Many nonmalignant vulvar skin disorders present with the same symptoms as premalignant or malignant vulvar conditions. A skin biopsy will result in a definitive diagnosis via histopathology; however, it requires technical skill and may result in scarring. The original classification system of nonneoplastic dermatologic conditions of the vulva by the International Society for the Study of Vulvovaginal Disease (ISSVD) was based on gross description and histopathology (Table 22.2). However, in 2011, the ISSVD presented a stepwise diagnostic approach that aims to help providers make a diagnosis based on clinical presentation. The classification was based on pathophysiology, etiology, and commonalities among clinical presentation. The goal of the ISSVD was to simplify the terminology in order to arrive at a differential diagnosis. In contrast to the original classification of vulvovaginal skin diseases, the most recent classification aims to diagnose without a biopsy. However, if a biopsy is necessary, it should be performed by a health-care provider trained in the procedure and ideally interpreted by a dermatopathologist (pathologist with specialty training in dermatological disorders) whenever possible.

The classification of vulvar disorders is shown in Table 22.2.

All women, especially postmenopausal women, should be asked about symptoms related to vulvovaginal atrophy and vulvar skin conditions. These symptoms include vulvar itching, burning, bleeding, changes in pigmentation, sores, lumps, ulcers, or pain with intercourse. When present, symptom duration, intensity, and modifying factors should be assessed. All vulvovaginal contacts should be reviewed, including over-the-counter remedies, soaps, shampoos, and sanitary products. These topical irritants should be eliminated. See Table 22.3 for a list of common irritants and possible suggestions for their removal.

For intermittent symptoms, contact/symptom diaries can be recommended. Skin conditions elsewhere, such as oral lesions or eczema should be documented. Systemic disease and all medications should be reviewed.

The ISVVD 2012 publication’s diagnostic approach to vulvar disorders represents a philosophical return to the physical exam. Table 22.4 offers descriptive terms useful for universal documentation of vulvar lesions and clear communication with specialists.

Physical exam of the vulva begins with visual inspection. Optimal patient positioning is in dorsal lithotomy position in gynecological stirrups, although a frog-legged position on a non-gyn examination table also may be used. Lighting should be adequate. A magnifying glass can aid visualization, but is not always necessary. Acetic acid application is not recommended because its accuracy in enhancing vulvar lesions has not been validated.

Visual inspection should include assessment of aging changes in the vulvovaginal area. Loss of hair, thinning skin, increase or loss of pigmentation should be noted. Ulcerations, excoriations, or any lesions should be noted and documented as specifically as possible in the patient chart. Localization, symmetry, and number and distribution of abnormal areas should be recorded. Border asymmetry, color, and size are also key elements to note. A cotton swab can be used to diagnose provoked vestibulodynia (through elicitation of pain with point contact through the vestibule).

The surface appearance of a lesion provides clinically helpful information. If the surface is rough, it may be due to either a crust or a scale. A crust commonly appears yellow or hemosiderin colored, and its presence implies a lesion that has involved excoriation, erosion, or any type of disruption of the epithelial layer. A scale is characterized by reactive keratinization, such as in eczema.

Lichenification refers to the process of cutaneous thickening that occurs secondary to chronic itching or rubbing contact. As a dermatological description, it lacks specificity for the naked eye or magnifying glass. The skin may be erythematous (red), white, or skin colored. It may or may not have excoriations. Histologically, lichenification appears as a thickened epidermal layer.

Properties that may indicate an allergic reaction include red plaques and evidence of itching, such as excoriations. A clinical history of sensitive skin, seasonal allergies, and/or asthma suggests a person with “atopy.” Atopic or contact dermatitis may be causing or contributing to the chronic itching.

Inflammation typically includes the appearance of excoriations and erythema (representing reactive microvasculature). Eczematous conditions are related to atopic dermatitis but typically occur without contact irritants.

Differentiating a premalignant condition from vulvar carcinoma is difficult based on appearance alone. A biopsy is recommended in cases of nonhealing erosions, ulcerative lesions, hyperpigmented lesions, or any vulvar lesion demonstrating lack of response to treatment.

A trained health-care provider can perform a vulvar punch biopsy in the office. The site is prepped with an antibacterial solution, and 1 or 2 % lidocaine is injected subcutaneously. A punch biopsy instrument is pressed against the skin in a circular fashion to penetrate the dermis. The biopsy site should include the most abnormal appearing skin lesion as well as an adjacent potion of normal appearing skin (the very edge of the lesion). Multiple biopsy sites may be needed. Forceps and scissors are used to remove the sample. Direct pressure, Monsel solution, silver nitrate, or suture may be utilized for hemostasis. Post-biopsy care includes keeping the area clean and dry, although a topical antibiotic ointment may be prescribed.

General principles of therapy include the removal of topical irritants, focus on skin hygiene, and anti-inflammatory therapy, usually in the form of topical steroid cream. Systemic treatment for vulvar dysplasias or dermatoses is prescribed for severe, recurrent or resistant disease, or in an immunocompromised individual.

Additionally, in a patient with concomitant vulvovaginal atrophy, the addition of local estrogen therapy, in the absence of contraindications, should be considered as an adjunct to any specific therapies. Vaginal creams, rings, or tablets may be used. If VIN or cancer is present on biopsy, referral to a gynecologic oncologist is recommended.

Nonneoplastic epithelial lesions fall into one of the following three categories: (1) lichen sclerosis, (2) squamous cell hyperplasia/lichen simplex chronicus, and (3) other dermatoses.