Porth's Essentials of Pathophysiology, 4e - page 1042

C h a p t e r 4 0
Disorders of the Female Genitourinary System
1025
Bartholin gland cysts and benign and malignant disor-
ders of the vulva.
Bartholin Gland Cyst and Abscess
A Bartholin gland cyst is a fluid-filled sac located near
the vaginal introitus that results from obstruction of the
main duct of the gland (see Fig. 40-2).
6–8
It may become
large, up to 3 to 5 cm in diameter, and produce pain and
local discomfort. Infection of a Bartholin cyst produces
acute inflammation within the gland and may result in
an abscess. Acute symptoms are usually the result of
infection and include pain, tenderness, and dyspareunia.
The treatment of symptomatic Bartholin gland cysts
consists of the administration of appropriate antibiotics,
local application of moist heat, and incision and drain-
age. Cysts that frequently are abscessed or are large
enough to cause blockage of the introitus may require
surgical intervention (i.e., marsupialization, a procedure
that involves removal of a wedge of vulvar skin and the
cyst wall).
8
Because the Bartholin glands usually shrink
during menopause, a vulvar growth in postmenopausal
women should be evaluated for malignancy.
Benign and Malignant Disorders of theVulva
Disorders of the vulva often present as white lesions of
the vulvar skin and mucosa that may produce pruritus
(itching) and scaling. Although commonly referred to
as
leukoplakia
because of their plaquelike appearance,
these lesions can represent a number of disorders includ-
ing inflammatory dermatoses (e.g., psoriasis or chronic
dermatitis, discussed in Chapter 46), non-neoplastic
epithelial disorders, and premalignant and malignant
lesions of the vulva.
6
Non-neoplastic Epithelial Disorders.
The term
non-
neoplastic epithelial disorders
refers to nonmalignant
atrophic and hyperplastic changes of the vulvar skin and
mucosa.
6,7,9
Depending on clinical and histologic charac-
teristics, the lesions can be further categorized as lichen
sclerosus, lichen planus, and squamous cell hyperplasia
(also known as
lichen simplex chronicus
).
Lichen sclerosus
is an inflammatory disease of the
vulva characterized by plaquelike areas that may prog-
ress to parchment-thin epithelium with focal areas of
ecchymosis and superficial ulcerations secondary to
scratching. Atrophy and contracture of the vulvar tis-
sues with eventual stenosis of the introitus are common
when this condition becomes chronic. Itching is com-
mon and dyspareunia (painful intercourse) is frequent.
The condition develops insidiously and is progressive.
Lichen planus
, while similar to lichen sclerosus, is an
intensely inflammatory autoimmune disorder. Although
the symptoms and sequelae are similar to lichen scle-
rosus, lichen planus may also involve the mucosal and
keratinized skin of the oral, anal, and vulvovaginal
mucosa. Erosive lichen planus is characterized erythem-
atous erosions surrounded by white lacey edges called
Wickam’s striae
. Unlike lichen sclerosus, which is usu-
ally limited to the vulva, up to 70% of women with ero-
sive lichen planus have vaginal involvement.
9
Squamous cell hyperplasia,
or
lichen simplex chroni-
cus
, which usually presents as thickened, gray-white
plaques with an irregular surface, is a nonspecific con-
dition resulting from rubbing or scratching the skin to
relieve pruritus. Presumed to be a response of the geni-
tal skin to some type of irritant, this diagnosis is used
only when human papillomavirus (HPV) infection (see
Chapter 41), fungal infections, or other known caus-
ative conditions have been excluded. Pruritus is the
most common presenting complaint. Scaling is generally
present, and excoriations due to recent scratching can
often be seen. There is generally no increased predispo-
sition to cancer, but similar lesions are often present at
the margins of established cancer of the vulva.
Current treatment for lichen sclerosus, lichen planus,
and squamous cell hyperplasia includes the use of topi-
cal corticosteroids. Lichen sclerosus frequently recurs,
and lifetime maintenance therapy may be required.
Hyperplastic areas that occur in the field of lichen scle-
rosus may be sites of malignant change and warrant
close follow-up and possible biopsy.
Premalignant andMalignant Neoplasms.
Carcinoma
of the vulva is a relatively rare cancer that accounts for
approximately 3% to 5% of all cancers of the female
reproductive system in the United States, occurring
most often in women 60 years of age or older.
6,10,11
Approximately 85% to 95% of vulvar malignancies
are squamous cell carcinomas.
11
Less common forms
of vulvar cancer include malignant melanomas, adeno-
carcinoma, basal cell carcinoma, and metastatic cancers
from various other sites.
In terms of etiology, pathogenesis, and clinical pre-
sentation, vulvar carcinoma can be divided into two
general groups: (1) basaloid and warty carcinomas,
related to infection with oncogenic strains of HPV
(about 30% of cases), and (2) keratinizing squamous
cell carcinoma, not related to the human papillomavi-
rus (HPV) infection (about 70% of cases).
6,7
Invasive
basaloid and warty-type carcinomas develop from a
precancerous in situ lesion called
vulvar intraepithelial
neoplasia
(VIN). This type of lesion occurs in reproduc-
tive-age women, and risk factors are similar to those for
cancer in situ of the cervix.
6
Spontaneous resolution of
VIN lesions has occurred. The risk of progression to
invasive cancer increases in older women and in women
with suppressed immune function. The second form of
vulvar cancer, which is seen more often in older women,
is generally preceded by vulvar non-neoplastic disorders
such as chronic vulvar irritation or lichen sclerosus. The
etiology of this group of vulvar cancers is unclear, but
they are not typically associated with HPV. Neoplastic
changes may arise from lichen sclerosus lesions or
hyperplasia, leading directly to invasion, or through an
intermediate step involving cellular atypia.
6
Approximately 50% of women with vulvar cancer
present with pruritus and a visible lesion.
11
The lesion
may appear as an inconspicuous thickening of the skin,
a small raised area or lump, or an ulceration that fails to
heal. It may be single or multiple and vary in color from
white to velvety red or black. The lesions may resemble
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