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Erdheim-Chester disease: a comprehensive review.
[erdheim-chester disease]
Erdheim-
Chester
disease
is
a
rare
form
of
non-
Langerhans
'
cell
histiocytosis
characterized
by
multi-system
infiltration
by
xanthogranulomas
composed
of
foamy
histiocytes
surrounded
by
fibrosis
.
Approximately
400
cases
have
been
reported
in
the
literature
,
and
the
recent
increase
in
the
number
of
cases
is
likely
due
to
the
increased
awareness
of
its
associated
morbidity
and
mortality
.
The
etiology
of
this
disease
remains
unknown
,
the
clinical
course
is
variable
and
treatment
is
still
not
well-established
.
The
objective
of
this
review
is
to
describe
the
pathogenesis
,
clinical
manifestations
,
and
diagnosis
of
this
rare
disorder
,
and
to
review
its
prognosis
and
treatment
.
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
form
of
non-
Langerhans
'
cell
histiocytosis
.
It
was
first
described
in
1930
.
Approximately
400
cases
have
been
reported
in
the
literature
.
