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A child with laryngo-onychocutaneous syndrome partially responsive to treatment with thalidomide.
[junctional epidermolysis bullosa]
Laryngo-onychocutaneous
syndrome
(
LOCS
)
is
a
condition
characterized
by
erosive
or
ulcerative
skin
lesions
associated
with
excessive
granulation
tissue
,
at
sites
of
trauma
such
as
the
digits
,
elbows
and
knees
.
Similar
lesions
can
occur
within
the
conjunctival
mucosa
,
leading
to
corneal
scarring
and
blindness
.
The
main
complications
,
however
,
occur
in
the
respiratory
tract
,
where
a
similar
process
of
erosions
and
subsequent
formation
of
granulation
tissue
causes
airway
obstruction
which
may
lead
to
premature
death
.
LOCS
is
now
believed
to
be
a
nonblistering
variant
of
junctional
epidermolysis
bullosa
and
to
date
there
are
no
efficacious
treatments
available
.
We
report
a
16
-
year
-old
girl
with
LOCS
who
failed
to
respond
to
methylprednisolone
and
cyclophosphamide
,
but
had
a
partial
response
to
oral
thalidomide
with
marked
decrease
in
granulation
tissue
and
tracheal
secretions
.
Interruption
of
treatment
resulted
in
prompt
resurgence
of
the
granulation
tissue
which
was
again
controlled
by
reintroduction
of
thalidomide
.
We
propose
that
in
the
absence
of
effective
therapies
for
LOCS
,
a
trial
of
thalidomide
in
these
patients
should
be
considered
.
