Rare Diseases Symptoms Automatic Extraction
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Omenn syndrome: therapeutic effects of cyclosporin.
[omenn syndrome]
Omenn
syndrome
is
a
severe
combined
immunodeficiency
with
features
of
generalised
erythroderma
alopecia
and
evidence
of
Th
2
inflammation
(
eosinophilia
and
raised
IgE
)
.
We
describe
a
differential
effect
of
2
calcineurin
inhibitors
,
cyclosporine
A
(
CsA
)
and
tacrolimus
,
with
CsA
rapidly
improving
the
erythroderma
and
lymphocytosis
but
tacrolimus
having
little
effect
.
Diseases
Validation
Diseases presenting
"erythroderma"
symptom
child syndrome
cutaneous mastocytosis
harlequin ichthyosis
lamellar ichthyosis
omenn syndrome
This symptom has already been validated