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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
"vasculitis"
symptom
coats disease
dracunculiasis
dystrophic epidermolysis bullosa
erdheim-chester disease
erythropoietic protoporphyria
familial mediterranean fever
focal myositis
inclusion body myositis
malignant atrophic papulosis
papillon-lefèvre syndrome
pyruvate dehydrogenase deficiency
scrub typhus
sneddon syndrome
typhoid
waldenström macroglobulinemia
This symptom has already been validated