Nigerian Journal of Paediatrics 2011;38 (4):182 - 185
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
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
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
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.
rapid onset of symptoms and gastrointestinal tract
Juvenile dermatomyositis (JDM) is the most common
childhood inflammatory myopathy. It is a systemic,
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
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.
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.
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
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
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.
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
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
findings. Typical skin findings in combination with
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
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.
done due to lack of necessary facilities. The
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
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.
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.
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.
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.
penicillamine, vaccinations, and bone marrow
transplants among other triggers.
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.
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,
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.
Pachman et al
hydroxychloroquine are reported to be effective
reported that two out of five patients with definite
especially for skin involvement.
JDM had normal muscle enzyme when re-evaluated
immunoglobulin (IVIG) may be indicated by relapse,
incomplete response, or for steroid sparing purposes,
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
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
potential use is also investigated in a broad range of
conditions involving B-cells.
pathological finding of JDM in the patient despite
Treatment adjuncts include calcium and vitamin D to
progression with a recurrence of active disease after a
prolonged remission have been reported.
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.
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
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
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.
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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