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Quality of life in Swedish children with congenital ichthyosis.
[harlequin ichthyosis]
Congenital
ichthyosis
encompasses
a
large
group
of
keratinizing
disorders
with
widespread
scaling
and
a
variable
degree
of
erythema
.
Little
is
known
about
the
quality
of
life
in
children
with
congenital
ichthyosis
and
the
impact
of
the
disease
on
their
family
.
Fifteen
children
aged
5
-
16
years
with
lamellar
ichthyosis
,
Netherton
's
syndrome
,
epidermolytic
hyperkeratosis
or
Harlequin
ichthyosis
,
were
investigated
concerning
the
effect
of
their
ichthyosis
on
their
quality
of
life
.
This
was
measured
with
the
established
Children
's
Dermatology
Life
Quality
Index
(
CDLQI
)
,
and
the
Dermatitis
Family
Impact
questionnaire
(
DFI
)
modified
by
substituting
the
word
ichthyosis
for
eczema
.
The
questionnaires
covered
the
preceding
seven
days
and
each
had
a
maximum
score
of
30
:
the
higher
the
score
,
the
greater
the
Quality
of
Life
impairment
.
The
median
score
was
9
.
0
(
range
2
-
19
)
for
the
CDLQI
and
9
.
0
(
range
3
-
21
)
for
DFI
.
There
was
a
significant
correlation
between
the
DFI
and
the
CDLQI
scores
.
The
item
in
the
CDLQI
questionnaire
that
showed
the
highest
score
was
"
itchy
,
scratchy
,
sore
or
painful
skin
"
and
the
most
highly
scored
item
in
the
DFI
questionnaire
was
effect
on
"
housework
,
e
.
g
.
washing
,
cleaning
"
;
both
items
related
to
the
children
's
symptoms
.
The
results
of
the
study
clearly
establish
that
congenital
ichthyosis
impairs
the
quality
of
life
of
the
affected
children
and
their
families
.
