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[Malignant atrophic papulosis (Köhlmeier-Degos disease)].
[malignant atrophic papulosis]
On
the
basis
of
106
patients
with
malignant
atrophic
papulosis
in
the
literature
,
including
one
case
of
our
own
,
the
clinical
,
histopathological
and
nosological
features
of
this
disease
are
reviewed
.
Histopathologically
,
the
typical
papules
with
central
porcelain-
white
atrophy
show
broad
wedge-shaped
necrosis
of
the
connective
tissue
due
to
thrombotic
occlusions
of
small
vessels
in
the
corium
.
The
disease
leads
to
death
in
50
%
of
these
cases
,
mostly
due
to
involvement
of
the
gut
and
the
central
nervous
system
.
Malignant
atrophic
papulosis
is
the
prototype
of
a
disease
,
in
which
skin
lesions
are
the
primary
sign
of
a
systemic
disorder
demanding
the
interdisciplinary
cooperation
of
various
specialists
.
Diseases
Validation
Diseases presenting
"skin lesions"
symptom
child syndrome
cowden syndrome
cutaneous mastocytosis
cystinuria
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
familial mediterranean fever
focal myositis
gm1 gangliosidosis
heparin-induced thrombocytopenia
hirschsprung disease
junctional epidermolysis bullosa
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
oligodontia
omenn syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
proteus syndrome
severe combined immunodeficiency
sneddon syndrome
waldenström macroglobulinemia
werner syndrome
wiskott-aldrich syndrome
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