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Degos syndrome: malignant atrophic papulosis.
[malignant atrophic papulosis]
Malignant
atrophic
papulosis
is
a
rare
disorder
characterized
by
pathognomonic
cutaneous
lesions
that
have
been
associated
with
multiple
infarctive
thrombotic
lesions
of
other
viscera
,
most
notably
the
gastrointestinal
tract
and
the
central
nervous
system
.
Systemic
involvement
may
develop
from
weeks
to
years
after
the
onset
of
the
characteristic
cutaneous
lesions
or
,
rarely
,
may
precede
the
cutaneous
lesions
.
However
,
the
existence
of
patients
with
a
prolonged
,
purely
cutaneous
variant
of
this
disease
has
been
increasingly
appreciated
,
and
this
brings
into
question
the
appropriateness
of
applying
the
term
"
malignant
"
to
all
patients
who
have
the
peculiar
characteristic
cutaneous
lesions
of
malignant
atrophic
papulosis
.
Despite
half
a
century
of
sporadic
investigation
,
the
precise
cause
of
this
disease
remains
unknown
,
and
accurate
classification
of
this
entity
as
a
primary
vasculopathy
or
primary
coagulopathy
has
not
been
possible
.
Unfortunately
,
no
effective
therapy
exists
for
those
patients
in
whom
systemic
involvement
develops
.
Diseases
Validation
Diseases presenting
"peculiar characteristic cutaneous lesions"
symptom
malignant atrophic papulosis
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