Porth's Essentials of Pathophysiology, 4e

1025

Disorders of the Female Genitourinary System

C h a p t e r 4 0

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