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Degos cutaneous disease with features of connective tissue disease.
[malignant atrophic papulosis]
A
30
-
year
-old
woman
was
referred
on
April
2002
for
a
plaque
that
involved
the
internal
aspect
of
the
right
leg
,
an
erythema
nodosum
-like
lesion
on
the
lower
extremities
,
and
periarthritis
on
her
left
ankle
.
Subsequently
,
the
patient
developed
anular
,
atrophic
,
growing
,
porcelain-
white
papules
,
with
a
thin
rim
of
erythema
and
telangiectases
over
her
upper
and
lower
extremities
.
Clinically
and
histologically
,
these
lesions
were
the
characteristics
of
Degos
disease
.
Despite
arthritis
and
myositis
that
required
treatment
,
low
level
C
3
and
C
4
,
positive
antinuclear
antibodies
,
and
elevated
anticardiolipin
antibodies
only
once
,
in
a
follow-up
of
6
years
the
patient
never
developed
a
specific
connective
tissue
disease
or
other
systemic
involvement
.
In
conclusion
,
because
clinical
and
histological
findings
of
Degos
disease
might
mimic
connective
tissue
diseases
,
rheumatologists
must
be
aware
that
this
reaction
pattern
can
be
seen
in
a
wide
clinical
spectrum
of
diseases
.
Diseases
Validation
Diseases presenting
"right leg"
symptom
focal myositis
malignant atrophic papulosis
neuralgic amyotrophy
oculocutaneous albinism
proteus syndrome
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