DISORDERS OF THE EXTERNAL GENITALIA

Vulvitis and Folliculitis Vulvitis is characterized by inflammation and pruritus of the vulva. It is not
considered a specific disease but typically accompanies other local and systemic disorders. The cause
often is an irritating vaginal discharge. Candida albicans, a yeast, is the most common cause of chronic
vulvar pruritus, particularly in women with diabetes mellitus. Vulvitis also may be a component of sexually
transmitted diseases (STDs) such as herpes genitalis and human papillomavirus (HPV) infection (i.e.,
condyloma). Local dermatologic reactions to chemical irritants, such as laundry products, perfumed soaps
or sprays, and spermicides, or to allergens such as poison ivy, also can cause inflammation. Vulvar
itching also may be caused by atrophy that is part of the normal aging process. Management of vulvitis
focuses on appropriate treatment of underlying causes and comfort measures to relieve the irritation.
These include keeping the area clean and dry; using warm sitz baths with baking soda, wet dressings, or
Burows solution soaks (a mild astringent); or applying a mild hydrocortisone cream for the immediate
relief of symptoms. Folliculitis is an infection that involves the hair follicles of the mons or labia majora.
The infection, characterized by small red papules or pustules surrounding the hair shaft, is relatively
common because of the density of bacteria in this area and the occlusive nature of clothing covering the
genitalia. Treatment includes thorough cleaning of the area with germicidal soap, followed by the
application of a mild antibacterial ointment (e.g., Neosporin or Polysporin). Bartholins Gland Cyst and
Abscess Bartholins cyst is a fluid-filled sac that results from the occlusion of the duct system in
Bartholins gland. When the cyst becomes infected, the contents become purulent; if the infection goes
untreated, an abscess can result. The obstruction that causes cyst and abscess formation most
commonly follows a bacterial, chlamydial, or gonococcal infection. Cysts can attain the size of an orange
and frequently recur (Fig. 47-1). Abscesses can be extremely tender and painful. Asymptomatic cysts
require no treatment. The treatment of symptomatic cysts consists of the administration of appropriate
antibiotics, local application of moist heat, and incision and drainage. 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).1
Epidermal Cysts Epidermal cysts (i.e., sebaceous or inclusion cysts) are common semisolid tumors of the
vulva. These small nodules are lined with stratified squamous epithelium and contain cellular debris with a
sebaceous appearance and odor. Epidermal cysts may be solitary or multiple and have a yellow
appearance when stretched or compressed. They usually resolve spontaneously, and treatment is
unnecessary unless they become infected or significantly enlarged. Nevi Nevi (moles) occur on the vulva
as elsewhere on the body. They can be singular or multiple, flat or raised, and may vary in degree of
pigmentation from flesh colored to dark brown or black. Nevi are asymptomatic but should be observed
for changes that could indicate cancer. Nevi may resemble melanomas or basal cell carcinomas, and
excisional biopsy is recommended when doubt exists (see Chapter 61
Current treatment of lichen sclerosus favors the use of potent topical corticosteroids (clobetasol or
halobetasol).3 Hyperplastic areas respond well to a combination corticosteroid (e.g., betamethasone
valerate) and antipruritic cream (e.g., crotamiton) and to the removal of any irritants (e.g., detergents,

