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Noninfectious cutaneous granulomas in primary immunodeficiency disorders: report from a national registry.
[omenn syndrome]
:
The
association
of
noninfectious
cutaneous
granulomas
with
primary
immunodeficiency
disorders
(
PIDs
)
is
a
rare
but
well-recognized
phenomenon
.
With
the
recent
advent
of
new
classification
and
broadening
of
the
list
of
PIDs
,
there
is
now
ever-growing
number
of
PIDs
having
being
reported
with
noninfectious
cutaneous
granulomas
or
granulomatous
tissue
reactions
.
The
authors
observed
4
patients
with
cutaneous
granulomas
associated
with
PIDs
that
constitute
2
%
of
total
PIDs
registered
with
them
.
In
this
report
,
the
authors
describe
these
4
patients
with
cutaneous
granulomas
/
granulomatous
skin
reactions
associated
one
each
with
common
variable
immunodeficiency
,
Omenn
syndrome
,
combined
immunodeficiency
,
and
Blau
syndrome
(
BS
)
,
and
briefly
review
the
literature
on
various
clinicopathological
patterns
of
cutaneous
granulomas
with
possible
underlying
pathogenetic
mechanisms
responsible
for
such
tissue
reactions
in
patients
with
PID
.
Diseases
Validation
Diseases presenting
"immunodeficiency"
symptom
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
cushing syndrome
dracunculiasis
hirschsprung disease
hodgkin lymphoma, classical
homocystinuria without methylmalonic aciduria
kabuki syndrome
legionellosis
malignant atrophic papulosis
oculocutaneous albinism
omenn syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
primary hyperoxaluria type 1
pyomyositis
severe combined immunodeficiency
sneddon syndrome
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated