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[Case of disseminated intestinal and cutaneous thromboangiitis (Kohlmeier-Degos' syndrome)].
[malignant atrophic papulosis]
Disseminated
intestinal
and
cutaneous
thromboangiitis
(
Kohlmeier-
Degos
'
syndrome
)
is
a
rare
syndrome
of
unknown
etiology
,
which
is
characterized
by
cutaneous
and
visceral
involvement
.
Two
(
cutaneous
and
visceral
)
stages
are
identified
.
A
fatal
outcome
is
inevitable
although
there
may
be
a
long
(
8
-
20
-
year
)
course
of
the
disease
during
a
benign
process
.
The
causes
of
death
are
peritonitis
,
central
nervous
system
impairments
,
pleurisy
,
pericarditis
.
The
paper
describes
a
case
of
disseminated
intestinal
and
cutaneous
thromboangiitis
(
Kohlmeier-
Degos
'
syndrome
)
in
a
32
-
year
-old
female
patient
who
has
died
from
intoxication
due
to
severely
impaired
permeability
of
the
epithelial
barrier
mainly
of
the
large
bowel
(
diphtheritic
inflammation
of
the
mucosa
,
severe
inflammatory
edema
of
the
submucous
layer
,
and
fibrinous
impregnation
of
the
serous
membrane
)
.
Diseases
Validation
Diseases presenting
"year-old female patient"
symptom
adrenal incidentaloma
aniridia
benign recurrent intrahepatic cholestasis
cowden syndrome
cushing syndrome
epidermolysis bullosa simplex
esophageal squamous cell carcinoma
holt-oram syndrome
krabbe disease
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
oral submucous fibrosis
proteus syndrome
pyomyositis
sneddon syndrome
thoracic outlet syndrome
triple a syndrome
von hippel-lindau disease
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