perfumes). Lichen sclerosus frequently recurs, and lifetime maintenance therapy may be required.
Hyperplastic areas that occur in the field of lichen sclerosus may be sites of malignant change and
warrant close follow-up and possible biopsy. Vulvodynia Vulvodynia is a syndrome of unexplained vulvar
pain, also referred to as vulvar pain syndrome or burning vulva syndrome. It is a chronic disorder
characterized by burning, stinging, irritation, and rawness. Several forms or subsets of vulvodynia have
been identified, including cyclic vulvaginitis, vulvar dermatoses, vulvar vestibular syndrome, and vulvar
dysesthesia. Because vulvodynia is a multifaceted condition, certain subsets may coexist with others.
Cyclic vulvodynia demonstrates episodic flares that occur only before menses or after coitus. Vulvar
dermatoses are manifested by pruritus, and in some cases, pain develops progressively during the
perimenopausal or postmenopausal period. Vulvar dermatoses include thick and scaly (e.g.,
papulosquamous) lesions. Erosions may occur from excessive scratching. Vulvar vestibulitis syndrome
(VVS) is characterized by pain at onset of intercourse (i.e., insertional dyspareunia), localized point
tenderness near the vaginal opening, and sensitivity to tampon placement, tight-fitting pants, bicycling, or
prolonged sitting. It is the leading cause of dyspareunia in women younger than 50 years of age. VVS can
be primary (present from first contact) or secondary (developing after a period of comfortable sexual
relations). Etiology is unknown but VVS can evolve from chronic vulvar inflammation or trauma. Nerve
fibers to the vestibular epithelium become highly sensitized, causing neurons in the dorsal horn to
respond abnormally, which transforms the sensation of touch in the vestibule into pain (allodynia).4 Vulvar
dysesthesia, also known as idiopathic or essential vulvodynia, involves severe, constant, widespread
burning that interferes with daily activities. No abnormalities are found on examination, but there is diffuse
and variable hypersensitivity and altered sensation to light touch. The quality of pain shares many of the
features of neuropathic pain, particularly complex regional pain syndrome (see Chapter 50) or pudendal
neuralgia. Although the cause of the neuropathic pain is unknown, it has been suggested that it may
result from myofascial restrictions affecting sacral and pelvic floor nerves. Surface electromyographyassisted pelvic floor muscle rehabilitation has been shown to be an effective and long-term cure for
dysesthetic vulvodynia.5 Possible causes for other forms of vulvodynia include candidal hypersensitivity
related to chronic recurrent yeast infections; chemical irritation or drug effects, especially prolonged use of
topical steroid creams; the irritating effects of elevated urinary levels of calcium oxalate; immunoglobulin A
deficiency; and dermatoses such as lichen sclerosus, lichen planus, or squamous cell hyperplasia.
Herpes simplex virus may be related to episodic vulvodynia, and long-term viral suppressive therapy may
be of benefit to women with known herpes simplex virus infection who experience multiple outbreaks each
year. Previous links to HPV infection have not been supported by studies, and the finding of subclinical
HPV infection in women with vulvodynia now is thought to be a secondary or unrelated phenomenon.
Treatment of this chronic, often debilitating problem is aimed at symptom relief and elimination of
suspected underlying problems. Careful history taking and physical assessment are essential for the
differential diagnosis and treatment. Regimens can include long-term vaginal or oral antifungal therapy,
avoidance of potential irritants, cleaning with water only or a gentle soap, sitz baths with baking soda,
emollients such as vitamin E or vegetable oil for lubrication, low-oxalate diet plus calcium citrate
supplements (calcium binds oxalate in the bowel, and citrate inhibits the formation of oxalate crystals),
topical anesthetic or steroid ointments, physical therapy, and surgery. The tricyclic antidepressants are
often used to treat the neuropathic pain associated with vulvar dysesthesia. Psychosocial support often is

needed because this condition can cause strain in sexual, family, and work relationships. Vulvodynia often
needs to be managed from a multidimensional, chronic pain perspective.

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arcinoma of the vulva accounts for approximately 4% of all cancers of the female genitourinary system.
This translates to approximately 4000 U.S. cases of vulvar cancer in 2003, resulting in 800 deaths.6
Vulvar cancer appears to exist as two separate diseases affecting different age groups. Invasive
carcinoma occurs most frequently in women who are 60 years of age or older, and its incidence has
remained relatively stable since the early 1980s. The mean age for carcinoma in situ is 20 years younger
than for invasive carcinoma (45 to 50 years), and the incidence of vulvar cancer

