Nigerian Journal of
Paediatrics 2011;38 (4):182 - 185
CASE REPORT
Mustapha MG
Juvenile Dermatomyositis in a Nigerian
Ashir
MG
Mayun
AA
Girl: a Case Report.
Machoco Y
Ibrahim AB
Received: 31st May
2011
Summary A
case of
Juvenile
clinical findings
consistent with
Accepted: 25th
September 2011
dermatomyositis (JDM)
in a 10
JDM were found. Her
condition
year old Nigerian girl
is herein
improved with steroids,
cytotoxic
Mustapha MG (
)
reported to discuss
some of the
therapy and
physiotherapy. Some
Department of
features of the disease
and
investigation
and
treatment
Paediatrics,University
of
challenges in
management of such
modalities could not be
accessed for
Maiduguri Teaching
Hospital.
a rare but crippling
autoimmune
the benefit of the
patient. Although,
PMB 1414,
Maiduguri.
vasculopathy of
childhood. She
the outcome of patients
with JDM
Email:
mgofama@yahoo.com
was referred to the
University of
has improved with the
discovery of
Tel.
+2348038087639.
Maiduguri Teaching
Hospital
steroids, the disease
is shown to
(UMTH) with an
eight-month
have a variable course,
with
history
of
recurrent
fever,
attendant social and
financial
Ashir MG,
MayunAA,
abdominal pain, and a
four-month
implications especially
to the
MachocoY,
IbrahimAB
history of body rash
and inability
immediate
family.
Department of
Pathology,
to walk or sit. Muscle
biopsy and
University of
Maiduguri
Teaching
Hospital
PMB 1414,
Maiduguri.
Introduction
rapid onset of symptoms
and gastrointestinal tract
involvement.
5,6
Juvenile
dermatomyositis (JDM) is the most common
childhood inflammatory
myopathy. It is a systemic,
1
We report a case of JDM
in a Nigerian girl which to
autoimmune inflammatory
muscle disorder and
the best of our
knowledge has not been previously
vasculopathy that
affects children younger than 18
reported in Nigeria
probably because of its rarity or
years, primarily
affecting the skin and the skeletal
because it was usually
missed by clinicians. Some of
muscles. Characteristic
findings include Gottron
the features disease
and challenges encountered in
papules, a heliotrope
rash, calcinosis cutis, and
management are also
highlighted.
symmetrical proximal
muscle weakness.
Case
report
An estimated annual
incidence of 3.4, 3.3 and 2.7
cases per million
children were reported in whites,
HUA was a 10 year old
primary school girl referred
blacks and Hispanics
respectively, and a female to
2
from a general hospital
to University of Maiduguri
male ratio of 2.3:1 and
5:1 in the United States and
Teaching Hospital
(UMTH) with an eight month
United Kingdom
respectively.
2,3
The median age
of
history of recurrent
fever, abdominal pain, and facial
onset of JDM is 6.8
years in girls and 7.3 years in
puffiness, four months
history of body rash,
boys, with a median
diagnosis delay of 3-4 months.
4
dysphonia and inability
to walk or sit. Prior to
Prior to the advent of
corticosteroids, the prognosis
referral, she was being
treated at the referring facility
was poor. Brunsting and
Banker types of JDM have
for acute
glomerulonephritis (AGN) and anaemia,
been described in
children, with the former type
where she had received
blood transfusion and other
characterized by slow
progression and better
medications without
improvement. Although, a
outcome compared to the
latter type characterized by
history of sore throat
preceding the onset of
183
symptoms was obtained,
no history of change in
Skeletal muscle CK
iso-enzyme analysis was not
urinary frequency,
volume or colour was found.
done due to non
availability of the reagents. Other
immunologic tests such
as anti-nuclear antibody,
There was also no
history of haematuria or dark
myositis associated
autoantibody were not done due
coloured urine. The
body rash was more on the upper
unavailability in our
centre. Stool for occult blood,
torso, extensor
surfaces of both upper and lower
urine microscopy,
culture and sensitivity were
limbs and the buttocks.
No associated joint swelling
negative. Complete
blood count and serum
or pain but there was
generalised body ache with
electrolytes, urea and
creatinine were not remarkable.
classical history of
proximal muscle weakness that
Histology of incisional
muscle biopsy from the right
Progressed to inability
to walk, sit-up and hold the
upper thigh
demonstrated focal areas of
neck. At about the same
time, she was noticed have
inflammation, necrosis,
few myofibre fragmentation
nasal quality speech
with occasional regurgitation of
as well as atrophy and
fibrin which are in keeping
fluid via the nose. She
had no preceding history of
with JDM (Fig
2).
exposure to vaccines,
drugs or insect bite. HUA was
not a known sickle cell
disease patient and had no
Fig
2
history suggestive of
diabetes mellitus. There was no
family history of such
or similar presentation.
