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Prevalence of inherited ichthyosis in France: a study using capture-recapture method.
[lamellar ichthyosis]
Inherited
ichthyoses
represent
a
group
of
rare
skin
disorders
characterized
by
scaling
,
hyperkeratosis
and
inconstant
erythema
,
involving
most
of
the
tegument
.
Epidemiology
remains
poorly
described
.
This
study
aims
to
evaluate
the
prevalence
of
inherited
ichthyosis
(
excluding
very
mild
forms
)
and
its
different
clinical
forms
in
France
.
Capture
-
recapture
method
was
used
for
this
study
.
According
to
statistical
requirements
,
3
different
lists
(
reference
/
competence
centres
,
French
association
of
patients
with
ichthyosis
and
internet
network
)
were
used
to
record
such
patients
.
The
study
was
conducted
in
5
areas
during
a
closed
period
.
The
prevalence
was
estimated
at
13
.
3
per
million
people
(
/
M
)
(
CI
95
%
,
[
10
.
9
-
17
.
6
]
)
.
With
regard
to
autosomal
recessive
congenital
ichthyosis
,
the
prevalence
was
estimated
at
7
/
M
(
CI
95
%
[
5
.
7
-
9
.
2
]
)
,
with
a
prevalence
of
lamellar
ichthyosis
and
congenital
ichthyosiform
erythroderma
of
4
.
5
/
M
(
CI
95
%
[
3
.
7
-
5
.
9
]
)
and
1
.
9
/
M
(
CI
95
%
[
1
.
6
-
2
.
6
]
)
,
respectively
.
Prevalence
of
keratinopathic
forms
was
estimated
at
1
.
1
/
M
(
CI
95
%
[
0
.
9
-
1
.
5
]
)
.
Prevalence
of
syndromic
forms
(
all
clinical
forms
together
)
was
estimated
at
1
.
9
/
M
(
CI
95
%
[
1
.
6
-
2
.
6
]
)
.
Our
results
constitute
a
crucial
basis
to
properly
size
the
necessary
health
measures
that
are
required
to
improve
patient
care
and
design
further
clinical
studies
.