Vulvar Paget Disease of Urothelial Origin:
A Report of Three Cases and a Proposed
Classification of Vulvar Paget Disease
EDWARD J. WILKINSON, MD, AND HEATHER M. BROWN, MD
Extramammary Paget disease is generally considered a distinct
entity that can involve the genital tract skin and may be associated
with underlying adenocarcinoma. Evidence is presented that vulvar
Paget disease represents a heterogeneous group of epithelial neo-
plasms that can be similar both clinically and histopathologically.
Three cases of vulvar Paget-like disease that were manifestations of
urothelial carcinoma are investigated. Vulvar Paget disease can be
classified based on the origin of the neoplastic Paget cells as either
primary (of cutaneous origin) or secondary (of noncutaneous origin).
Each classification has 3 subtypes: primary, intraepithelial cutaneous
Paget disease of the usual type; intraepithelial cutaneous Paget dis-
ease with invasion, and intraepithelial cutaneous Paget disease as a
manifestation of underlying skin appendage adenocarcinoma; sec-
ondary, Paget disease of anorectal origin, Paget disease of urothelial
origin, and Paget disease of other origin. This subclassification is
based on a review of the literature and the current study of 3 patients
Paget disease has traditionally been divided into
mammary and extramammary disease. Mammary Paget
disease is almost always associated with underlying
breast neoplasia and represents a cutaneous manifesta-
tion of underlying ductal neoplasia.1,2
Extramammary Paget disease (EMPD) , as first
described by Crocker in 1888, was reported in a man
who had bladder carcinoma and presented with an
eczematous lesion of the scrotum and penis, which was
interpreted as Paget disease.3 EMPD most commonly
involves the external female genitalia and less com-
monly, the perianal region. However, EMPD has also
been reported in the perineum, axilla, umbilicus,
groin, eyelids, and external ear canal.4-7 All of these
regions share the characteristic of being rich in apo-
crine glands.5 Toker cells, the cells of origin of clear-
cell papulosis, have also been considered a possible
origin of Paget cells.6
EMPD can be further subdivided into primary and
secondary disease. Primary (cutaneous) EMPD is de-
fined as an intraepithelial adenocarcinoma arising
within the epidermis and extending into the contigu-
ous epithelium of skin appendages. Less commonly, it
arises from underlying skin appendages, usually from
the apocrine glands, and involves the overlying epider-
mis by epidermotropism. Focal invasion of intraepithe-
From the Department of Pathology, Immunology and Labora-
tory Medicine, University of Florida College of Medicine, Gainesville,
FL.
Address correspondence and reprint requests to Edward J.
Wilkinson, MD, Department of Pathology, University of Florida, P.O.
Box 100275, 1600 SW Archer Rd, Gainesville, FL 32610.
Copyright 2002, Elsevier Science (USA). All rights reserved.
0046-8177/02/3305-0015$35.00/0
doi:10.1053/hupa.2002.124788
549
with Paget-like disease of urothelial neoplastic origin. The 3 subtypes
of vulvar Paget disease studied here can present similarly as eczema-
toid skin or vulvar mucosal lesions and may appear similar on routine
hematoxylin and eosin–stained slides. Immunohistochemical studies
can be used to help differentiate them. The distinction between these
3 types of Paget-like lesions is essential in that the specific diagnosis
has a significant influence on current treatment. The difference in
surgical approach to the subtypes of vulvar Paget disease justifies
classifying them into distinct lesions to avoid potential confusion and
unnecessary surgery. HUM PATHOL 33:549-554. Copyright 2002,
Elsevier Science (USA). All rights reserved.
Key words: vulvar Paget disease, secondary Paget disease, immu-
nohistochemistry
Abbreviations: CK, cytokeratin; UP-III, uroplakin-III; GCDFP-15,
gross cystic disease fluid protein-15; CEA, carcinoembryonic antigen;
VIN, vulvar intraepithelial neoplasia.
