Extramammary
Paget's
disease
Extramammary
Paget’s
disease (EMPD),
also
Extramammary
Paget
disease, is
a usually
non-invasive
adenocarcinoma
of the skin
outside of
the mammary
gland and
includes
Paget's
disease of
the vulva
and Paget's
disease of
the penis.
The origin
of the
neoplastic
cells could
be apocrine
glands or
epithelial
stem cells.
Skin areas
rich in
apocrine
glands such
as the
genital
region are
typical
sites of
EMPD.
Signs and
symptoms are
a skin
lesion often
mistaken as
an eczema
that may be
itchy or
painful. A
biopsy will
establish
the
diagnosis.
The
histology of
the lesion
is the same
as for
Paget's
disease of
the breast.
Paget's
disease of
the vulva
may be a
primary
lesion or
associated
with
adenocarcinoma
originating
from local
organs such
as the
Bartholin
gland, the
urethra, or
the rectum
and thus be
secondary.
Patients
tend to be
in the
postmenopausal
range.
Paget's
disease of
the penis
may also be
primary or
secondary
and is less
common than
genital
Paget’s
disease in
women.
It is
important to
exclude that
the lesion
is
associated
with another
cancer.
Primary
disease is
usually
treated by
surgical
excision.
History
James Paget
had
described
Paget
disease of
the nipple
in 1874.
Radcliffe
Crocker
reported the
first case
of EMPD in
1889 when he
described a
patient with
a skin
lesion
affecting
the penis
and scrotum
the findings
of which
were
identical to
those
described by
Paget. EMPD
is found in
a number of
locations
where
apocrine
glands are
common.
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