The appearance of a male-type escutcheon (see Chapter 7
) can be a virilizing sign. However, there is normally a continuum, and a family history may be helpful. Terminal1
hair was found on the abdomen (above the pubic triangle) of 35% of 400 English and Welsh university women (McKnight, 1964
In patients afflicted with pubic lice, nits may be seen at the base of the hairs, along with signs of excoriation of the skin.
The skin covering the labia majora may be afflicted with the same lesions as the skin of the rest of the body, including malignant melanomas, psoriasis, and seborrheic dermatitis. The labia are particularly rich in sebaceous glands, and hence are subject to sebaceous retention cysts and hair follicle infections. The apocrine glands may be afflicted with hidradenitis suppurativa (Fox-Fordyce
disease). Also check for condylomata and lesions of molluscum contagiosum.
Because the labia majora are the analog of the scrotum, the occurrence of labioinguinal hernias, although rare, should not be surprising. In certain hermaphrodites, testicles are found in the labia majora.
An abscess of the Bartholin2 gland, when fully formed, is an obvious tender red mass in the posterior labium. Patients will walk in a peculiar way and complain of severe pain. However, considerable enlargement of the gland (as from a retention cyst or rarely an adenocarcinoma) can be missed unless it is searched for. Palpate the posterior part of the labia majora between the finger and thumb, searching for a swelling. In patients with a Bartholin abscess, there is a high incidence of sexually transmitted disease (STD); Gram stain and cultures for gonorrhea and Chlamydia should be performed, and diagnostic tests for syphilis, human immunodeficiency virus (HIV), and hepatitis B should be considered.
Hypertrophy of the labia majora occurs in lipodystrophy.
Simple adherence of the labia minora (labial agglutination or labial adhesions) in young girls is to be distinguished from imperforate hymen and congenital absence of the vagina. In one study, the incidence was fivefold higher (2.9%) in children who were proven victims of sexual abuse (Muram, 1988
). However, in a study of prepubertal girls selected for nonabuse, labial adhesions were detected in 35 of 90 subjects. More than 50% of these adhesions were less than 2 mm in length and detected only on review of magnified colposcopic images (McCann et al., 1990
Check for condylomata, herpetic lesions, and erythema.
Enlargement of the clitoris is an unmistakable sign of virilization. The adult clitoral index, defined as the vertical times the horizontal dimensions, is normally from 9 to 35 mm (Rittmaster and Loriaux, 1987). Borderline values, often seen in idiopathic hirsutism, are from 36 to 99 mm. If the index is 100 mm or more, it is a sign of severe hyperandrogenicity (Tagatz et al., 1979
), and demands an explanation. In the infant, clitoral enlargement may signal an adrenogenital syndrome or a maternal ovarian tumor.
A purulent discharge from the urethra can result from gonorrhea, a urethral diverticulum, or another cause of urethritis. Sometimes the discharge is apparent only after stroking the anterior vaginal wall in the direction of the meatus. A urethral caruncle, a tender, inflamed red mass at the meatus, may be a complication of urethritis. A prolapsed urethra may form a hemorrhagic, painful mass, superficially resembling a cancer because of its friability. The condition may occur in children or elderly women, especially after straining. In gonorrhea, pus may be expressed from the ducts of Skene glands located just lateral and somewhat posterior to the urethral orifice. They may be quite difficult to see.
. A special maneuver in a woman complaining of urinary incontinence is to place a lubricated cotton swab in the urethra to the level of the bladder neck while she is in the lithotomy position. Ask her to perform a Valsalva maneuver, and measure the change in the axis of the free end of the swab. It should remain horizontal if no anatomical defect is present. A positive test is not very helpful, but a negative one decreases the likelihood of stress incontinence (negative likelihood ratio 0.41). The Q tip test is primarily used by specialists as part of an evaluation for incontinence surgery (Holroyd-Leduc et al., 2008
Inspection of the genitalia should be part of the examination of all infant girls in order to check for the presence of a vagina and for imperforate hymen. In the event of the latter, performance of a rectal examination with the little finger may reveal a bulging in the vagina due to an accumulation of endocervical mucus (hydrocolpos) from maternal hormone secretion. This bulge can become very large and has led to laparotomy for “abdominal mass” (Green, 1971
). An imperforate hymen is an indication for an ultrasound examination.
Imperforate hymen should be ruled out in adolescent girls with abdominal pain. Painful hematocolpos and hematosalpinx, sometimes with rupture into the abdomen, have resulted from failure to recognize this condition before several menstrual periods have occurred. Even before menarche, mucus accumulation behind an imperforate hymen has on rare occasions caused obstruction of the ureters and bilateral hydronephrosis.
An excessive amount of tough, fibrous tissue can be the cause of dyspareunia.
Hymenal changes attributed to sexual abuse are discussed below.
