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Degos disease with dermatomyositis-like phenomenon: a diagnostic dilemma and a therapeutic challenge.
[malignant atrophic papulosis]
Degos
disease
is
a
type
of
vasculopathy
characterized
by
progressive
occlusion
of
small
-sized
blood
vessels
primarily
involving
the
skin
,
gastrointestinal
system
,
and
central
nervous
system
as
well
as
various
other
systems
.
Owing
to
the
rarity
of
the
condition
,
the
diagnosis
is
often
a
challenge
;
consequently
,
management
is
even
more
difficult
owing
to
the
paucity
of
experience
and
literature
for
the
effective
treatment
of
this
entity
.
We
report
a
case
of
a
50
-
year
-old
male
patient
with
classic
skin
lesions
and
rapidly
progressive
fatal
clinical
course
involving
multiple
organs
associated
with
dermatomyositis
-like
features
.
Therapeutic
challenge
in
such
a
case
of
secondary
Degos
disease
with
multiorgan
dysfunction
is
discussed
.
Secondary
Degos
disease
with
underlying
multisystem
dysfunction
is
a
rapidly
fatal
disease
.
The
patient
must
be
thoroughly
investigated
to
watch
for
systemic
involvement
and
aggressively
treated
preferably
with
antiplatelet
drugs
and
immunosuppresants
so
as
to
moderate
the
severity
of
the
disease
.
Corticosteroids
are
best
avoided
to
prevent
gastrointestinal
perforation
.
Diseases
Validation
Diseases presenting
"rapidly fatal disease"
symptom
malignant atrophic papulosis
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