In a patient with acute posterior multifocal placoid pigment epithelio
pathy (APMPPE), a pontine infarction occurred about 6 months after the
ophthalmological manifestation. We report the first case with histopa
diologically proven vasculitis shown by muscle biopsy and the firs pos
itron emission tomographic documentation in APMPPE. The ophthalmologic
al and cerebral symptoms responded well to steroid treatment. Long-ter
m immunosuppression (e.g. azathioprine 1-2 mg/kg) seems to decrease th
e risk of recurrent systemic vasculitis.