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n women younger than 50 years of age has increased from 2% to 21% during the past 20 years.7
Approximately 90% of vulvar malignancies are squamous cell carcinomas. Less common types of cancer
found on the vulva include adenocarcinoma in the form of extramammary Paget disease (intraepithelial or
invasive) or carcinoma of Bartholins gland, basal cell carcinoma, and malignant melanoma.6 Vulvar
intraepithelial neoplasia (VIN), which is a precursor lesion of squamous cell carcinoma, represents a
spectrum of neoplastic changes that range from minimal cellular atypia to invasive cancer. VIN appears to
be caused by the oncogenic (cancer-promoting) potential of certain strains of HPV (subtypes 16 and 18)
that are sexually transmitted and is associated with the type of vulvar cancer found in younger women.7
VIN lesions may take many forms. The lesions may be singular or multicentric, macular, papular, or
plaquelike. VIN frequently is multicentric, and approximately 50% are associated with squamous
neoplasms in the vagina and cervix.2 Microscopically, VIN presents as a proliferative process
characterized by cells with abnormal epithelial maturation, nuclear enlargement, and nuclear atypia. The
same system that is used for grading cervical cancer is used for vulvar cancer.2,3 The extent of
replacement of epithelial cells by abnormal cells determines the grade of involvement (VIN I, II, or III).
Full-thickness replacement, VIN III, is synonymous with carcinoma in situ. Spontaneous resolution of VIN
lesions has occurred. The risk for progression to invasive cancer increases in older women and in
immunosuppressed women. A second form of vulvar cancer, which is seen more often in older women, is
generally preceded by vulvar nonneoplastic epithelial disorders (VNED) such as chronic vulvar irritation or
lichen sclerosus. The pruritus associated with VNED causes an itchscratch cycle that can lead to
squamous cell hyperplasia. If left untreated, the hyperplasia progresses to atypia (differentiated VIN), and
many of these women develop invasive cell carcinoma after 6 to 7 GYNECOLOGIC CANCERS
Cancers of the vulva, cervix, endometrium, and ovaries represent a spectrum of malignancies. Cancers
of the vulva and cervix are mainly squamous cell carcinomas. Certain types of sexually transmitted

human papillomaviruses are risk factors for cervical intraepithelial neoplasia, which can be a precursor
lesion of invasive carcinoma. Endometrial cancers, which are seen most frequently in women 55 to 65
years of age, are strongly associated with conditions that produce excessive estrogen stimulation and
endometrial hyperplasia. Ovarian cancer is the second most common female cancer and the most
lethal. The most significant risk factors for ovarian cancers are the length of time that a womans ovarian
cycles are not suppressed by pregnancy, lactation, or oral contraceptive use, and family history. years.7
The etiology of this type of VIN is infrequently associated with HPV. The initial lesion of squamous cell
vulvar carcinoma 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 eczema or dermatitis and may produce few symptoms, other than
pruritus, local discomfort, and exudation. A recurrent, persistent, pruritic vulvitis may be the only
complaint. The symptoms frequently are treated with various home remedies before medical treatment is
sought. The lesion may become secondarily infected, causing pain and discomfort. The malignant lesion
gradually spreads superf icially or as a deep furrow involving all of one labial side. Because there are
many lymph channels around the vulva, the cancer metastasizes freely to the regional lymph nodes. The
most common extension is to the superficial inguinal, deep femoral, and external iliac lymph nodes.
Overall incidence of lymph node metastasis is approximately 30%.7 Early diagnosis is important in the
treatment of vulvar carcinoma. Because malignant lesions can vary in appearance and commonly are
mistaken for other conditions, biopsy and treatment often are delayed. Any vulvar lesion that is increasing
in size or has an unusual warty appearance should be biopsied.7 Treatment is primarily wide surgical
excision of the lesion for noninvasive cancer and radical excision or vulvectomy with node resection for
invasive cancer. Postoperative groin and pelvic radiation is recommended when groin lymph nodes are
involved. Nonsurgical treatment options such as photodynamic therapy or topical immunotherapy are
currently under investigation for patients with early-stage vulvar cancer.8 The 5-year survival rate for
women with lesions less than 3 cm in diameter and minimal node involvement is approximately 90% after
surgical treatment. Follow-up visits every 3 months for the first 2 years after surgery and every 6 months
thereafter are important to detect recurrent disease or a second primary cancer. The 5-year survival rate
for patients who have larger lesions in conjunction with pelvic lymphadenopathy drops to 30% to 55%
after surgical treatment. Outlook for survival diminishes with increasing nodal involvement.6 DISORDERS
OF THE VAGINA The normal vaginal ecology depends on the delicate balance of hormones and bacterial
flora. Normal estrogen levels maintain a thick, protective squamous epithelium that contains glycogen.
Dderleins bacilli, part of the normal vaginal flora, metabolize glycogen, and in the process produce the
lactic acid that normally maintains the vaginal pH below 4.5. Disruptions in these normal environmental
conditions predispose to infection. Vaginitis Vaginitis is inflammation of the vagina; it is characterized by
vaginal discharge and burning, itching, redness, and swelling of vaginal tissues. Pain often occurs with
urination and sexual intercourse. Vaginitis may be caused by chemi

