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A systematic review of clinical trials of treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.
[lamellar ichthyosis]
The
ichthyoses
comprise
a
group
of
inherited
disorders
of
keratinization
.
Because
of
the
need
for
lifelong
treatment
,
it
is
important
that
therapies
are
beneficial
,
safe
,
and
well
tolerated
.
We
sought
to
review
the
evidence
on
existing
treatments
for
the
congenital
ichthyoses
,
excluding
ichthyosis
vulgaris
.
We
undertook
a
systematic
review
using
the
methodology
of
the
Cochrane
Collaboration
.
Articles
published
in
MEDLINE
,
EMBASE
,
and
CENTRAL
and
registered
clinical
trials
were
screened
.
Randomized
controlled
trials
involving
patients
with
the
inherited
ichthyoses
,
either
syndromic
or
nonsyndromic
but
excluding
ichthyosis
vulgaris
,
were
considered
.
Six
trials
met
the
inclusion
criteria
.
Topical
treatments
including
5
%
urea
,
20
%
propylene
glycol
alone
or
in
combination
with
5
%
lactic
acid
,
calcipotriol
ointment
,
and
liarozole
5
%
cream
showed
therapeutic
benefit
.
Oral
liarozole
,
a
retinoic
acid
metabolism
blocking
agent
,
showed
no
advantage
over
oral
acitretin
.
Most
studies
were
performed
on
a
small
sample
of
patients
and
lacked
methodological
and
reporting
quality
.
The
small
number
of
trials
and
the
nearly
constant
positive
results
make
publication
bias
likely
.
The
absence
of
standardization
of
outcome
measures
precluded
the
comparison
of
studies
.
Topical
treatments
including
emollients
,
calcipotriol
ointment
,
and
liarozole
cream
seem
to
have
therapeutic
benefit
and
a
good
safety
profile
,
although
the
use
of
topical
calcipotriol
is
limited
by
a
maximum
weekly
dose
of
100
g
.
The
advantage
of
oral
liarozole
over
acitretin
is
uncertain
.
Multicenter
trials
comparing
oral
and
topical
interventions
and
evaluation
of
long
-term
outcomes
are
needed
.
Diseases
Validation
Diseases presenting
"inherited disorders of keratinization"
symptom
lamellar ichthyosis
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