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Malignant atrophic papulosis (Köhlmeier-Degos disease) - a review.
[malignant atrophic papulosis]
DEFINITION
OF
THE
DISEASE
:
Malignant
atrophic
papulosis
(
MAP
)
,
described
independently
by
Köhlmeier
and
Degos
et
al
.
,
is
a
rare
,
chronic
,
thrombo-obliterative
vasculopathy
characterized
by
papular
skin
lesions
with
central
porcelain-
white
atrophy
and
surrounding
teleangiectatic
rim
.
Less
than
200
cases
have
been
described
in
the
literature
.
The
first
manifestation
of
MAP
usually
occurs
between
the
20
th
and
50
th
year
of
life
.
The
cutaneous
clinical
picture
is
almost
pathognomonic
.
The
histology
is
not
consistent
but
in
most
cases
it
shows
a
wedge-shaped
connective
tissue
necrosis
in
the
deep
corium
due
to
a
thrombotic
occlusion
of
the
small
arteries
.
In
the
systemic
variant
,
manifestations
mostly
occur
at
the
intestine
and
central
nervous
system
.
The
etiopathogenesis
of
the
disease
remains
unknown
,
a
genetic
predisposition
may
occur
.
Vasculitis
,
coagulopathy
or
primary
dysfunction
of
the
endothelial
cells
have
been
implicated
.
Diagnosis
is
only
based
on
the
characteristic
skin
lesions
.
DIFFERRENTIAL
DIAGNOSIS
:
It
depends
on
the
clinical
presentation
of
MAP
,
but
systemic
lupus
erythematosus
and
other
connective
tissue
diseases
need
to
be
considered
.
No
effective
treatment
exists
for
the
systemic
manifestations
,
while
compounds
that
facilitate
blood
perfusion
have
achieved
a
partial
regression
of
the
skin
lesions
in
single
cases
.
An
apparently
idiopathic
,
monosymptomatic
,
cutaneous
,
benign
variant
and
a
progressive
,
visceral
one
with
approx
.
50
%
lethality
within
2
-
3
years
have
been
reported
.
Systemic
manifestations
can
develop
years
after
the
occurrence
of
skin
lesions
leading
to
bowel
perforation
and
peritonitis
,
thrombosis
of
the
cerebral
arteries
or
massive
intracerebral
hemorrhage
,
meningitis
,
encephalitis
,
radiculopathy
,
myelitis
.
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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