Diagnosis and Management of Vulvar Skin Disorders Reviewed  CME

News Author: Laurie Barclay, MD
CME Author: Charles Vega, MD
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Physicians - maximum of 0.25 AMA PRA Category 1 Credit(s)™ for physicians; Family Physicians - up to 0.25 AAFP Prescribed credit(s) for physicians

May 5, 2008 — The American College of Obstetricians and Gynecologists (ACOG) has issued a practice bulletin reviewing available diagnostic methods and management strategies for vulvar skin disorders. The new recommendations are reported in the May issue of Obstetrics & Gynecology.

"Symptoms of vulvovaginal disorders are common, often chronic, and can significantly interfere with women's sexual function and sense of well-being," write Lori A.,Boardman, MD, ScM, and colleagues from ACOG. "In the evaluation of women who report symptoms of vulvar disorders, the most common diagnoses are dermatologic conditions and vulvodynia (both generalized and localized forms). The purpose of this document is to review diagnostic approaches and provide a structured framework for the management of vulvar disorders."

The most common presenting symptoms of vulvar disorders are pruritus and pain, with or without visible changes. Vulvodynia is defined as burning, stinging, rawness, or soreness. Characterizing vulvodynia by location, by whether it is generalized or localized, and whether it is spontaneous or provoked may aid in diagnosis.

Acute conditions often associated with vulvar pruritis include allergic or irritant contact dermatitis and infections. Fungal infections include candidiasis and tinea cruris; other infectious causes are trichomoniasis, vulvovaginal candidiasis, molluscum contagiosum, and scabies and pediculosis infestations.

Chronic causes of vulvar pruritis include dermatoses such as atopic and contact dermatitis, lichen sclerosus, lichen planus, lichen simplex chronicus, psoriasis, and genital atrophy. Neoplastic causes are vulvar intraepithelial neoplasia, vulvar cancer, and Paget's disease. Human papillomavirus (HPV) infection is an infectious cause, and Crohn's disease is a vulvar manifestation of systemic disease.

Common vulvar irritants and allergens that may cause allergic or irritant contact dermatitis include adult or baby wipes, antiseptics, body fluids, colored or scented toilet paper, condoms, vaginal contraceptives and lubricants, dyes, emollients, detergents and fabric softeners, rubber and latex, female hygiene pads or tampons, soaps and shampoos, tea tree oil, and vaginal hygiene products.

Topical medications that can also cause allergic or irritant contact dermatitis include anesthetics, antibacterials, antimycotics, corticosteroids, trichloroacetic acid, 5-fluorouracil, podofilox, and podophyllin.

Unless labial adhesions cause symptoms such as urinary obstruction, they should be observed, with topical estrogen cream as first-line treatment. Girls should be monitored for adverse effects of estrogen therapy, such as breast budding and vaginal bleeding.

Estrogen treatment is also useful for vaginal atrophy. Management options include lifestyle modification strategies, use of vaginal moisturizers, and low-dose topical estradiol preparations.

Although vulvovaginal lichen planus is unlikely to remit spontaneously, treatment options include topical or systemic steroids, topical or oral cyclosporine, topical tacrolimus, hydroxychloroquine, oral retinoids, methotrexate, azathioprine, and cyclophosphamide. This chronic, recurring condition requires long-term maintenance therapy.

Specific clinical recommendations, and their accompanying level of evidence, are as follows:

• For lichen sclerosus, an ultrapotent topical corticosteroid, such as clobetasol propionate, is recommended treatment (level of evidence, B).
• Biopsy is recommended to rule out carcinoma when lesions are hyperpigmented, exophytic, unresolved, or have changes in vascular patterns (level of evidence, C).
• Patients with biopsy-confirmed Paget's disease should undergo further evaluation of the breast, genitourinary tract, and gastrointestinal tract (level of evidence, C).

A proposed performance measure is the percentage of women with lichen sclerosus who are offered high-potency topical corticosteroids as first-line treatment.

