Benign Conditions and Congenital Anomalies of the Vulva and Vagina

White Lesions

Conditions that cause whitening of the vulvar epithelium range from processes that result from simple loss of pigment (vitiligo) to those that can profoundly change the architecture of the vulva (lichen sclerosus).

Vitiligo affects 1-2% of the population and is often associated with autoimmune conditions. Its initial presentation can be on the vulva, but it often involves pigment loss on other parts of the body. The diagnosis is made clinically; rarely is biopsy needed. A notable characteristic is the brightness of the depigmented areas amplified by increased pigment in the adjacent unaffected areas.

Lichen sclerosus may affect men and women of any age, and may involve the skin on any part of the body, but is most commonly found in the anogenital area of midlife women. It often presents with intense pruritus, dyspareunia and burning pain, but can also be an asymptomatic finding. Anal involvement can cause constipation. The skin is white, thin, and inelastic, with a crinkled cigarette-paper appearance on gentle stretch. Widespread excoriation is common. It starts with isolated pearly white papules and plaques that coalesce over time and form scars. The result is a “figure of 8” field of scarring from the area encircling the labia, constricting down around the perineal body and ballooning out again around the perineal area into the gluteal cleft. The clitoral hood scars, burying the clitoris ( Figure 18-1 ). There is shrinkage or loss of the labia minora, contraction of the tissue in the vestibule and introitus, and scarring around the anus. Painful, bleeding fissures are common, especially in areas where the inelastic epithelium is put under tension, such as with attempted coitus (introital trauma) or defecation (perianal trauma). Biopsy demonstrates characteristic loss of rete ridges, a thin epidermis, and a mixed lymphocytic infiltrate lining the basement membrane. The process can be arrested by treatment with higher potency corticosteroids, but recurrence is not uncommon and up to 10% of untreated and 3% of treated women eventually develop squamous cell carcinoma of the vulva. The histologic features of lichen sclerosus are illustrated in Figure 18-2, A .

Lichen planus is an inflammatory, autoimmune process that involves epithelial changes on the vulva, or in the vagina and the mouth. Symptoms include severe burning, irritation, and dyspareunia. It induces an intense erythema and erosions on the vulva, which is often surrounded by reticulate white striae (Wickham striae) that appear as classic fernlike or lacy patterns. These same findings are commonly found on the mucous membranes of the mouth. Scarring, especially in the vaginal vault, is common.

Lichen simplex chronicus (referred to as squamous hyperplasia histologically), represents thickening of the epithelium, which usually results from rubbing or scratching in the absence of an underlying dermatosis. It generally appears on keratinized skin as white plaques or darker red areas; the surface is often marked with excoriations. It can often mimic other processes, such as psoriasis, lichen planus or lichen sclerosus, so biopsy is indicated. Histologically, the rete ridges deepen with hyperkeratosis of the superficial layer of the epidermis (see Figure 18-2, B ). Intermediate potency topical corticosteroids are the cornerstone of treatment.

Vulvovaginal atrophy occurs in many postmenopausal women with the loss of ovarian production of estradiol (E2). The subcutaneous fat in the labia majora is diminished and the labia minora also shrink. The epithelium thins, appears white, and is less elastic. The vaginal introitus can constrict, creating an almost prepubertal caliber, which may make coitus uncomfortable. Estrogen creams are usually effective. Moisturizers can help women who are not candidates for hormonal therapies. Lubricants can be used for intercourse, if needed.

Red Lesions

Many of the epithelial lesions on the vulva can be accompanied by erythema, and many infectious processes, such as candidal infections, can appear red. However, the lesions included in this category represent benign underlying dermatologic changes that present primarily as red lesions. These include eczema, seborrheic dermatitis, and psoriasis. In the genitalia, the characteristics of many of these processes are altered by the moisture and heat in the area, friction from clothing or scratching, and secondary infection or irritation from sweat, urine, stool, or overcleansing. Fecal or urinary incontinence may cause irritation and can accelerate skin breakdown due to other etiologies.

Eczema is a term that is broadly applied to a wide range of chronic skin conditions characterized by rashes with dryness, swelling, itching, as well as crusting, flaking, cracking, blistering, oozing, or frank bleeding. The reddish appearance of eczema in the vulva is profoundly influenced by rubbing and scratching, which produce thickening and excoriation, respectively. The history of pruritus, relieved by scratching, and the shape of the lesions help with the diagnosis, but other etiologies must be ruled out first. One of the most common subtypes of eczema on the vulva is contact dermatitis. Contact dermatitis (either irritant or allergic) is increasing in frequency on the vulva with the use of a wide range of products on the genital tissue. Common irritants include overwashing, female hygiene products, and topical medications. These same medications, as well as chemicals found in sanitary protection products, can induce allergic reactions on the vulva with widespread erythema and even edema.

Seborrheic dermatitis causes a dry or greasy peeling, reminiscent of dandruff in the scalp, face, or eyebrows. In the genital area, the lesions tend to concentrate in the skin folds, with less prominent lesion borders or scaling. The skin has a red glazed, shiny texture, occasionally with scaling plaques, in the absence of candida or psoriasis. A diagnosis of seborrheic dermatitis is strongly supported by finding characteristic lesions elsewhere.

Psoriasis is a common chronic relapsing immune-mediated skin disease affecting 2-3% of the population. Psoriasis may involve the entire genital area, including the gluteal cleft, but there may be less extensive involvement. Both the skin fold and hair-bearing areas may be involved. Red plaques with clear borders are the hallmark of classic genital psoriasis, but the silvery-white scales are generally less prominent on the genitalia. Itching is a frequent complaint. Involvement of knees, elbows, and nails helps to make the diagnosis. Pustular psoriasis can be confused with candidal infection.