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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
"weakness"
symptom
achondroplasia
adrenomyeloneuropathy
alexander disease
aniridia
aromatase deficiency
coats disease
congenital toxoplasmosis
epidermolysis bullosa simplex
esophageal adenocarcinoma
familial hypocalciuric hypercalcemia
familial mediterranean fever
focal myositis
gm1 gangliosidosis
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
neuralgic amyotrophy
pendred syndrome
pleomorphic liposarcoma
pyomyositis
pyruvate dehydrogenase deficiency
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
triple a syndrome
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
werner syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated