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[Benign familial Degos disease].
[malignant atrophic papulosis]
Degos
'
disease
or
atrophic
malignant
papulosis
is
defined
by
porcelain
white
cutaneous
lesions
with
atrophic
scarring
,
often
associated
with
severe
and
fatal
systemic
involvement
(
visceral
and
neurological
)
.
Benign
forms
are
rare
or
under-reported
and
the
familial
forms
are
exceptional
.
It
is
a
very
rare
disease
,
only
two
hundred
cases
have
been
reported
in
the
literature
with
a
sex
ratio
of
3
M
/
1
F
.
The
pathogenesis
of
Degos
'
disease
remains
controversial
.
The
exceptional
observation
of
familial
form
raises
the
question
of
a
genetic
predisposition
of
this
disease
or
an
infectious
aetiology
with
a
low
virus
.
A
41
year
-old
woman
was
known
to
have
Degos
'
disease
for
26
years
with
only
cutaneous
manifestations
.
One
of
her
two
sons
developed
atrophic
cutaneous
lesions
at
the
age
of
20
.
In
both
patients
,
no
thrombotic
or
immunological
abnormalities
were
found
.
The
karyotype
was
performed
with
normal
results
.
Degos
'
disease
or
malignant
atrophic
papulosis
can
have
a
long
lasting
benign
evolution
.
Our
patient
,
who
had
presented
a
benign
form
for
26
years
,
had
the
longest
evolution
ever
documented
in
literature
.
We
can
not
be
sure
that
her
son
will
have
a
benign
course
of
his
Degos
'
disease
because
the
diagnosis
is
recent
and
because
the
systemic
involvement
can
appear
after
many
years
of
evolution
.
In
the
familial
forms
,
from
our
study
and
the
31
cases
previously
described
in
the
literature
,
with
ten
different
families
,
the
course
of
the
disease
seems
to
be
less
severe
than
in
sporadic
forms
.
Among
these
familial
forms
of
Degos
'
disease
,
only
4
patients
presented
a
malignant
form
,
which
in
one
case
did
not
prove
a
relationship
between
the
death
and
the
Degos
'
disease
.
Are
the
sporadic
forms
with
only
skin
involvement
less
frequently
reported
?
Has
the
familial
form
of
Degos
'
disease
the
same
course
as
a
very
severe
common
sporadic
form
?
Diseases
Validation
Diseases presenting
"often associated with severe and fatal systemic involvement"
symptom
malignant atrophic papulosis
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