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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
.
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