A 27-year-old woman and a 13-year-old girl diagnosed with juvenile der
matomyositis in childhood developed clinical findings of partial lipod
ystrophy 10 years after diagnosis. Exhaustive clinical and laboratory
examinations showed an association with other abnormalities: hypertric
hosis, steatohepatitis, and an abnormal insulin response to the glucos
e loading test in the first patient. Hypertrichosis, steatohepatitis,
insulin-resistant diabetes mellitus, and acanthosis nigricans were obs
erved in the second patient. Renal function was normal in both patient
s. Although a localized form of lipodystrophy has been reported associ
ated with connective tissue disease (connective tissue lipoatrophy), t
he partial form has been infrequently described in association with ju
venile dermatomyositis.