"Genital HPV, the most common sexually transmitted viral infection, is associated with a number of vulvar epithelial disorders, including genital warts, vulvar intraepithelial neoplasia (VIN), and some vulvar carcinomas," the authors of the bulletin write. "Distinguishing warts from vulvar neoplasia on the basis of appearance alone is not always possible because VIN can present as red, white, dark, raised, or eroded lesions. In general, a biopsy should be performed on hyperpigmented, indurated, fixed, or ulcerative lesions, or lesions that do not respond to treatment or worsen during treatment."

Obstet Gynecol. 2008;111:1243-1254.

Clinical Context

Vulvar skin disorders are usually localized and are benign, although systemic disease can be associated with vulvar lesions. Approximately one third of women with Crohn's disease experience gynecologic complications, including vulvar inflammation. Paget's disease is a rare form of intraepithelial neoplasia that accounts for approximately 2% of vulvar neoplasms. Paget's disease is also associated with a significant risk for other types of cancer, and a diagnosis of vulvar Paget's disease should prompt evaluation for tumors of the breast, genitourinary tract, and gastrointestinal tract.

The current ACOG Practice Bulletin focuses on the diagnosis and management of more common vulvar skin disorders.

Study Highlights

• For vulvar lesions that require biopsy, a punch biopsy is the usual preferred method. The leading edge of an ulcerative lesion should be sampled, whereas a biopsy of the thickest portion of hyperpigmented lesions is recommended.
• Topical estrogen cream is the first-line medical treatment of labial adhesions, and surgery is usually only required in cases of acute urinary retention.
• Dermatitis can be present in 20% to 60% of women with chronic vulvar symptoms. Clinical signs of vulvar dermatitis can vary from mild erythema and swelling to fissures, erosions, and ulcers.
• Many cases of vulvar dermatitis improve by reducing exposure to potential irritants and allergens, including vaginal hygiene products, soaps, and even emollients.
• Vulvar atrophy affects approximately half of women after menopause, and many women continue to have vulvovaginal symptoms despite the use of systemic hormone therapy. Regular vaginal intercourse can protect against urogenital atrophy by increasing blood flow to the pelvic organs. Nonhormonal vaginal moisturizers, but not vaginal lubricants, might be as effective as local hormone therapy to reduce symptoms of vulvar atrophy.
• Lichen simplex chronicus usually occurs in midlife or later, and most patients with lichen simplex chronicus have a history of atopic disease. In longstanding cases of lichen simplex chronicus, the skin can be thick and leathery.
• Lichen sclerosus is most commonly seen on the vulva, and the mean age of onset occurs during the fifth to sixth decade. Clinical examination reveals porcelain-white papules and plaques as well as thin, crinkled "cigarette paper" epithelium. Women with perianal involvement can have a "figure-of-eight" distribution of skin lesions.
• Lichen sclerosus should be treated with high-potency topical steroids such as clobetasol propionate, which can reduce symptoms in 96% of patients. Once-daily application of corticosteroid cream is usually sufficient, and long-term maintenance therapy with topical corticosteroids has generally not been associated with significant skin damage. The decision whether to use maintenance therapy vs as-needed topical therapy is best made on a case-by-case basis.
• Women with lichen sclerosus should undergo periodic surveillance of vulvar lesions because there is a 5% incidence of squamous cell carcinoma among these patients.
• Lichen planus can present in different morphologic states, but the most common form is the erosive form. Erosive lichen planus promotes deep, painful, erythematous erosions on the posterior vestibule, and these lesions often extend to the labia. The classic presentation of lichen planus is that of white, reticulate, lacy, or fernlike striae.
• Women should be advised that complete control of lichen planus is unusual, and most patients experience chronic and recurring disease. The most frequently recommended initial treatments of lichen plus are high-potency topical steroids. Other treatments include topical or oral cyclosporine, topical tacrolimus, and oral immunosuppressants.

Pearls for Practice

• Among vulvar skin disorders, the presence of white, reticulate, lacy, or fernlike striae suggests lichen planus, whereas porcelain-white papules and plaques as well as thin, crinkled "cigarette paper" epithelium suggest lichen sclerosus. Erosive lichen planus is characterized by deep erosions on the posterior vestibule.
• High-potency topical corticosteroids are the first-line treatment of vulvar lichen sclerosus and lichen planus.

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