Rare Diseases Symptoms Automatic Extraction
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Case for diagnosis.
[malignant atrophic papulosis]
Degos
disease
,
also
known
as
malignant
atrophic
papulosis
,
is
a
rare
occlusive
vasculopathy
of
unknown
etiology
characterized
by
infarcts
in
the
dermis
,
gastrointestinal
tract
,
central
nervous
system
,
and
other
organs
.
It
is
characterized
by
papules
,
which
become
umbilicated
and
evolve
with
a
depressed
porcelain-
white
central
area
,
with
an
erythematous
halo
with
telangiectasias
.
Histological
findings
include
wedge-shaped
dermoepidermal
necrosis
and
blood
vessel
thrombosis
.
Approximately
50
-
60
%
of
patients
with
systemic
symptoms
die
within
2
-
3
years
,
most
due
to
gastrointestinal
perforation
.
We
report
a
typical
case
,
with
lethal
outcome
,
in
a
45
-
year
-old
woman
.
Diseases
Validation
Diseases presenting
"central nervous system"
symptom
22q11.2 deletion syndrome
adrenomyeloneuropathy
alexander disease
aniridia
aromatase deficiency
canavan disease
child syndrome
classical phenylketonuria
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cystinuria
dracunculiasis
erdheim-chester disease
fabry disease
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
hodgkin lymphoma, classical
kabuki syndrome
kallmann syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
legionellosis
liposarcoma
malignant atrophic papulosis
monosomy 21
neonatal adrenoleukodystrophy
phenylketonuria
proteus syndrome
scrub typhus
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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