On admission, physical
examination revealed a
chronically ill looking
pale, girl with facial puffiness
but was afebrile, not
jaundiced and had no significant
peripheral
lymphadenopathy. Her weight was 26kg
(80% of expected for
age) and her length was 137cm
(100% of expected for
age). The main findings were
in the musculoskeletal
system which revealed
Photomicrograph showing
myofibre necrosis, atrophy and
generalised body
tenderness. She was unable to raise
lymphocytic infiltrates:
MG X 200). Radiograph of the upper
arms showed no evidence
of calcifications.
her arms even to feed
herself. She was curled up
almost in a foetal
position, unable to sit and had some
She was commenced on
prednisolone 2mg/kg/24hr
contractures at the
elbow and the knee joints.
and four weeks later,
weekly methotrexate 15mg/m
2
Maculopapular skin
eruptions were observed in the
was added because of
slow response to the steroid.
upper torso, extensor
surfaces of arms and legs and
Following resolution of
muscle pain and tenderness,
the buttocks. A crop of
peri-orbital eruptions was
physiotherapy was
commenced. The patient
noted below the right
medial canthus (heliotrope) and
responded to the
treatment with resolution of the
the skin over the
metacarpal and proximal
fever; headache, muscle
pain and desquamation and
interphalengeal joints
fitting the pattern of typical
healing of skin
eruptions. She regained ability to feed
Gottron papules.
herself and was able to
sit unsupported for up to an
hour. About a week
after initial improvement, she
The lesions were
hypertrophied and erythematous
developed high grade
continuous fever and anorexia.
Fig
1
Sepsis work up was
done, but no organism was
isolated and no focus
for the infection was found. She
was given anti-malarial
and antibiotic medications to
no avail. HUA
subsequently died two weeks
following the onset of
the fever.
Discussion
Although, no
subcutaneous mass or swelling was
found, an ulcer on the
right elbow measuring 2x3cm
The diagnosis of JDM is
challenging especially in a
in diameter with
lobulated floor was noted. Diagnosis
developing country like
Nigeria where adequate
of Juvenile
Dermatomyositis
(JDM) was
facilities, equipment
and expertise may be lacking. In
1975, Bohan and Peter
proposed criteria for the
7
entertained. Blood
pressure and urinalysis remained
normal throughout the
period of admission. HIV
diagnosis of JDM. These
criteria include
antibody test and
Rheumatoid factor were negative.
characteristic skin
rash, proximal muscle weakness,
Antistreptolysin O
titre was positive (400 IU) but
elevated muscle
enzymes, myopathic changes on
total creatine-kinase
(CK) titer was within normal
electromyography and
abnormal muscle biopsy
limits.
findings. Typical skin
findings in combination with
184
Three other criteria
are necessary to make a definitive
The fact that the
biopsy was not guided by MRI
diagnosis. Patients
with the characteristic skin
probably suggest
extensive muscle involvement in
eruptions who fulfill
only two criteria are adjudged
the patient. Other
evidence of severe disease in the
to have probable JDM.
The proposed criteria have
patient include nasal
speech and regurgitation of
been expanded to include
typical MRI and
liquids through the
nose and gastrointestinal tract
symptoms.
1,8
ultrasonography
findings, including affected muscle,
nail fold
capillaroscopy, presence of calcinosis, and
dysphonia. The index
patient had fulfilled almost all
Calcifications occur in
20-40% of children with
the above criteria
except the tests that could not be
dermatomyositis and is
implicated in increasing the
morbidity and mortality
of the disease.
11
done due to lack of
necessary facilities. The
In JDM,
symptoms, signs and
skin eruptions found in the
calcifications are more
frequent at anatomic sites that
patient were typical of
JDM. The median time for
are normally exposed to
daily minor trauma but not
diagnosis after onset
of the disease was put at four
usually mineralized,
such as the elbows or behind the
knees. Calcinosis on
the other hand, is seen as
9
months,
1,4
diagnosis was made 8
months into the
illness in our patient.
The delay in diagnosis was due
crusted papules or
plaques around joints or as non-
to late presentation,
referral, and lack of suspicion of
healing sores. The
intractable ulcers at the elbows in
the disease, due to its
rare occurrence.
the patient may suggest
the existence of calcinosis.
Sometimes, the
calcified material is extruded through
Although the cause of
JDM is not known, infection-
the skin as a white
cheesy exudate, leaving behind a
triggered autoimmunity
has been proposed as a
dry pitted scar.
8
Muscle calcification
results in
possible precipitating
factor. Infectious agents
contractures or severe
muscular pain. Calcinosis is
implicated include
coxsackie B virus, parvovirus
thought to be
dystrophic, as damaged muscles release
B19, enteroviruses,
and Streptococcus species.
1,4
mitochondrial calcium
into matrix vesicles that
Preceding history of
sore throat was obtained in the
promote mineralization.