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cal irritants, foreign bodies, or infectious agents. The causes of vaginitis differ in various age groups. In
premenarchal girls, most vaginal infections have nonspecific causes, such as poor hygiene, intestinal
parasites, or the presence of foreign bodies. C. albicans, Trichomonas vaginalis, and bacterial vaginosis
are the most common causes of vaginitis in the childbearing years, and some of these organisms can be
transmitted sexually2,3,9 (see Chapter 48). In postmenopausal women, atrophic vaginitis is the most
common form. Atrophic vaginitis is an inflammation of the vagina 1069 bleach (i.e., a fungicide) may be
preferable for women to prevent such infections. Swimsuits and other garments that cannot withstand hot
water or bleaching should be hung in the sunlight to dry. Women should be taught to wipe the perineal
area from front to back to avoid bringing rectal contamination into the vagina. Avoiding sexual contact
whenever an infection is known to exist or suspected should limit that route of transmission. Cancer of the
Vagina that occurs after menopause or removal of the ovaries and their estrogen supply. Estrogen
deficiency results in a lack of regenerative growth of the vaginal epithelium, rendering these tissues more
susceptible to infection and irritation. Dderleins bacilli disappear, and the vaginal secretions become less
acidic. The symptoms of atrophic vaginitis include itching, burning, and painful intercourse. These
symptoms usually can be reversed by local application of estrogen.2,3 Every woman has a normal
vaginal discharge during the menstrual cycle, but it should not cause burning or itching or have an
unpleasant odor. These symptoms suggest inflammation or infection. Because these symptoms are
common to the different types of vaginitis, precise identification of the organism is essential for proper
treatment. A careful history should include information about systemic disease conditions, the use of
drugs such as antibiotics that foster the growth of yeast, dietary habits, stress, and other factors that alter
the resistance of vaginal tissue to infections. A physical examination usually is done to evaluate the nature
of the discharge and its effects on the genital structures. Microscopic examination of a saline wet-mount
smear (prepared by placing a sample of vaginal mucus in one to two drops of normal saline) is the
primary means of identifying the organism responsible for the infection. A small amount of 10% potassium
hydroxide (KOH) is added to a second specimen on the slide to aid in the identification of C. albicans.
KOH destroys the cellular material, causing the epithelial cells to become increasingly transparent so that
the hyphae and buds that are characteristic of Candida become much easier to see. Culture methods may
be needed when the organism is not apparent on the wetmount preparation.3 The prevention and
treatment of vaginal infections depend on proper hygiene habits and accurate diagnosis and treatment of
ongoing infections. Measures to prevent infection include development of daily hygiene habits that keep
the genital area clean and dry, maintenance of normal vaginal flora and healthy vaginal mucosa, and
avoidance of contact with organisms known to cause vaginal infections. Perfumed products, such as
feminine deodorant sprays, douches, bath powders, soaps, and even toilet paper, can be irritating and
may alter the normal vaginal f lora. Tight clothing prevents the dissipation of body heat and evaporation of
skin moisture and promotes favorable conditions for irritation and the growth of pathogens. Nylon and
other synthetic undergarments, pantyhose, and swimsuits hold body moisture next to the skin and harbor
infectious organisms, even after they have been washed. Cotton undergarments that withstand hot water
and Primary cancers of the vagina are extremely rare. They account for approximately 3% of all cancers
of the female reproductive system. Like vulvar carcinoma, carcinoma of the vagina is largely a disease of
older women. Approximately half of women are 60 years of age or older at the time of diagnosis. The
exception to that is the clear cell adenocarcinoma associated with diethylstilbestrol (DES) exposure in
utero, which is associated with an average age at diagnosis of 19 years. Vaginal cancers may result from

