The evaluation of vulvar lesions can be challenging because there are a variety of potential causes including infectious diseases, nonneoplastic epithelial disorders, and benign and malignant neoplasms. In addition, these conditions will frequently coexist;
for example, the patient with recurrent vulvovaginal candidiasis may develop contact dermatitis from frequent application of irritating topical products to the affected skin. In addition, some women present with chronic vulvar itching and burning due to dysesthetic vulvodynia and will have no obvious physical findings on examination.
This group of disorders includes the conditions that are sometimes referred to as the vulvar dystrophies or dermatoses. The current recommended classification system developed by the International Society for the Study of Vulvar Disease for these disorders is (1):
1. lichen sclerosus,
2. squamous cell hyperplasia,
An updated classification based on pathological criteria is nearing completion. While some of these conditions are discussed in more detail in other sections, they are briefly reviewed here.
Formerly known as lichen sclerosus et atrophicus, lichen sclerosus is a chronic der-matologic condition that is more common in women and may involve both vulvar and extragenital lesions. Patients typically present with severe itching and may also report burning and dyspareunia. The entire vulvar area may be involved, including the clitoris and perianal regions. The skin often has the typical "parchment appearance" with white papules and plaques that may appear crinkled. Fissures, telangiectasias, and stenosis of the introitus may occur. With long-standing disease, there is often alteration of the normal vulvar architecture resulting in absence of the labia minora, adhesions of the labia majora, and phimosis of the clitoris in extreme cases. While it can affect women of any age, it is more commonly seen in the postmenopausal years and approximately 10% to 15% of cases occur in pediatric patients (2).
This category includes lichen simplex chronicus and epithelial hyperplasia, and in the past was often called leukoplakia or hyperplastic dystrophy. Clinically, this typically presents with complaints of itching and burning, and often follows contact with a topical irritant, although the patient may not recall such an exposure. Any part of the vulva may be affected, and the skin may appear erythematous with fissuring and excoriation. Lichenification, or the accentuation of normal skin markings, which results in thickening and raised white plaques, is also commonly seen. The process may be localized or diffuse and can been seen in all of the reproductive stages. This condition frequently may be associated with long-term use of topical therapies such as antifungals or steroids for other conditions, and may be causally related. A history of atopy, allergies, or eczema is also a likely risk factor.
A challenging disease to both diagnose and manage, lichen planus is a papulosquamous disorder that may involve both cutaneous and mucosal surfaces. On the vulva, lesions are often violaceous papules that cause pruritus. A whitish reticular pattern may appear on the inner labia majora where there is more exposure to moisture, and on the vestibular and vaginal mucosa the lesions are usually erythematous erosions. Vaginal disease is often associated with a heavy yellow discharge and significant dys-pareunia. Long-standing disease is usually associated with adhesions of the vagina that begin distally and can result in shortening and narrowing or even obliteration of the vaginal canal. The most common site for extragenital disease is the oral cavity, and up to three-quarters of all patients with lichen planus will develop gingival or oral lesions at some point (3).
While the vulva is not usually the primary site for other dermatologic diseases, many skin conditions will involve the genital area at some point. The most commonly seen in clinical practice of these is psoriasis. Vulvar psoriasis may have the classic appearance of erythematous plaques with silvery, dry scale, but if the area is subject to chronic moisture, then it may have a macerated appearance with fissuring (4). The plaques generally are located on the mons or labia majora and extend to the genitocrural creases and buttocks.
Infections involving the vulva are commonly seen in clinical practice and should be included in the differential diagnosis of any vulvar complaint.
Almost every woman experiences at least one episode of vulvovaginal candidiasis during her lifetime. While women typically report vaginal itching with these infections, the site of symptoms is more accurately the vestibule and inner vulva in most cases. Itching, burning, external dysuria, and dyspareunia are all common. While the presence of a curdy white discharge is quite sensitive for candida, the absence of this sign does not rule out the infection. It is important to question the patient about possible attempts at self-treatment, because the recent application of over-the-counter antifungal creams and suppositories may affect the appearance of the vulva and the vagina. Approximately 90% of vulvovaginal candida infections are due to Candida albicans but nonalbicans species, particularly Candida glabrata, are becoming increasingly common (5).