lial vulvar Paget disease can occur, although this is a
relatively uncommon observation. Vulvar Paget disease
represents most cases of EMPD of cutaneous origin.8
Secondary vulvar EMPD is defined as involvement of
the vulvar skin by a noncutaneous internal neoplasm,
either by epidermotropic metastases or by direct exten-
sion. The most common neoplasm leading to second-
ary vulvar EMPD is anal or rectal adenocarcinoma in-
volving the skin of the anogenital region.9-11 This must
be distinguished from primary vulvar EMPD, which may
arise from the skin of the perianal region and may
secondarily involve the skin of the vulva. Approximately
20% of cases of primary EMPD arise in the perianal
region.8
Urothelial carcinoma (transitional cell carcinoma)
arising from the urothelium of the bladder or urethra
represents the second most common cause of second-
ary vulvar EMPD.9,12,13 Intraepithelial pagetoid spread
of urothelial carcinoma within the bladder is recog-
nized.14 The 3 cases reported herein demonstrate that
this intraepithelial spread of urothelial carcinoma can
extend to adjacent genital epithelium, where it may
simulate primary vulvar Paget disease of cutaneous or-
igin. In that the vulvar vestibule is the homologue of the
male distal urethra, that a urothelial neoplasm may
extend to the vulvar vestibule is not unexpected. Cases
of secondary vulvar EMPD due to adenocarcinoma of
the cervix, endometrium, and ovary also have been
reported.15
Based on our findings in this study, we propose a
classification of vulvar Paget disease to underscore the
importance of differentiating primary and secondary
genital Paget disease for treatment purposes (Table 1).
This classification is based on the etiologic origin of the
 HUMAN PATHOLOGY Volume 33, No. 5 (May 2002)

TABLE 1. Proposed Classification of Vulvar
Paget Disease
Primary vulvar Paget disease, a primary cutaneous neoplasm
Paget disease as a primary intraepithelial neoplasm
Paget disease as an intraepithelial neoplasm with invasion
Paget disease as a manifestation of an underlying primary
adenocarcinoma of a skin appendage or a subcutaneous vulvar
gland
Secondary vulvar Paget disease
Paget disease secondary to anal or rectal adenocarcinoma
Paget disease secondary to urothelial neoplasia
Paget disease secondary to adenocarcinomas or related tumors of
other sites
Paget-like cells identified within the vulvar skin and
mucosa.
We have previously reported a case of a 76-year-old
woman with a high-grade urothelial carcinoma in situ
of the bladder who was clinically thought to have a
primary vulvar Paget lesion (case 1). The pathology
proved to be intraepithelial urothelial neoplasia that
had spread to the vulva and presented as a Paget-like
lesion. For this lesion, we initially used the terms “pseu-
do-Paget disease”13 or “pagetoid urothelial intraepithe-
lial neoplasia (PUIN).”16 However, the term “secondary
Paget disease of urothelial origin” is descriptive and is
used in this report.
We present case 1 along with 2 additional cases of
vulvar EMPD secondary to urothelial carcinoma that we
have subsequently encountered in our study of this
process.
duit surgery, an inflammatory lesion of the vulva was
observed, and a tissue sample was obtained for biopsy.
The lesion was described clinically as velvety, red, and
completely circumscribing the left part of the labium
minus and extending to within 2.0 cm of the upper
vagina across the urethral orifice. Biopsies of the vulva
were reported at an outside institution as vulvar Paget
disease. A total vulvectomy was performed, encompass-
ing the visible lesion and extending to the underlying
deep fascia (Fig 2). The surgical resection specimen
had no underlying invasive carcinoma, and the surgical
margins of excision were free of disease. The case was
sent to our institution for consultation. Hematoxylin
and eosin–stained sections demonstrated tumor cells
within the vulvar squamous epithelium in nests and as
single cells, predominantly in the basal layer of the
epithelium. Individual tumor cells were as large or
larger than the adjacent keratinocytes. The tumor cells
had hyperchromatic nuclei but small nucleoli, and the
cytoplasm contained few vacuoles. Mitotic figures were
numerous. Immunohistochemical studies performed
on the vulvar resection specimen demonstrated cyto-
keratin 7 (CK 7) and uroplakin-III (UP-III) immunore-
activity. The tumor cells were not immunoreactive for
CK 20, gross cystic disease fluid protein-15 (GCDFP-

CASE REPORT
Case 1
The patient is a 76-year-old woman who was treated
at an outside institution 18 years earlier for carcinoma
in situ of the bladder (Fig 1). Treatment involved par-
tial cystectomy and intravesical Mycobacterium bovis BCG.
Three years before her most recent admission, recur-
rent carcinoma in situ of the bladder was identified,
and she was again treated with intravesical BCG, to
which she had an excellent response. The patient had
no evidence of recurrence until 1 year before her most
recent admission, at which time she was experiencing
urinary urgency, frequency, and reduced bladder ca-
pacity.
