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Dyschromia related to severe combined immunodeficiency.
[omenn syndrome]
Severe
combined
immunodeficiency
includes
a
group
of
diseases
characterized
by
different
inherited
immunological
defects
.
A
4
-
month
-old
girl
diagnosed
with
Omenn
syndrome
,
a
subtype
of
severe
combined
immunodeficiency
presenting
with
generalized
erythroderma
,
was
referred
to
our
hospital
for
an
allogeneic
stem
cell
transplantation
.
Days
before
transplantation
,
she
developed
hyperpigmented
macules
that
increased
in
number
in
the
following
months
.
As
the
erythroderma
resolved
after
transplantation
,
diffuse
hypopigmentation
was
simultaneously
noted
together
with
the
expansion
of
hyperpigmented
lesions
.
Cutaneous
biopsy
samples
were
taken
at
different
moments
,
showing
features
of
Omenn
syndrome
at
first
,
and
2
months
later
changes
consistent
with
hypopigmentation
and
repigmentation
were
observed
.
Although
pigmentary
disorders
are
rarely
described
in
this
context
,
these
must
be
taken
into
account
as
a
possible
alternative
diagnosis
to
graft-versus-host
disease
and
toxicoderma
in
immunosuppressed
patients
.
Diseases
Validation
Diseases presenting
"a subtype of severe combined immunodeficiency presenting with generalized erythroderma"
symptom
omenn syndrome
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