195
F. F. ÖZKİNAY, et al.
Introduction
A link between dermatomyositis and neoplasia has
long been suspected. The precise incidence of this as-
sociation remains controversial, owing to varying cri-
teria used to diagnose dermatomyositis. Although juve-
nile dermatomyositis is believed to display no
association with neoplasm, there have been few re-
ported cases of dermatomyositis associated with tu-
mors of any kind in pediatric age group (1-4). We de-
scribe another such case and review briefly the current
status of the association of neoplasia with juvenile der-
matomyositis.
This female child was normal until the age of 15
months when she began to have fever and swelling of
extremities. When she was 21months old she was
brought to Pediatrics Clinic of University Hospital with
the complaining of fever, flashing on the face, pain
and swelling of the arms and legs and difficulty in
walking. She was born to 17-year-old mother and
parents were first cousins. Physical examination re-
vealed nonpitting edema on dorsal side of the extrem-
ities, patchy erythematous and nonitching rash in the
skin, periorbital purplish discoloration associated with
mild edema (heliotrope), proximal muscle weakness
and hypertension (150/80 mmHg). Liver was palpable
2 cm below the costal margin. Laboratory in-
vestigations showed Hb, 9.7 gr/dl; Hct, 31%; ESR,
Tr. J. of Medical Sciences
28 (1998) 195-197
© TÜBİTAK
68mm/h; CPK, 97 IU/L; LDH, 913 IU/LP; AST, 65 IU/
L; ALT, 85 IU/L. IgM were increased, 1720 mg/dl and
261 mg/dl respectively. Rest of the biochemical tests
were normal. EMG revealed small, short duration poly-
phasic motor unit potentials. Muscle biopsy showed
perifascicular muscle fiber atrophy and scattered mus-
cle fiber necrosis and regeneration, and mononuclear
cell infiltration of muscle fibers. Immunocytochemical
studies demonstrated endomysial IgG deposition. Skin
biopsy from sclerotic area showed increased collagen
fibers and atrophic sweat glands. Renal biopsy was
normal. On the basis of the presence of proximal mus-
cle weakness, chronic inflammatory changes in skin
and muscle biopsy and characteristic EMG findings the
diagnosis of dermatomyositis was made.
Hypertension was treated with antihypertensive
drugs. Prednisolone therapy (2 mg/kg) was instituted
to treat active dermatomyositis. ESR was gradually de-
creased from 68 mm/h to 20 mm/h. Prednisolone was
reduced to 5 mg/d within one month. After the fol-
lowing-up as out-patient for five months, she was hos-
pitalized again because of the gradually developing
subcutaneous solid nodules and plaques. She had dif-
ficulties in walking and sitting. Physical examination
revealed marked proximal muscle weakness and hep-
atosplenomegaly. Laboratory studies showed ESR, 50
mm/h; LDH, 250 IU/L; CPK, 250 IU/L. Rest of the la-
boratory results were unremarkable. Plain radiographs
F. Ferda ÖZKİNAY
1
Sevgi MİR
1
Nurettin Onur KUTLU
1
Caner KABASAKAL
1
Eren DEMİRTAS
2
Kaan KAVAKLI
1
Güngör NİŞLİ
1
Juvenile Dermatomyositis Associated with Acute
Lymphocytic Leukemia
Departments of
1
Pediatics,
2
Pathology, Fa-
culty of Medicine,Ege University, Bornova,
İzmir-Turkey
Key Words: Juvenile dermatomyositis, Leu-
kemia, Malignancy
Received: June 19, 1996
Short Report