Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Clinical and histologic findings in Degos' syndrome (malignant atrophic papulosis).
[malignant atrophic papulosis]
Records
of
nine
patients
(
aged
ten
to
sixty-
four
years
)
seen
at
the
Mayo
Clinic
with
the
typical
cutaneous
lesions
of
Degos
'
syndrome
were
reviewed
.
The
three
patients
who
died
all
had
central
nervous
system
involvement
.
The
six
patients
now
alive
have
been
evaluated
for
two
to
fourteen
years
and
have
had
their
disease
for
four
to
fourteen
years
.
Histopathologic
examination
was
performed
in
all
nine
cases
(
total
of
27
skin
biopsy
specimens
)
.
Wedge-shaped
infarction
in
the
dermis
and
subcutaneous
tissue
was
observed
only
in
one
biopsy
specimen
from
each
of
three
patients
,
and
the
infarction
occurred
in
older
,
well-formed
skin
lesions
.
Thrombosis
of
the
arterioles
was
found
in
two
patients
.
Hyperkeratosis
and
dermal
acid
mucopolysaccharide
deposits
were
common
.
The
most
consistent
histopathologic
finding
was
lymphocytic
infiltrate
around
and
in
the
walls
of
venules
and
arterioles
.
Various
degrees
of
lymphocyte-mediated
necrotizing
vasculitis
were
present
in
all
patients
.
Diseases
Validation
Diseases presenting
"vasculitis"
symptom
coats disease
dracunculiasis
dystrophic epidermolysis bullosa
erdheim-chester disease
erythropoietic protoporphyria
familial mediterranean fever
focal myositis
inclusion body myositis
malignant atrophic papulosis
papillon-lefèvre syndrome
pyruvate dehydrogenase deficiency
scrub typhus
sneddon syndrome
typhoid
waldenström macroglobulinemia
This symptom has already been validated