While these disorders are typically described as vaginal infections, they may be associated with vulvar symptoms such as itching, burning, external dysuria, and dys-pareunia. Therefore, thorough evaluation of vaginal pH and wet mount is important in all patients with vulvovaginal complaints.
Recent studies suggest that the prevalence of genital herpes virus infection in the U.S. population is approximately 25%, and many of those who are infected are unaware of their status; thus, it should certainly be included in the differential diagnosis of any vulvar complaint (6). Primary infection usually presents with multiple extensive and painful vesicles that rapidly become shallow ulcers and may be anywhere on the vulva as well as vagina and cervix. Patients may also have systemic symptoms of viral infection such as fever, fatigue, and myalgias. Recurrent infections are typically less severe with fewer lesions and no systemic complaints. Some patients report prodromal tingling or itching prior to the appearance of lesions, but others may be asymptomatic. While the possibility of herpes infection is usually quite distressing to the patient, testing for herpes simplex virus (HSV) should be considered in the evaluation of any vulvar blister, ulcer, or fissure of uncertain etiology.
Condylomata acuminata or genital warts are caused by the human papilloma virus (HPV). Patients may complain of vulvar irritation or itching and typically note the appearance of multiple small warty lesions of the vestibule, inner labia minora, and perianal region.
While less common than HSV and condylomata, many other sexually transmitted infections may present with a vulvar lesion. The lesion of primary syphilis is typically a painless erythematous ulcer with a smooth base and well-defined edges. Granuloma inguinale presents with multiple erythematous granulomatous ulcers that are usually quite friable. Chancroid, caused by the bacterium Hemophilus ducreyi, usually presents as either a pustule with surrounding erythema or a tender vulvar ulcer with a ragged edge and necrotic base. Lymphogranuloma venereum is seen mainly in tropical climates and presents initially with a small papule or ulcer, and then progresses to involvement of the inguinal lymph nodes.
Folliculitis, Cellulitis, and Abscesses
Because of the nature of the vulvar skin with numerous follicles and glands, these infections are not uncommon on the vulva. Personal hygiene practices such as shaving and application of powders may exacerbate these conditions in susceptible women. Poorly controlled diabetes mellitus and immune suppression from AIDS or other conditions also increase the risk. Small pustules or furuncles are frequently seen and patients should be encouraged to avoid picking at these lesions because it can worsen the infection. Infections and blockage of the ducts of the Bartholin's glands, Skene's glands, or minor vestibular glands will result in abscess formation, which necessitates incision and drainage or marsupialization as well as antibiotic therapy. Cellulitis of the vulva may result from the spread of any of the above conditions as well as following repair of obstetrical lacerations or other surgical procedures of the vulva. While rare, necrotizing fasciitis of the vulva should be suspected in the patient with signs of sepsis in the presence of cellulitis (7). Hidradenitis suppurativa is a chronic inflammatory condition of the apocrine glands, which can involve the groin area in addition to the axillary regions. Draining sinus tracts will be present along with local induration and scarring of the skin of the labia majora, mons, genitocrural folds, and buttocks.
Cysts, nodules, and tumors of a variety of types may be present on the vulva and patients will typically report the presence of "a lump down there'' when presenting for evaluation. Sebaceous and epidermal inclusion cysts are frequently seen on the skin of the labia majora and minora, and in the vestibule. Bartholin's duct and vestibular cysts are also quite common. A urethral diverticulum or Skene's duct cyst may also present as a vulvar mass. Lipomas and fibromas may develop in the labia majora or mons. Leiomyoma and endometriomas are less commonly seen. Other benign lesions such as acrochordons, seborrheic keratoses, and nevi may also be found on the vulvar skin.
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