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Evaluation of wound care options in patients with recessive dystrophic epidermolysis bullosa: a costly necessity.
[dystrophic epidermolysis bullosa]
Recessive
dystrophic
epidermolysis
bullosa
(
RDEB
)
is
a
genetic
disorder
in
which
mutations
in
collagen
VII
,
the
main
component
of
the
anchoring
fibril
,
lead
to
skin
fragility
and
to
the
development
of
acute
and
chronic
wounds
.
Wound
care
and
dressing
changes
are
an
important
part
of
the
daily
lives
of
individuals
with
RDEB
.
Ideal
wound
care
should
improve
wound
healing
,
minimize
pain
,
and
improve
quality
of
life
.
The
objective
of
the
current
study
was
to
review
wound
care
options
that
might
be
used
in
a
patient
with
RDEB
and
calculate
the
cost
of
these
various
options
based
on
publicly
available
pricing
of
wound
care
products
.
There
is
a
wide
range
of
costs
for
wound
care
options
in
patients
with
RDEB
.
For
example
,
a
1
-
day
supply
of
dressing
for
a
neonate
boy
with
RDEB
ranges
from
$
10
.
64
for
the
least
expensive
option
to
$
127
.
54
for
the
most
expensive
option
.
Wound
care
in
patients
with
severe
,
generalized
RDEB
has
not
only
a
significant
economic
effect
,
but
also
directly
affects
quality
of
life
in
this
patient
population
.
Although
randomized
controlled
trials
evaluating
different
wound
care
products
in
patients
with
RDEB
are
lacking
,
small
studies
and
expert
opinion
support
the
use
of
specialized
nonadherent
dressings
that
minimize
skin
trauma
and
promote
wound
healing
.
Until
there
is
a
cure
,
prospective
studies
are
needed
to
assess
pain
,
quality
of
life
,
and
wound
healing
associated
with
the
use
of
specialized
wound
care
products
for
this
life-altering
condition
.
Diseases
Validation
Diseases presenting
"pain"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
aniridia
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital diaphragmatic hernia
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentin dysplasia
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
focal myositis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
neuralgic amyotrophy
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
phenylketonuria
pleomorphic liposarcoma
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
trochlear dysplasia
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wolf-hirschhorn syndrome
This symptom has already been validated
