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Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever.
[familial mediterranean fever]
Familial
Mediterranean
Fever
(
FMF
)
is
a
hereditary
autoinflammatory
disease
,
which
is
characterized
by
recurrent
and
paroxysmal
fever
,
peritonitis
,
arthritis
,
myalgia
,
and
skin
rashes
.
Although
various
skin
lesions
such
as
"
erysipelas
-like
erythema
"
,
urticaria
,
nonspecific
purpura
,
and
subcutaneous
nodules
have
been
described
,
cutaneous
vasculitis
is
rare
.
We
report
a
Japanese
case
of
sporadic
FMF
accompanied
by
cutaneous
arteritis
at
the
time
of
febrile
attacks
of
FMF
.
Gene
analysis
revealed
M
694
I
mutation
in
a
single
allele
of
the
MEFV
gene
,
and
oral
colchicine
successfully
controlled
both
periodic
fever
and
subcutaneous
nodules
of
arteritis
.
Cutaneous
necrotizing
vasculitis
repeatedly
emerging
with
febrile
attacks
should
be
included
among
the
skin
manifestations
of
FMF
.
Diseases
Validation
Diseases presenting
"skin manifestations"
symptom
child syndrome
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
familial mediterranean fever
harlequin ichthyosis
inclusion body myositis
kindler syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
sneddon syndrome
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