Sexually Transmitted Diseases
Lesions that can occur on the penis (see Chapter 21
) can also occur on the vulva or inside the vagina. These lesions include the primary chancre of syphilis (Fig. 22-4
), the lesion of lymphogranuloma venereum, granuloma inguinale, chancroid, the ulcerating vesicles of herpes, condylomata lata (due to secondary syphilis; Fig. 22-5
), and condylomata acuminata (venereal warts; Fig 22-4
Condylomata acuminata are variably sized, flesh colored to purplish papillomatous growths generally confined to the anogenital region. Giant, nodular lesions with a strawberry-like surface may occasionally be seen in children. Perianal or vulvar lesions may also be associated with warts in the anal canal or in the vagina or cervix.
Condylomata may be flat and visible only with application of dilute acetic acid, or under the colposcope. A careful search is part of the workup for persistent vulvovaginitis; clearing up one condition helps to clear the other (R. Allen, personal communication, 2004).
Condylomata acuminata are caused by the human papilloma virus (HPV), some serotypes of which have been implicated in
the recent increased incidence of cervical carcinoma, especially in younger women (Raymond, 1987a
), as well as vulvar carcinoma (vide infra
). The prevalence of HPV in asymptomatic, sexually active young women is 20% to 40%, using amplified or nonamplified DNA hybridization methods to detect the virus (Ansink, 1996
FIGURE 22-4 Condylomata acuminata and a chancre due to primary syphilis. This is a reminder that patients with one sexually transmitted disease (STD) frequently have one or more others also. (Courtesy of Division of Sexually Transmitted Diseases, Center for Prevention Services, Centers for Disease Control, Atlanta, GA.)
FIGURE 22-5 Vulvar condylomata lata due to secondary syphilis in a child who had been sexually molested. (Courtesy of Division of Sexually Transmitted Diseases, Center for Prevention Services, Centers for Disease Control, Atlanta, GA.)
There is an increased incidence of genital warts in persons seropositive for HIV (Boyd, 1990
). Thus, this lesion suggests the need to be on the alert for accompanying conditions, including sexual abuse in children (vide infra
A diffuse reddening and edema of the vulva may result from the presence of certain types of vaginal discharge (Table 22.1
Gonorrheal vaginitis may be distinguished by its tendency to involve the urethra, the vulvovaginal glands, and the Bartholin and Skene glands.
Evidence of one STD should raise the index of suspicion for others, especially those that are often asymptomatic for long periods, such as Chlamydia. Cultures or even presumptive treatment may be indicated to prevent long-term complications such as infertility.
With increased sexual activity with more partners at younger ages, the prevalence of STDs is very high. More than one in five Americans over the age of 12 is infected with genital herpes, and less than 10% of those who tested positive in a household survey realized that they were infected. Silent spread of the infection is the rule. The prevalence increased by 30% from the late 1970s to early 1990s (Fleming et al., 1997
). Most striking is the increase in young persons: seroprevalence quintupled in white teenagers and doubled in white persons in their twenties (Arvin and Prober, 1997
For further discussion of STDs, see the section on “Vaginal Discharge” below.
Although meticulous use of condoms is advocated, the efficacy of condoms in preventing transmission of all potential infectious agents is not precisely known and it is certainly far from perfect. In one study of participants with known gonococcal or chlamydial exposure, consistent use of condoms effected a reduction in prevalence of gonorrhea and chlamydia from 43% to 30% (Warner et al., 2004
). Data are limited on the effectiveness of female condoms at preventing STDs. In a study of female patients attending an STD clinic, postintervention STD incidence (of early syphilis, gonorrhea, chlamydia, or trichomoniasis) per 100 woman-months of observation was 6.8 in the female condom group and 8.5 in the male condom group. The difference was not statistically significant (French et al., 2003
A review of published studies on the effect of condom use on transmission of HPV found that all methods had significant limitations. Three studies found a protective effect, but most did not (CDC, 2004
Although physicians will inquire about condom use, they should not allow assurances about consistent use to impede a careful search for STDs. Nor should they overstate the effectiveness of condoms when counseling patients about sexual activity. Sexual intercourse is an extremely effective mechanism for transferring biologic material for the perpetuation of the species. No technology has come close to equalling the effectiveness of monogamy, sexual fidelity, and premarital abstinence in curtailing the spread of STDs.
