Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Anakinra-responsive lichen planus in a woman with Erdheim-Chester disease: a therapeutic enigma.
[erdheim-chester disease]
Anakinra
is
a
recombinant
form
of
interleukin-
1
receptor
antagonist
.
It
is
the
drug
of
choice
for
Schnitzler
syndrome
and
cryopyrin-associated
periodic
syndromes
.
It
has
also
recently
been
demonstrated
to
have
activity
in
the
treatment
of
the
non-
Langerhans
cell
histiocytosis
known
as
Erdheim-
Chester
disease
.
To
describe
the
activity
of
anakinra
in
a
patient
with
co
-existing
lichen
planus
and
Erdheim-
Chester
disease
.
A
43
-
year
-old
woman
with
progressive
Erdheim-
Chester
disease
presented
for
management
of
her
night
sweats
and
chills
,
systemic
skeletal
bone
pain
,
and
neurologic
(
diabetes
insipidus
)
manifestations
.
She
also
had
widespread
cutaneous
lichen
planus
.
Anakinra
,
100
mg
subcutaneously
daily
,
was
initiated
for
the
treatment
of
her
Erdheim-
Chester
disease
.
Within
2
days
of
starting
anakinra
,
there
was
prompt
resolution
of
her
Erdheim-
Chester
disease
-related
symptoms
.
Subsequently
,
her
bone
pain
resolved
and
her
diabetes
insipidus
improved
.
Also
,
the
lichen
planus-associated
pruritus
rapidly
ceased
and
most
of
the
skin
lesions
improved
.
Our
experience
confirms
the
efficacy
of
anakinra
for
the
treatment
of
Erdheim-
Chester
disease
.
The
concomitant
improvement
of
her
lichen
planus
on
anakinra
suggests
that
this
agent
warrants
additional
study
in
this
disorder
.