Calcinosis lesions are rarely
patient, so also
elevated ASO titre. It is probable
present at diagnosis
but are usually found later during
therefore that a
streptococcal infection triggered the
the course of the
disease.
1,5
Delayed treatment
and
autoimmunity in the
patient. Noninfectious agents
severe disease have
been reported as risk factors for
implicated in the onset
of JDM include D-
development of
calcinosis.
1,4,8
penicillamine,
vaccinations, and bone marrow
transplants among other
triggers.
4
The goals of treatment
include control of
inflammation
manifesting as pain, muscle weakness,
Negative rheumatoid
factor and anaemia are
skin lesion and
prevention and management of short
common occurrence in
patients with JDM.
1,4
term and long term
complications of the disease and
Although, an elevated
skeletal muscle creatinine
treatment. The main
stay of therapy is steroids. For
kinase (CK) is an
important finding in patients,
1,7,8
a
severe and refractory
cases, intravenous methyl
normal level does not
exclude JDM as the enzyme
prednisolone can be
given. Second line drugs
level is usually
elevated in the early phase of the
methotrexate,
cyclosporine, azathioprine and
disease, during the
period of maximum muscle
h y d r o x y c h l o r
o q u i n e .
C y c l o s p o r i
n
a n d
inflammation and
destruction.
1
Pachman et al
9
hydroxychloroquine are
reported to be effective
reported that two out
of five patients with definite
especially for skin
involvement.
1,8
Intravenous
JDM had normal muscle
enzyme when re-evaluated
immunoglobulin (IVIG)
may be indicated by relapse,
incomplete response, or
for steroid sparing purposes,
8
some months later,
though they had elevated enzyme
levels initially at the
time of diagnosis. The patient
the addition of the
other second line drugs in the
presented to us eight
months into the illness and had a
management of JDM also
has the benefit of steroid
normal total CK. It is
possible that she had elevated
sparing effect.
CK in the early phase
of the disease consistent
withthe natural history
of JDM. Other muscle
Due to the effect and
contribution of cytokine like
enzymes That may be
elevated in JDM are aspartate
TNF-α in the
pathophysiology of dermatomyositis,
aminotransferase,
lactic dehydrogenase and aldolase.
the use of biologic
agents in management of JDM is
currently gai ning
ground s .
The use of
anti-TNF-α
Radiographic MRI using
T2 weighted images and fat
has shown mixed results
in the management of
suppression localizes
the active site of disease for
JDM.
4,8
Rituximab, a monoclonal
CD20+B cell-
diagnostic muscle
biopsy and electromyogram,
depleting antibody, is
another potential promising
which are each non
diagnostic in 20% of instances if
new biologic agent in
the management of JDM, its
Not directed by MRI.
The typical muscle biopsy
1
potential use is also
investigated in a broad range of
conditions involving
B-cells.
4,8
pathological finding of
JDM in the patient despite
10
185
Treatment adjuncts
include calcium and vitamin D to
progression with a
recurrence of active disease after a
prolonged remission
have been reported.
8
correct the osteopenia
of JDM and to decrease the
frequency of bone
fracture, avoidance of exposure to
sun and also use of
sunscreen, to provide protection
The period of active
symptoms has decreased from
against ultraviolet A
and B.
1,8
Antacids or H2
about 3.5 years to less
than 1.5 years with more
blockers, if dyspeptic
symptoms are precipitated
aggressive
immunosuppressive therapy and a
bysteroids and
antibiotics, especially for an infected
mortality of about
1-10% was reported in western
literature.
1,4,5
ulcer. Vitamin
supplements may also be beneficial.
Prior to the advent of
corticosteroids, it
Physiotherapy provides
passive stretching early
was reported that one
third of affected children died
and another one third
were disabled. The outcome of
1
inthe disease and once
active inflammation has
resolved, direct
reconditioning of muscles to regain
treatment of JDM in
Nigeria is not known. Although
strength and range of
motion is initiated. Bed rest is
HUA had initial
clinical improvement, sepsis set in
not indicated. Social
work services may help
(but cultures were
sterile) and scuttled the
facilitate adjustment
to the frustration of physical
management of the
patient towards achieving
impairment in a
previously active child. Formation
complete
remission.
of social support
groups as in other chronic
debilitating illnesses
may assist children with this
Even though, the health
burden of rare disease
condition and their
parents or care givers.The course
conditions like JDM on
the Nigeria nation may be
of JDM may be as brief
as eight months
low, its effect and
burden on the affected family is
high, with attendant
social and financial
With complete recovery,
or it can last two or more
implications. The
challenges posed by this disease to
years with a continuing
requirement for treatment.
8
care givers especially
in developing countries like
Acute exacerbations and
remission without any
Nigeria are enormous.
These concerns are depicted
stabilization of the
initial course of the disease, late
by this case
report.
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