Porth's Essentials of Pathophysiology, 4e

1026

Genitourinary and Reproductive Function

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eczema or dermatitis and may produce few symptoms other than pruritus, local discomfort, and exudation. The lesion may become secondarily infected, causing pain and discomfort. The malignant lesion gradually spreads superficially 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, including those of the inguinal and femoral chains. Early diagnosis is important in the treatment of vul- var carcinoma. Because malignant lesions can vary in appearance and commonly are mistaken for other con- ditions, biopsy and treatment often are delayed. Any vulvar lesion that is increasing in size or has an unusual wartlike appearance should be biopsied. Treatment is primarily wide surgical excision of the lesion for non- invasive cancer and radical excision or vulvectomy with node resection for invasive cancer. Vulvodynia Vulvodynia is a syndrome of unexplained vulvar pain, previously referred to as vulvar pain syndrome or burn- ing vulva syndrome . 12,13 The terminology and diag- nostic criteria used for this chronic disorder remain in flux, but the most recent classification system of the International Society for the Study of Vulvovaginal Disorders (ISSVD) defines it as a condition charac- terized by a sensation of burning, stinging, irritation, soreness or rawness in the absence of relevant visible findings or a specific, clinically identifiable neurological disorder. 12,14 Vulvodynia is further classified as localized or generalized, and as to whether it is provoked, unpro- voked, or of mixed origin. Localized vulvodynia or vestibulodynia , formerly referred to as vulvar vestibulitis syndrome , is charac- terized by pain at onset of intercourse, localized point tenderness near the vaginal opening, and sensitivity to tampon placement, tight-fitting pants, bicycling, or pro- longed sitting. It is the leading cause of dyspareunia in women younger than 50 years of age. The pain can be primary (present from first contact) or secondary (devel- oping after a period of comfortable sexual relations). The etiology is unknown, but the problem may evolve from chronic vulvar inflammation or trauma. Nerve fibers that supply the vestibular epithelium may become highly sensitized, causing neurons in the dorsal horn of the spinal cord to respond abnormally, thus transform- ing the sensation of touch in the vestibule into pain. Generalized vulvodynia , formerly called vulvar dys- esthesia or essential vulvodynia , involves severe, con- stant, widespread burning the vulvar area that interferes with daily activities. No abnormalities are found on examination, but there is diffuse and variable hypersen- sitivity and altered sensation to light touch. The quality of this unprovoked pain shares many of the features of other neuropathic pain disorders, particularly complex regional pain syndrome (see Chapter 35) or pudendal neuralgia. Although the cause of the neuropathic pain is unknown, it has been suggested that it may result from myofascial restrictions affecting the sacral and pelvic floor nerves. 12,13

There are many proposed triggers for vulvodynia, including chronic recurrent vaginal infections; chemi- cal irritation or drug effects, especially prolonged use of topical steroid creams; the irritating effects of elevated urinary levels of calcium oxalate; and immunoglobulin A deficiency or other disorders of immune regulation. Often it is multifactorial in origin. Careful history taking and physical assessment are essential for differential diagnosis and treatment. Vulvodynia is a diagnosis of exclusion after ruling out infections, such as candidiasis and genital herpes; inflammatory conditions, such as squamous cell hyper- plasia and lichen sclerosus; vulvar cancer; or neurologic disorders, such as herpes neuralgia or spinal nerve com- pression, as causes for the pain. Treatment of vulvodynia is aimed at symptom relief, is frequently long term, and often needs to be managed from a multidimensional, chronic pain perspective. 12,13 Local measures include avoidance of harsh soaps and perfumed products, use of sitz baths, and application of topical anesthetic agents (i.e., lidocaine gel). Because dermatologic conditions such as atopic dermatitis and candidiasis are responsible for many of the symptoms of vulvodynia, some health care providers recommend treatment with antihistamines and oral antifungal medi- cations, as well as avoiding contact with potential irri- tants. 14 Biofeedback and physical therapy may be used to reverse the changes in pelvic floor musculature and help women control the muscles, regaining strength and improving relaxation. 14 Oral medications, including tricyclic antidepressants and other antidepressants, are often used to treat the neuropathic pain associated with vulvodynia. Botulism toxin A injections block the cho- linergic innervation of the target tissues and have been shown to be effective in some women with vulvodynia. 14 Another treatment option for women with severe dis- comfort is surgical excision of the vestibule. It is com- monly the last option and should be reserved for women with long-standing severe symptoms after all other man- agement has yielded unsatisfactory results. 14 Disorders of the Vagina The normal vaginal ecology depends on the delicate bal- ance of hormones and bacterial flora. Normal estrogen levels maintain a thick, protective squamous epithelium that contains glycogen. Döderlein bacilli, part of the normal vaginal flora, metabolize glycogen, and in the process produce the lactic acid that normally maintains the vaginal pH of 3.8 to 4.5. 15 Disruptions in these nor- mal environmental conditions predispose to infection. Vaginitis Vaginitis represents an inflammation of the vagina that is characterized by vaginal discharge and burning, itch- ing, redness, and swelling of vaginal tissues. 15,16 Pain often occurs with urination and sexual intercourse. Vaginitis may be caused by chemical irritants, foreign bodies, or infectious agents. The causes of vaginitis dif- fer in various age groups. In premenarchal girls, most vaginal infections have nonspecific causes, such as poor