I VULVAR SKIN DISORDERS ^427 ^623 ^657

In women who report symptoms of vulvar disorders, the most common diagnoses are dermatologic conditions and vulvodynia. Vulvar pruritus and vulvar pain may occur in the presence of obvious dermatologic dis­ease or in conditions with few visible skin changes.

Evaluation

In evaluating vulvar pruritus, it can be helpful to group women into those conditions with acute symptoms and those with chronic symptoms (see Box 4-6). In cases of acute vulvar pruritus, common etiologies include vulvovaginal candidiasis and contact dermatitis. Chronic vulvar pruritus should prompt a search for underlying dermatoses, such as lichen sclero- sus, lichen simplex chronicus, or psoriasis; neoplasia; or vulvar manifes­tations of systemic disease. Patients presenting with pain should first be evaluated to rule out underlying organic causes, including inflammatory conditions, neoplasia, infections, or neurologic disorders. When organic causes are ruled out, the diagnosis of vulvodynia can be made (see also “Chronic Gynecologic Pain” earlier in Part 4).

During evaluation, the medical history should include questions about the onset, duration, location, and nature of vulvar symptoms, as well as possible precipitating or known risk factors. The vulva and vagina should be carefully inspected as part of the pelvic examination. Microscopy of vaginal secretions, using saline and potassium hydroxide preparations in conjunction with vaginal pH determination, will help evaluate for infec­tious causes.

For autoimmune disorders, or in suspected cases of vulvar dermatoses (eg, lichen sclerosus and lichen planus) or neoplasia, a biopsy may be

Box 4-6. Conditions Commonly Associated With Vulvar Pruritus ^

Acute

• Infections

— Fungal, including candidiasis and tinea cruris

— Vulvovaginal candidiasis

— Trichomoniasis

— Molluscum contagiosum

— Infestations, including scabies and pediculosis

• Contact dermatitis (allergic or irritant)

Chronic

• Dermatoses

— Atopic and contact dermatitis

— Lichen sclerosus, lichen planus, lichen simplex chronicus

— Psoriasis

— Genital atrophy

• Neoplasia

— Vulvar intraepithelial neoplasia, vulvar cancer

— Paget disease

• Infection

— Vulvovaginal candidiasis, recurrent

— Human papillomavirus infection

• Vulvar manifestations of systemic disease

— Crohn disease

Modified from Diagnosis and management of vulvar skin disorders. ACOG Practice Bulletin No. 93. American College of Obstetricians and Gynecologists. Obstet Gynecol 2008;111:1243-53.

necessary for diagnosis. Findings, such as thickening, pebbling, hypopig­mentation, or thinning of the epithelium, indicate a possible dermato­logic process, and biopsy will aid in diagnosis and management. Biopsy of hyperpigmented or exophytic lesions, lesions with changes in vascular patterns, or unresolving lesions is particularly important and should be performed in order to rule out carcinoma. Diagnostic delays in identifying vulvar cancer are exceedingly common and have been linked to failures or procrastination in the performance of biopsies of abnormal-appearing vulvar skin.

Diagnosis and Management

Treatments of acute and chronic vulvar skin disorders are targeted toward the specific cause of the symptoms, based on history and physical exami­nation, evaluation of vaginal secretions, cultures, and biopsy. Chronic or recurrent forms of vulvovaginal disease can be difficult to diagnose and treat.

Vulvar Dermatoses

Lichen Sclerosus

A chronic disorder of the skin, lichen sclerosus is most commonly seen on the vulva, with extragenital lesions reported in up to 13% of women with vulvar disease. Patients presenting with lichen sclerosus most com­monly report pruritus, followed by irritation, burning, dyspareunia, and tearing. On examination, typical lesions of lichen sclerosus are porcelain­white papules and plaques, often with areas of ecchymosis or purpura. The skin commonly appears thinned, whitened, and crinkling (leading to the description “cigarette paper”). Because other vulvar diseases can mimic lichen sclerosus, a biopsy is necessary to confirm the diagnosis, except in a prepubertal child.

Treatment for lichen sclerosus involves chronic treatment with a topical high-potency steroid, such as clobetasol propionate. A reasonable approach is to begin with once-daily application of ultrapotent topical steroids for 4 weeks, tapering to alternate days for 4 weeks, followed by 4 weeks of twice weekly application. Monitoring at 3 months and 6 months following initial therapy is recommended to assess the patient’s response to therapy and to ensure proper application of the medication. Annual examinations are suggested for patients whose lichen sclerosus is well controlled, with more frequent visits for those with poorly controlled disease (for whom intralesional steroid injections also may be beneficial).

