous or
concurrent DM diagnosis. The search was
conducted using the following terms:
“dermatomyositis” and “spontaneous
hematoma” or “spontaneous hemorrhage” or
“spontaneous hemorrhagic myositis.” The
search was performed from inception of each
database through August 2019. The references
in each selected study were reviewed to
identify other relevant articles. We excluded
non-English articles, conference abstracts, and
articles describing hemorrhagic myositis in
patients not previously or concurrently
diagnosed with DM. Two authors reviewed the
search results and agreed on the included
articles.
From each article selected, data were collected
on age, gender, location of the hematoma(s),
presence of DM-associated antibodies, the use
of DVT prophylaxis prior to identification of
the spontaneous hematoma, the treatment for
the spontaneous hematoma, the listed
complications, and the case outcome.
Results
We identified eight reports describing ten
cases of spontaneous hemorrhage in patients
with DM (Table 1) [4,5,6,7,8,9,10,11]. The
cases occurred most commonly in adults older
than 50, with roughly equal rates in males and
females [10]. The sites of hemorrhage were
varied, including the deltoid and trapezius
muscles, rectus abdominus, retroperitoneum,
and psoas muscles; five out of ten patients
experienced involvement of the iliacus and
psoas [5, 7, 8, 11]. Six out of ten patients
presented with at least two hematomas in
distinct anatomical locations [5,6,7, 11].
Treatment strategies included observation,
conservative treatment with blood transfusion,
and embolization. There was a 60% mortality
rate among reported cases.
Table 1 Reported cases of
spontaneous hemorrhagic myositis
in dermatomyositis patients
Discussion
Hemorrhagic myositis is a rare complication of
DM, with only ten prior cases reported in the
literature. It occurs most commonly in adults
over 50 years of age with roughly equal rates
in males and females, and various muscle
groups can be affected. In the majority of
reported cases, DM-associated hemorrhagic
myositis is fatal.
There is no consensus on treatment strategies
for hemodynamically significant hemorrhagic
myositis in patients with DM. Three
management strategies have been previously
described: observation, conservative treatment
with transfusions, and arterial embolization.
Orrell et al. described two patients treated
successfully without procedural intervention,
one of whom was observed and the other who
was supported with blood transfusions [10].
Three patients underwent arterial
embolization, all of whom eventually died. In
one patient, arterial embolization initially
achieved hemostasis; however, bleeding
recurred at the same site 1 week later, and the
patient subsequently expired [4]. In one case,
interventional radiologists were unable to
isolate the source of a patient’s hemorrhage,
and the patient died from multiorgan failure
[7]. These findings are consistent with the
diffuse muscle hemorrhage seen in our
patient, which would not have been amenable
to treatment with embolization due to lack of a
single identifiable bleeding vessel.
In non-DM patients, spontaneous hematomas
most commonly occur within the rectus sheath
or iliopsoas muscle and with use of
anticoagulant or antiplatelet therapies
[12,13,14]. It is worth noting that the latter
occurs due to bleeding from a single blood
vessel, in contrast to DM-associated
hematomas which occur due to diffuse
bleeding from multiple small vessels. As such,
non-DM hematomas are typically treated
conservatively or, in cases of continued
expansion or hemodynamic instability, with
catheter embolization of the affected vessel.
Risk factors for DM-associated hemorrhagic
myositis leading to hematoma remain poorly
understood. The use of prophylactic
anticoagulation has been linked as an
exacerbating factor increasing the risk of
bleeding in DM patients, a factor that may
have contributed to or exacerbated the rapid
evolution of pectoral hematoma in our case [5,
9]. However, at least two patients developed
spontaneous hemorrhage without prophylactic
anticoagulation [10]. Furthermore, among the
eight patients who developed a hematoma
after anticoagulation, two had normal
activated partial thromboplastin times [4, 6].
Patients with DM may have increased risk of
DVT, complicating the decision of whether to
initiate anticoagulant or antiplatelet therapy
[15]. The inflammatory nature of DM is
thought to cause hypercoagulability through
alterations to the coagulation cascade,
including an imbalance of procoagulants and
anticoagulants and suppression of fibrinolysis
[16]. Furthermore, DM patients have a
number of other risk factors for DVT,
including concomitant malignancies and
immobilization secondary to disease activity.
Despite this increased risk of DVT, DM
patients are not routinely maintained on
outpatient antiplatelet or anticoagulant
therapy, and recent attempts at consensus
guidelines for management of DM have yet to
address the issue [17].
The case presented herein describes the
occurrence of two hematomas in the same
patient, one treated by surgical evacuation and
the other non-operatively. This case is unique
as it is the first report of DM involving the
pectoralis major muscle. Diffuse bleeding from
many small intramuscular vessels was
encountered with pathology showing a chronic
myopathic process, consistent with the
suspected pathophysiology of DM-associated
microangiopathy. The bleeding may have been
exacerbated by administration of prophylactic
heparin at the time of admission. Weakness in
the left thigh has persisted where the
hematoma was managed conservatively.
Physicians lack specific recommendations
regarding management of hemorrhagic
myositis in DM patients. We suggest the risk
of hemorrhagic myositis be remembered when
starting prophylactic anticoagulation. Our case
suggests that in addition to aggressive clinical
management, operative intervention is
warranted when active extravasation is
identified. Successful treatment modalities
have included conservative therapy with
transfusions and surgical evacuation. Although
rare, the presence of a spontaneous hematoma
should take into consideration the possibility
of DM as an underlying cause.
Conclusion
Spontaneous hemorrhagic myositis in the
setting of DM is rare but can be fatal. When
admitting patients with DM symptoms,
physicians should be cognizant that multiple
sites may be involved and should consider the
risk of exacerbating hemorrhagic myositis
when starting DVT prophylaxis. Embolization
as a means of treating diffuse extravasation
has not been shown to be effective. More
research remains to be done to elucidate the
risk factors and pathophysiology of
spontaneous hemorrhagic myositis in
dermatomyositis.
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Acknowledgements
The authors would like to thank Dr. Matthew
Lewis for his review of the manuscript.
Funding
There was no funding provided for this report.
Author information
Authors and Affiliations
Department of Surgery, Stanford
University School of Medicine, 300
Pasteur Drive, Rm H3591, Stanford, CA,
94305, USA
Julia M. Chandler & Irene L. Wapnir
Stanford University School of Medicine,
Stanford, CA, 94305, USA
Yoo Jung Kim
Department of Radiology, Stanford
University School of Medicine,
Stanford, CA, 94305, USA
Justin L. Bauer
Contributions
JC conceived the study and drafted and
critically revised the manuscript. YK
performed the literature search and drafted
and critically revised the manuscript. JB
critically revised the manuscript and provided
guidance on radiologic findings. IW critically
revised the manuscript and provided overall
guidance for the project. All authors read and
approved the final manuscript and agree to be
accountable for all aspects of the work.
Corresponding author
Correspondence to Julia M. Chandler.
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Conflict of interest
The authors report no conflicts of interest.
Informed consent
Informed consent was obtained from the
patient described in the study, including
permission for release of photographs.
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Cite this article
Chandler, J.M., Kim, Y.J., Bauer, J.L. et al. Dilemma in
management of hemorrhagic myositis in
dermatomyositis. Rheumatol Int 40, 331–336 (2020).
https://doi.org/10.1007/s00296-019-04501-7
Received
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Published
2019
2019
2019
16 September
Issue Date
11 December
23 December
Keywords
Dermatomyositis complications
Hemorrhage
Myositis
Hematoma
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