Other Infectious Diseases Affecting the Vulva
The vulva may be involved by parasites such as pinworms (especially in children) or scabies. Impetigo, a staphylococcal and/or streptococcal lesion characterized by yellow-crusted erosions, is a common accompaniment of scabies (Whiting, 1983
Numerous fungi are saprophytes in the vulvar area and may become pathogens under conditions of lowered resistance, increased heat, or friction. Predisposing conditions include pregnancy, diabetes, oral contraceptives, and the use of broad-spectrum antibiotics. The most common fungi are tinea cruris and Candida
(monilia). The rash of tinea cruris tends to have a butterfly
appearance, with clearly defined, raised, scaly borders, also affecting the upper, inner thighs. A curdy, white vaginal discharge is diagnostic of candidal vulvitis. There may be red maculopapular lesions, “satellite lesions,” lying beyond the border of the inflamed area. However, the characteristic discharge is present in fewer than 50% of the cases; more often, there is redness, possibly a watery discharge, and intense discomfort (R. Allen, personal communication
TABLE 22.1 Differential diagnosis of vaginal discharge
Appearance of discharge
Appearance of vulva/vagina
Pseudohyphae on KOH prep.; budding yeast on Gram stain
Green, yellow, gray, may be frothy
Diffuse erythema or “strawberry vagina” or gray pseudomembrane
Motile organisms; many white blood cells (WBCs)
Itching, unpleasant odor or discharge, dysuria
Thin, “flour paste,” frothy in <10%
Usually no gross vulvovaginitis
Clue cells; lactobacilli eliminated; few WBCs unless another infection present
Few have irritation
Bartholinitis, skenitis, pelvic inflammatory disease (PID) may be present
Gram-negative intracellular diplococci
May have dysuria, acute abdominal pain if PID develops
Smooth, shiny, lacking rugae; pale; telangiectasia
Parabasal cells, WBCs, various bacteria, “dirty” background
Burning, itching, dyspareunia
Ulcerations of the vulva and vagina can be caused by Entamoeba histolytica (generally after a bout of uncontrolled diarrhea).
Other Systemic Diseases
Draining sinuses, abscesses, and deep ulcerations may result from Crohn disease. Other conditions causing vulvar ulcers include Behçet disease, pemphigus vulgaris, dermatitis herpetiformis, and erythema multiforme.
After menopause, the labia may shrink and flatten because of the loss of subcutaneous fat. The skin becomes thin and shiny, and elasticity diminishes. Changes of a similar nature generally coexist in the vagina (vide infra), and the vaginal orifice may become stenotic.
Vulvar skin is more sensitive to irritants than peripheral skin, and, additionally, is exposed to a wide variety of potential sensitizers and irritants, such as laundry powders, douches, contraceptive creams, and deodorants (Friedrich, 1985
). A long-standing itchscratch cycle can cause a hyperplastic dystrophy, which is a variant of lichen simplex chronicus (neurodermatitis). This may produce a diffuse erythema, or localized, elevated lesions, often with a white appearance due to hyperkeratosis. Chronic irritation is also thought to cause malignant changes.
Lichen sclerosus (also called lichen sclerosus et atrophicus
) typically produces white to pearly flat macules, which may coalesce into plaques, involving the vulva, medial thigh, and/or perianal region. The skin may come to resemble parchment or cigarette paper. Agglutination and fusion of the interlabial folds and a concentric stenosis of the introitus may occur. Half to two thirds of the patients are postmenopausal, but the disease does occur in children (of whom only 35% have genital lesions). Extragenital sites such as the axilla may be involved. A 34% incidence of clinically manifest autoimmune diseases has been reported in patients with this condition (Soper and Creasman, 1986
Carcinoma develops in about 3% of patients with lichen sclerosus (Soper and Creasman, 1986
). Conversely, lichen sclerosus was an associated lesion in 16 of 30 (53%) patients with invasive vulvar carcinoma (Punnonen et al., 1985
At the time of presentation, 2% to 5% of women with a vulvar dystrophy have an invasive carcinoma of the vulva, and an additional 4% to 8% have some cellular atypia (Soper and Creasman, 1986
Carcinoma In Situ of the Vulva
The lesions of squamous cell carcinoma in situ (sometimes referred to by the ambiguous terms Bowen disease or erythroplasia of Queyrat) can be unifocal or multifocal and discrete or coalescent. About 20% of the lesions are pigmented; the remainder are white or red. The relationship of the lesions to invasive carcinoma is not as strong as that with cervical carcinoma in situ. Previously considered
a disease of older women, there is an increasing incidence in younger women, with cases occurring in women as young as 17 (Al-Ghamdi et al., 2002
) years. Between the periods 1985-1988 and 1994-1997, the incidence of high-grade vulvar intraepithelial neoplasia in women aged 50 or under increased by 392%, and the incidence of invasive vulvar cancer by 157% (Joura et al., 2000
). Most but not all tumors are associated with HPV. Suspicious lesions must be biopsied.
Although formerly considered synonymous with carcinoma in situ, Paget disease of the vulva is a separate entity (Nichols and Evrard, 1985
), an intraepithelial adenocarcinoma, occasionally associated with an underlying invasive adenocarcinoma. It presents as a sharply demarcated, florid, red, pruritic, moist area, with occasional crusting. Islands of whitened skin appear between the reddened areas. The lesion may spread to the perineum and thighs.
Invasive Carcinoma of the Vulva
Vulvar cancer is extremely variable in appearance. In its early form, it may be an elevated papule or a small ulcer and may be easily confused with condyloma acuminatum, papillomata, ulcerated chancroid, gumma, or tuberculosis. (Thus, biopsy is very important.) A typical later lesion is an ulcerating mass. Previously, about 70% of patients were postmenopausal (Kistner, 1986
), but younger women are now more commonly afflicted, consequent to changes in sexual mores (vide supra
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