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An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease).
[malignant atrophic papulosis]
A
new
case
of
malignant
atrophic
papulosis
(
Köhlmeier-
Degos
disease
)
is
reported
.
Vascular
symptoms
began
at
17
months
of
age
with
cerebral
ischaemia
and
progressive
involvement
of
fingers
and
toes
with
torpid
ulcers
and
apical
necrotic
amputations
.
At
6
years
of
age
he
developed
chronic
intestinal
ischaemia
with
malabsorption
and
a
new
cerebral
attack
;
in
spite
of
anti-aggregant
therapy
the
disease
progressed
and
he
died
7
months
after
diagnosis
from
a
third
cerebral
ictus
.
Since
the
typical
skin
lesions
of
the
disease
were
absent
,
the
diagnosis
was
made
on
the
basis
of
a
pathological
pattern
of
an
occluded
biopsied
artery
.
The
elder
brother
presents
clinical
and
instrumental
vascular
involvement
without
cutaneous
lesions
and
could
be
slightly
affected
.
Diseases
Validation
Diseases presenting
"malabsorption"
symptom
cystinuria
homocystinuria without methylmalonic aciduria
lamellar ichthyosis
malignant atrophic papulosis
neonatal adrenoleukodystrophy
This symptom has already been validated
