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CNS involvement in malignant atrophic papulosis (Kohlmeier-Degos disease): vasculopathy and coagulopathy.
[malignant atrophic papulosis]
The
clinical
features
in
a
42
year
old
man
with
malignant
atrophic
papulosis
with
CNS
involvement
are
described
.
They
included
mental
dysfunction
,
paraesthesiae
,
weakness
of
left
limbs
,
with
pyramidal
tract
signs
,
bilateral
ptosis
,
progressing
to
total
ophthalmoplegia
and
obtundation
.
There
were
two
crops
of
characteristic
papular
lesions
on
trunk
and
limbs
,
with
terminal
evidence
of
a
coagulopathy
and
bruises
,
resulting
in
death
in
six
months
.
Papular
biopsy
revealed
multiple
microinfarcts
in
the
dermal
collagen
.
The
brain
showed
multiple
small
haemorrhagic
infarcts
of
both
hemispheres
,
the
lower
mid-
brain
,
pons
and
a
cerebellar
peduncle
.
Microscopy
showed
fibrin
exudation
in
the
leptomeninges
,
various
stages
of
thrombosis
of
small
arteries
without
inflammatory
reaction
,
and
corresponding
acute
and
subacute
microinfarcts
.
The
pathogenetic
basis
of
the
disease
appeared
to
be
a
combined
vasculopathy
and
coagulopathy
.
Diseases
Validation
Diseases presenting
"small arteries"
symptom
cadasil
hereditary cerebral hemorrhage with amyloidosis
malignant atrophic papulosis
sneddon syndrome
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