Lichen Planus

Lichen planus, an inflammatory disorder of the genital mucosa most likely related to cell-mediated immunity, exhibits a wide range of morphologies. The most common form and the most difficult to treat is the erosive form, which can lead to significant scarring and pain. The classic presentation of lichen planus on mucous membranes, including the buccal mucosa, is that of white, reticulate, lacy, or fernlike striae (Wickham striae). In erosive lichen planus, deep, painful, erythematous erosions appear in the posterior vestibule and often extend to the labia minora, which results in aggluti­nation and resorption of the labial architecture.

Vulvovaginal lichen planus is a chronic, recurring disease that requires long-term maintenance. Although symptomatic improvement is possible, patients should be advised that complete control is not the norm. Treatment options include topical and systemic corticosteroids, topical and oral cyclo­sporine, topical tacrolimus, hydroxychloroquine, oral retinoids, methotrex­ate, azathioprine, and cyclophosphamide. In some cases, consultation with a dermatology colleague for long-term management may be appropriate.

Symptomatic Vulvar and Vaginal Atrophy

It is estimated that up to 50% of all postmenopausal women experience vulvovaginal irritation, soreness, and dryness; lower urinary tract symp­toms; recurrent cystitis; and dyspareunia. As women approach menopause, vulvar tissue becomes increasingly sensitive to irritants, and, in the absence of estrogen, the vaginal mucosa becomes pale, thin, and often dry. Vaginal pH becomes more alkaline and the vaginal flora is altered. Depending on symptomatology and after any indicated pathologic evaluation has been obtained, management options for urogenital atrophy in adult women include lifestyle modification strategies (eg, maintaining regular vaginal intercourse or masturbation), the use of vaginal moisturizers, and low-dose topical estradiol preparations. In 2013, a new oral medication, ospemifene, was approved by the U.S. Food and Drug Administration for the treatment of dyspareunia in postmenopausal women. Ospemifene is an estrogen agonist/antagonist that acts similarly to estrogen by increasing vaginal wall thickness, which results in a reduction in the amount of pain women expe­rience with sexual intercourse.

Vulvar Intraepithelial Neoplasia

Vulvar intraepithelial neoplasia (VIN) is an increasingly common prob­lem, particularly among women in their 40s. The term VIN is used to denote high-grade squamous lesions and is subdivided into usual-type VIN (including warty, basaloid, and mixed VIN) and differentiated VIN.

Treatment is indicated for all cases of VIN. Wide local excision is recom­mended when cancer is suspected, despite a biopsy diagnosis of only VIN, to identify occult invasion. When occult invasion is not a concern, VIN can be treated with excision, laser ablation, or topical imiquimod (off-label use). Women with VIN should be considered at risk of recurrent VIN and vulvar cancer throughout their lifetimes. After resolution, women should be monitored at 6 months and 12 months and annually thereafter.

Bibliography

Diagnosis and management of vulvar skin disorders. ACOG Practice Bulletin No. 93. American College of Obstetricians and Gynecologists. Obstet Gynecol 2008;111:1243-53. [PubMed] [Obstetrics & Gynecology]

Food and Drug Administration. FDA approves Osphena for postmenopausal women experiencing pain during sex. Silver Spring (MD): FDA; 2013. Available at: http://www.fda.gov/newsevents/newsroom/pressannouncements/ucm341128. htm. Retrieved September 30, 2013.

Management of vulvar intraepithelial neoplasia. Committee Opinion No. 509. American College Obstetricians and Gynecologists. Obstet Gynecol 2011;118: 1192-4. [PubMed] [Obstetrics & Gynecology]

Resources

American College of Obstetricians and Gynecologists. Disorders of the vulva. Patient Education Pamphlet AP088. Washington, DC: American College of Obstetricians and Gynecologists; 2013.

British Association for Sexual Health and HIV. 2007 UK national guideline on the management of vulval conditions. Cheshire (UK): BASHH; 2007. Available at: http://www.bashh.org/documents/113/113.pdf. Retrieved August 9, 2013.

Rutanen EM, Heikkinen J, Halonen K, Komi J, Lammintausta R, Ylikorkala O. Effects of ospemifene, a novel SERM, on hormones, genital tract, climacteric symp­toms, and quality of life in postmenopausal women: a double-blind, randomized trial. Menopause 2003;10:433-9. [PubMed]