Examination at that time revealed a small amount
of erythema about the urethra. The results of the phys-
ical examination, including a pelvic examination, were
otherwise unremarkable. Cystoscopic studies revealed
erythema of the bladder mucosa and a normal-appear-
ing urethra. Biopsies of the bladder mucosa were neg-
ative. Urine cytology, however, was reported as positive
for a high-grade neoplasm. In addition to the recurrent
bladder carcinoma, the patient had developed crip-
pling urinary incontinence and thus underwent an ileal
conduit procedure. (Because of her extensive history of
coronary artery disease, the patient was not considered FIGURE 1. Bladder biopsy results showing urothelial carci-
a good candidate for complete cystectomy.) noma in situ associated with Paget disease of urothelial origin.
Six months later, at routine follow-up for ileal con- (Hematoxylin and eosin, original magnification ⫻400.)

550
 VULVAR PAGET DISEASE: A PROPOSED CLASSIFICATION (Wilkinson and Brown)
FIGURE 2. Gross photograph of vulvectomy specimen with
secondary Paget disease of urothelial origin. There is an exten-
sive erythematous lesion involving the vulvar vestibule (arrows)
and medial aspects of the labia minora.
15), or carcinoembryonic antigen (CEA). The his-
topathologic features and supporting immunohisto-
chemical profile were consistent with Paget-like
intraepithelial urothelial neoplasm. The bladder biopsy
was not available for immunohistochemical studies.
Case 2
The patient is an 81-year-old white woman with
known urothelial carcinoma in situ of the bladder,
diagnosed 6 years before her most recent admission.
The patient had been treated with multiple courses of
intravesical BCG. Three years before her current admis-
sion, a focal vulvar lesion was discovered during a
planned vaginal hysterectomy for uterine prolapse. Re-
sults of local excision and biopsy of the lesion at that
time was interpreted by another institution as vulvar
intraepithelial neoplasia 3 (VIN 3) with a pagetoid
appearance. These slides were not available for our
review. During a subsequent visit for follow-up of blad-
der cancer, her entire vulvar vestibule was erythema-
tous and eczematoid in appearance, and the lesion
clinically involved the periurethral and left vaginal in-
troital mucosa, with focal periclitoral extension. The
medial and lateral aspects of the left labium minus and
551
the medial aspect of the left labium majus were also
noted to be involved, but the lesion had a more gran-
ular eczematoid appearance in these locations. Exami-
nation of the vagina identified minor extension of the
erythematous lesion seen in the vestibule into the most
distal portion of the vagina, immediately adjacent to
the hymenal ring. A biopsy of tissue obtained from the
lower left labium majus performed by a private physi-
cian was interpreted at another laboratory as Paget
disease. A vaginal smear for cytologic study demon-
strated tumor cells interpreted as consistent with a
high-grade neoplasm. The rest of the patient’s physical
examination findings were unremarkable. Inguinal
femoral nodes were not palpable.
The patient was scheduled for radical vulvectomy
for vulvar Paget disease. Preoperative cystoscopic exam-
ination of the bladder revealed 2 erythematous areas 3
cm in diameter, 1 area adjacent to the right ureteral
orifice and the other on the right side of the posterior
bladder wall. The urethra was interpreted clinically as
within normal limits, and thus tissue was not obtained
for biopsy. The patient underwent total vulvectomy
with excision to the fascia and partial vaginal excision,
including excision of the hymen and approximately 1
cm of the lower distal vagina. During the same surgery,
the bladder lesions were biopsied. The histopathologic
findings of the vulvar resection specimen demonstrated
an extensive Paget-like intraepithelial neoplasm involv-
ing the entire epithelium of the vulvar vestibule, includ-
ing the labia minora and left labium majus, with exten-
sion into the lower third of the vagina. The resection
margins were free of neoplasia. The neoplasm involved
the intraepithelial components of the skin appendages
in the involved areas, along with the Bartholin duct. No
evidence of perianal involvement by the intraepithelial
neoplasm was noted, and no underlying areas of inva-
sion were seen.
Immunohistochemical findings of the involved vul-
var skin demonstrated the Paget-like cells within the
epithelium to be immunoreactive for CK 7, CK 20, and
UP-III but not immunoreactive with GCDFP-15 or CEA.
A bladder wash sample obtained intraoperatively con-
tained rare abnormal cells consistent with a high-grade
urothelial neoplasm. Biopsies of the bladder lesions
showed urothelial carcinoma in situ. Immunohisto-
chemical studies subsequently performed on the blad-
der biopsy samples demonstrated an immunohisto-
chemical profile identical to that of the vulvar lesion
(CK 7, CK 20, and UP-III positive; GCDFP-15 and CEA
negative). Follow-up examination of this patient
showed no recurrence at 9 months.
