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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
"blood vessels"
symptom
cadasil
coats disease
cushing syndrome
esophageal adenocarcinoma
hereditary cerebral hemorrhage with amyloidosis
hydrocephalus with stenosis of the aqueduct of sylvius
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
oral submucous fibrosis
pyomyositis
sneddon syndrome
von hippel-lindau disease
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