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Keratolysis exfoliativa (dyshidrosis lamellosa sicca): a distinct peeling entity.
[epidermolysis bullosa simplex]
Keratolysis
exfoliativa
(
KE
)
,
also
known
as
dyshidrosis
lamellosa
sicca
,
is
a
palmoplantar
dermatosis
characterized
by
air-filled
blisters
and
collarette
desquamation
.
It
has
been
regarded
as
a
subtype
of
dyshidrotic
eczema
,
a
fungal
infection
or
a
dermatophytid
reaction
.
KE
may
also
resemble
acral
peeling
skin
syndrome
and
localized
epidermolysis
bullosa
simplex
.
Although
KE
is
a
common
disorder
,
it
is
a
rarely
reported
and
is
an
under-recognized
dermatosis
.
To
delineate
the
characteristic
features
of
KE
.
We
investigated
the
clinical
,
immunohistopathological
,
ultrastructural
and
molecular
features
of
KE
.
Patients
were
included
from
the
clinical
records
.
Additional
diagnostic
research
consisted
of
mutation
analysis
of
the
candidate
genes
TGM
5
,
KRT
5
,
KRT
14
,
FLG
,
SPINK
6
and
SPINK
9
.
A
total
of
24
patients
with
KE
were
identified
,
six
with
familial
and
18
with
sporadic
KE
.
Lesions
consisted
of
air-filled
blisters
only
on
palmoplantar
skin
,
followed
by
collarette
and
lamellar
peeling
.
Both
light
microscopy
and
electron
microscopy
showed
cleavage
and
partially
degraded
corneodesmosomes
within
the
stratum
corneum
,
whereas
immunofluorescence
microscopy
showed
normal
expression
of
corneodesmosomal
components
.
No
mutations
were
found
in
TGM
5
,
KRT
5
/
14
and
SPINK
6
/
9
.
There
was
no
clear
link
with
atopy
or
with
FLG
mutations
.
O
ur
study
suggests
premature
corneodesmolysis
as
the
main
pathological
mechanism
of
this
palmoplantar
skin
disorder
.
We
conclude
that
KE
appears
to
be
a
distinct
peeling
entity
.
Diseases
Validation
Diseases presenting
"common disorder"
symptom
aniridia
congenital adrenal hyperplasia
cutaneous mastocytosis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
familial hypocalciuric hypercalcemia
sneddon syndrome
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