Case 3
The patient is a 76-year-old woman with a past
history of vulvar Paget disease diagnosed 4 years earlier.
The patient underwent 2 subsequent partial vulvecto-
mies to excise the Paget disease. She was subsequently
seen at our institution for urethral stenosis and urinary
retention. Examination revealed periurethral indura-
tion and scar tissue surrounding the urethral meatus.
 HUMAN PATHOLOGY
This scar tissue was excised, and pathologic examina-
tion showed Paget disease with scattered tumor cells in
the deep dermis. Subsequent cystoscopic examination
showed that the entire urethra had a friable mucosa. A
raised 2-cm nodule was identified in the bladder base,
and the mucosa of the left bladder neck was raised,
irregular and indurated. Biopsies of the bladder lesions
and urethra revealed a poorly differentiated carci-
noma. Immunohistochemical profiles of the vulva and
bladder specimens were identical and showed immuno-
reactivity for CK 7, CK 20, and CEA. GCFDP-15 and
UP-III were not immunoreactive in either specimen.
DISCUSSION
It is generally accepted that the cells of primary
cutaneous Paget disease arise from an intraepidermal,
552
Volume 33, No. 5 (May 2002)
FIGURE 3. (A) Secondary vulvar Paget dis-
ease of urothelial origin involving the vulvar
skin. Low magnification demonstrates the
clefts within the parabasal areas between
the normal epithelial cells and the small
groups of neoplastic urothelial Paget-like
cells (arrows). (Hematoxylin and eosin, origi-
nal magnification ⫻400.) (B) Secondary vul-
var Paget disease. Large pleomorphic cells
with hyperchromatic nuclei are within the ep-
ithelium. These neoplastic urothelial cell nu-
clei are as large or larger than the nuclei of
the adjacent epithelial cells. Some have nu-
cleoli. The neoplastic cells predominantly in-
volve the basal and parabasal epithelial lay-
ers. Some of the tumor cells have small
cytoplasmic vacuoles, and acantholysis is
present (arrows mark the neoplastic urothe-
lial cells adjacent to normal keratinocytes).
(Hematoxylin and eosin, original magnifica-
tion ⫻600.) (C) Primary cutaneous Paget dis-
ease. Large cells with pale cytoplasm are
within the epithelium occurring in clusters and
as single cells primarily within the basal and
parabasal areas, but also are within the en-
tire epithelium. (Hematoxylin and eosin, orig-
inal magnification ⫻400.) (D) Primary cutane-
ous Paget disease. The large Paget cells
have pale cytoplasm and large pleomorphic
nuclei, many of which have prominent nucle-
oli. The Paget cell nuclei are as large or larger
than the adjacent keratinocytes. The Paget
cells are distributed in clusters and as single
cells within the epithelium. (Hematoxylin and
eosin, original magnification ⫻600.)
pluripotent stem cell showing apocrine differentia-
tion.4,5 A case of Paget disease of apparent Toker cell
origin has been reported.6
The typical Paget cell of extramammary cutaneous
origin is large and pleomorphic, often several times
larger than an adjacent keratinocyte. The Paget cells
occur singly or in nests, and in some cases can form
small acini within the epithelium. The cutaneous Paget
cells have abundant pale cytoplasm, which may be vac-
uolated. The nucleus is round to oval with vesicular
chromatin and usually a prominent nucleolus. The nu-
clear contour is quite regular with no infolding. Mitoses
may be seen, but are uncommon (Fig 3C and D).
The cells of secondary Paget disease of anal or
rectal origin are similar to those of cutaneous origin,
although signet-ring cells, goblet cells and cells with
 VULVAR PAGET DISEASE: A PROPOSED CLASSIFICATION (Wilkinson and Brown)
TABLE 2. Differential Diagnosis of Vulvar
Intraepithelial Pagetoid Cells
● Primary Paget disease*
● Secondary Paget disease (ano-rectal, urothelial, or other origin)*
● Vulvar intraepithelial neoplasia (VIN)†
● Superficial spreading malignant melanoma†
● Pagetoid Spitz nevus†
● Sebaceous carcinoma†
● Merkel cell carcinoma†
● Clear cell papulosis (related to Toker cells)†
● Eccrine porocarcinoma†
● Cutaneous T-cell lymphoma†
● Histiocytosis X†
● Langerhans’ cell microabscess†
● Benign mucinous metaplasia of the vulva†16
*Usually requires immunohistochemical studies to distinguish.
†Can usually be distinguished from vulvar primary or secondary
EMPD by clinical or histopathologic appearance.
prominent cytoplasmic vacuoles may be prominent. In
some cases of anorectal Paget disease, the Paget cells
may be stratified columnar cells resembling the anorec-
tal glands, and intraluminal necrosis may be present.10
Secondary vulvar Paget disease of urothelial origin
is characterized by small groups of cells and single cells,
predominantly in the basal layer of the epithelium. In
some areas, prominent acantholysis may be present,
with groups of the neoplastic cells present in the areas
of acantholysis. The cellular features are essentially
those of a high-grade urothelial neoplasm. Individual
tumor cells are as large or slightly larger than the
adjacent keratinocytes, but are not as pleomorphic as
some cells seen in cutaneous Paget disease. (Fig 3A and
B) The tumor cells have hyperchromatic nuclei with
small and inconspicuous nucleoli. The nuclear mem-
branes appear irregular in contour and partially folded
in some areas. The cytoplasm contains only rare vacu-
oles, with no signet-ring cells. Mitotic figures are usually
numerous, and some are abnormal in appearance.
Diagnostic difficulty occurs when pagetoid spread
occurs within the vulvar epidermis by cells of unclear
origin, mimicking primary Paget disease. Pagetoid
spread is possible by cells of epithelial, melanocytic,
neuroendocrine, lymphoid, Toker cell, and histiocytic
differentiation.2,6 Therefore, the differential diagnosis
of intraepidermal pagetoid cells is extensive (Table 2).
In the vulva, most of these entities can be distinguished
based on clinical presentation and histologic appear-
ance. For example, vulvar melanoma in situ and VIN
usually do not clinically resemble vulvar Paget disease.
In contrast, clinically differentiating secondary vul-
var Paget disease from primary vulvar Paget disease may
be very difficult. The 3 cases studied here illustrate this
point. Both primary and secondary Paget disease often
present as eczematoid lesions,17 although some specific
clinical features do exist that may help distinguish
them. Primary vulvar Paget disease presents on vulvar
skin, often on the lateral aspects of the labia majora.
Paget disease as a manifestation of anal or rectal ade-
nocarcinoma typically involves the perianal skin along
with adjacent contiguous vulvar skin. Paget disease as a
manifestation of urothelial neoplasia primarily involves
553
the vulvar vestibule and periurethral vulvar vestibular
mucosa, but may extend to the adjacent vulvar skin.
Because the clinical appearance of primary and
secondary vulvar Paget disease are of similar clinical
appearance and thus may be indistinguishable by con-
ventional histopathologic examination, immunohisto-
chemical differentiation has been studied. We recently
reported the immunohistochemical findings of 17 pa-
tients with vulvar Paget disease, 14 with cutaneous dis-
ease and 3 with secondary vulvar Paget disease of
urothelial origin.18 Additionally, we reported the poten-
tial usefulness of immunohistochemistry for urothe-
lium (UP-III)19 in distinguishing among these subtypes
of vulvar Paget disease.18
Distinguishing primary from secondary vulvar
Paget disease is essential because the proper diagnosis
has a significant influence on current treatment. Pri-
mary cutaneous Paget disease is usually treated with
total excision of the visible lesion, with a 1- to 3-cm
margin of excision and excision to the fascia to exclude
invasion or underlying skin appendage adenocarci-
noma. Invasive primary vulvar Paget disease or under-
lying invasive adenocarcinoma, usually of skin append-
age origin, is reported in approximately 20% of the
published studies of vulvar Paget disease and may be
associated with an aggressive clinical course and meta-
static disease.15
In contrast, vulvar Paget disease related to anal or
rectal adenocarcinoma or as a manifestation of urothe-
lial neoplasia is usually entirely intraepithelial within
the vulva. Therefore, the treatment for secondary Paget
disease can be modified to address the primary neo-
plastic lesion in the primary site and treat the intraepi-
thelial vulvar lesion as an intraepithelial, noninvasive
process. In most cases, superficial involvement of the
vulvar or perianal skin can be treated with superficial
excision or a superficial ablative technique.
In summary, subclassification of vulvar Paget dis-
ease as primary and secondary neoplasms will contrib-
ute to directing appropriate therapy both for the vulvar
lesion as well as the primary neoplastic process second-
arily involving the vulva in the case of secondary vulvar
Paget disease. Our currently proposed subclassification
of vulvar Paget disease is intended to separate and
define these vulvar lesions and to aid clinical manage-
ment and treatment.
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