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Successful treatment with topical N-acetylcysteine in urea in five children with congenital lamellar ichthyosis.
[lamellar ichthyosis]
We
reported
the
efficacy
of
topical
cutaneous
N-
acetylcysteine
in
children
with
type
I
lamellar
ichthyosis
.
The
drug
was
applied
on
predetermined
body
surface
areas
two
times
a
day
for
6
weeks
,
followed
by
a
daily
maintenance
application
.
During
the
first
2
weeks
of
treatment
,
a
significant
improvement
occurred
.
After
4
months
of
maintenance
application
,
a
marked
overall
improvement
occurred
in
all
the
treated
areas
.
Only
two
patients
showed
mild
adverse
effects
such
as
light
burning
,
pruritus
,
and
irritation
.
Even
though
a
larger
group
of
patients
should
be
necessary
to
confirm
the
data
,
topical
10
%
N-
acetylcysteine
emulsion
prepared
in
urea
5
%
seems
to
be
a
valuable
and
safe
therapeutic
option
for
lamellar
ichthyosis
in
children
,
with
benefit
not
only
for
skin
lesions
but
also
for
ectropion
avoiding
a
surgical
procedure
.
Diseases
Validation
Diseases presenting
"ichthyosis"
symptom
child syndrome
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
harlequin ichthyosis
hirschsprung disease
junctional epidermolysis bullosa
kallmann syndrome
lamellar ichthyosis
This symptom has already been validated