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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
"osteoporosis"
symptom
achondroplasia
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
aromatase deficiency
congenital adrenal hyperplasia
cushing syndrome
cutaneous mastocytosis
dentinogenesis imperfecta
erythropoietic protoporphyria
fabry disease
familial hypocalciuric hypercalcemia
familial mediterranean fever
inclusion body myositis
kallmann syndrome
oligodontia
pyomyositis
werner syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated