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[Systemic mastocytosis: bone impact].
[cutaneous mastocytosis]
Systemic
mastocytosis
is
a
disease
characterized
by
accumulation
of
mast
cells
in
various
organs
of
which
the
most
affected
is
the
skin
.
The
bone
impact
of
this
disease
is
very
rare
and
generally
associated
with
the
development
of
secondary
osteoporosis
with
or
without
fractures
.
We
present
three
cases
of
patients
with
skin
mastocytosis
lesions
and
different
bone
manifestations
;
the
first
case
was
a
51
year
old
woman
in
whom
we
observed
a
rare
sclerosing
variant
with
a
normal-
high
bone
density
and
increased
density
presented
in
several
vertebrae
x-rays
.
An
iliac
crest
biopsy
confirmed
bone
involvement
of
the
underlying
disease
.
The
second
case
was
a
57
year
old
woman
who
had
characteristic
signs
of
systemic
mastocytosis
with
diarrhea
,
gastritis
,
flushes
and
specific
cutaneous
reactions
to
exposure
to
allergens
.
She
also
presented
severe
decrease
in
bone
mineral
density
in
both
lumbar
spine
and
femoral
neck
,
with
biochemical
parameters
of
increased
bone
resorption
,
so
had
to
be
treated
with
bisphosphonates
.
The
third
patient
,
a
67
-
year
-old
woman
,
had
several
vertebral
fractures
,
with
slightly
decreased
bone
densitometry
.
This
last
patient
showed
a
hiperplaquetosis
with
histological
diagnosis
of
essential
thrombocythemia
,
a
picture
usually
associated
with
systemic
mastocytosis
.
In
conclution
,
we
present
and
discuss
three
different
bone
variants
of
systemic
mastocytosis
.
Diseases
Validation
Diseases presenting
"bone density"
symptom
achondroplasia
aromatase deficiency
congenital adrenal hyperplasia
cutaneous mastocytosis
erdheim-chester disease
fabry disease
familial hypocalciuric hypercalcemia
kallmann syndrome
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