local extension of cervical cancer, from exposure to sexually transmitted HPV, or rarely from local irritation
such as occurs with prolonged use of a pessary.10 Approximately 85% to 90% of vaginal cancers are
squamous cell carcinomas, with other common types being adenocarcinomas (5% to 10%), sarcomas
(2% to 3%), and melanomas (2% to 3%).10 Squamous cell carcinomas begin in the epithelium and
progress over many years from precancerous changes called vaginal intraepithelial neoplasia (VAIN).
Maternal ingestion of DES in early pregnancy has been associated with the development of clear cell
adenocarcinoma in female offspring who were exposed in utero. Between 1938 and 1971, DES, a
nonsteroidal synthetic estrogen, commonly was prescribed to prevent miscarriage.11 The incidence of
clear cell adenocarcinoma of the vagina is low, approximately 0.1%, in young women who were exposed
to DES in utero. Although only a small percentage of girls exposed to estrogen actually develop clear cell
adenocarcinoma, 75% to 90% of them develop benign adenosis (i.e., ectopic extension of cervical
columnar epithelium into the vagina, which normally is stratified squamous epithelium), which may
predispose to cancer. Most DES-exposed daughters are now between 40 and 60 years of age, so they
are just entering the postmenopausal period when this malignancy develops in women who were not
exposed to DES. Because the upper age limit for this type of cancer is unknown, there is no age at which
a DESexposed daughter can be considered risk free.12 The most common symptom of vaginal
carcinoma is abnormal bleeding. Other signs or symptoms include an abnormal vaginal discharge, a
palpable mass, or pain during intercourse. Ten to twenty percent of women are asymptomatic, with the
cancer being discovered during a routine pelvic examination. The anatomic proximity of the vagina to
other pelvic structures (e.g., urethra, bladder, rectum) permits early spread to these areas. Pelvic pain,
dysuria, and constipation can be associated symptoms. Vaginal squamous cell carcinoma most often is
detected in the upper posterior one third of the vagina, with adenocarcinoma more often found on the
lower anterior and lateral vaginal vault. Women should continue to have vaginal cytology studies

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(Papanicolaous test [Pap smear]) every 3 to 5 years after hysterectomy to exclude development of vaginal
cancer if the hysterectomy was performed for a reproductive cancer. Diagnosis requires biopsy of
suspicious lesions or areas. Treatment of vaginal cancer must take into consideration the type of cancer;
the size, location, and spread of the lesion; and the womans age. Local excision, laser vaporization, or a
loop electrode excision procedure (LEEP) can be considered with stage 0 squamous cell cancer. Radical
surgery and radiation therapy are both curative with more advanced cancers. When there is upper vaginal
involvement, radical surgery may be required. This includes a total hysterectomy, pelvic lymph node
dissection, partial vaginectomy, and placement of a graft from the buttock to the area from which the
vagina was excised. Vaginal reconstruction often is possible to allow for sexual intercourse. The ovaries
usually are preserved unless they are diseased. Extensive lesions and those located in the middle or
lower vaginal area usually are treated by radiation therapy, which can be intracavitary, interstitial, or
external beam. The prognosis depends on the stage of the disease, the involvement of lymph nodes, and
the degree of mitotic activity of the tumor. With appropriate treatment and follow-up, the 5-year survival

rate for squamous cell and adenocarcinoma ranges from 96% for stage 0 and 73% when confined to the
vagina (stage I), to 36% for those with extensive spread (stages III and IV)