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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
"first case"
symptom
achondroplasia
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
carcinoma of the gallbladder
child syndrome
cholangiocarcinoma
classical phenylketonuria
coats disease
cohen syndrome
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
esophageal adenocarcinoma
esophageal carcinoma
fabry disease
familial mediterranean fever
focal myositis
gm1 gangliosidosis
harlequin ichthyosis
hodgkin lymphoma, classical
homocystinuria without methylmalonic aciduria
junctional epidermolysis bullosa
kabuki syndrome
krabbe disease
lamellar ichthyosis
legionellosis
liposarcoma
locked-in syndrome
malignant atrophic papulosis
monosomy 21
neonatal adrenoleukodystrophy
oculocutaneous albinism
omenn syndrome
papillon-lefèvre syndrome
pendred syndrome
pleomorphic liposarcoma
primary effusion lymphoma
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
systemic capillary leak syndrome
thoracic outlet syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
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