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Progressive overgrowth of the cerebriform connective tissue nevus in patients with Proteus syndrome.
[proteus syndrome]
Proteus
syndrome
is
a
rare
overgrowth
disorder
that
almost
always
affects
the
skin
.
Our
purpose
was
to
evaluate
progression
of
skin
lesions
in
patients
with
Proteus
syndrome
.
Skin
findings
were
documented
in
36
patients
with
Proteus
syndrome
.
Progression
of
skin
lesions
in
16
of
these
patients
was
assessed
by
comparing
photographs
obtained
on
repeated
visits
for
an
average
total
duration
of
53
months
.
The
skin
lesion
most
characteristic
of
Proteus
syndrome
,
the
cerebriform
connective
tissue
nevus
,
showed
progression
in
13
children
but
not
in
3
adults
.
The
cerebriform
connective
tissue
nevus
progressed
by
expansion
into
previously
uninvolved
skin
,
increased
thickness
,
and
development
of
new
lesions
.
Lipomas
increased
in
size
,
number
,
or
both
in
8
of
10
children
with
lipomas
.
In
contrast
,
epidermal
nevi
and
vascular
malformations
generally
did
not
spread
or
increase
in
number
.
Only
3
adults
with
Proteus
syndrome
were
evaluated
longitudinally
.
The
cerebriform
connective
tissue
nevus
in
Proteus
syndrome
grows
throughout
childhood
but
tends
to
remain
stable
in
adulthood
.
Diseases
Validation
Diseases presenting
"skin lesions"
symptom
child syndrome
cowden syndrome
cutaneous mastocytosis
cystinuria
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
familial mediterranean fever
focal myositis
gm1 gangliosidosis
heparin-induced thrombocytopenia
hirschsprung disease
junctional epidermolysis bullosa
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
oligodontia
omenn syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
proteus syndrome
severe combined immunodeficiency
sneddon syndrome
waldenström macroglobulinemia
werner syndrome
wiskott-aldrich syndrome
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