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Follow-Up Is Key in Vulvovaginal Lichen Planus


 

CHICAGO — Lichen planus is a tough disorder to treat and requires a strong commitment to patient education and follow-up, Dr. Lynette J. Margesson said at a conference on vulvovaginal diseases.

Lichen planus is an inflammatory, lymphocyte-mediated mucocutaneous disorder of an unknown cause that is relatively uncommon in the vulvovaginal area, and is too often missed. It usually involves the skin and less commonly the oral mucosa, scalp, nails, eyes, esophagus, bladder, larynx, and anus.

About 1% of the population has oral lichen planus. Recent studies have shown that up to 70% of women with oral lichen planus have genital involvement. Up to 44% can be asymptomatic, said Dr. Margesson of Dartmouth Medical School, Hanover, N.H.

Complications include scarring, loss of any or all of the vulvar structures, narrowing of the introitus, psychosexual problems, and synechiae and scarring that can eventually obliterate the vaginal space. Vulvar lichen planus is often confused with lichen sclerosus and, like that disease, is associated with an increased risk (3%–4%) of squamous cell carcinoma.

Prognosis is notoriously unpredictable, with about 75% of patients having improvement in symptoms and 10% having resolution of clinical signs without scarring, Dr. Margesson said at the conference, which was sponsored by the American Society for Colposcopy and Cervical Pathology. Patient education needs to be optimistic, even though there is often no “cure.” Some patients can go into remission permanently, but most have a tendency to relapse. Long-term follow-up is needed for compliance and cancer surveillance.

“Noncompliance is a big problem,” Dr. Margesson said. “They come back and their vulva is dying.”

Upon presentation, most patients complain of soreness, pain, and itching, which can be mild to moderate in the papular form and severe in the hypertrophic form.

On physical exam, the classic pattern on dry, keratinized skin includes raised, reddish brown to purple, well-defined, dry papules with fine lacy white streaks, referred to as Wickham's striae, on the surface.

Erosive lichen planus often shows deep red, glazed lesions around the posterior vestibule with associated loss of architecture and scarring. A whitish scalloped or reticulated edge to these lesions is a classic feature that is not present in lichen sclerosus and can help make the diagnosis of lichen planus, Dr. Margesson said. Oral involvement is seen in about 60% of these patients, with the same lacy reticulated pattern, plus varying degrees of ulceration and erosion present on the buccal or gingival mucosa and tongue. The hypertrophic form is the least common, and looks very much like thick lichen sclerosus on the vulva, with thickened, white, scarred skin and loss of normal architecture.

Regular histopathology biopsies are often unreliable, so Dr. Margesson recommends performing biopsies for both histopathology and direct immunofluorescence.

Nonspecific treatments include education and support, stopping all irritants, restoring the epidermal barrier, and offering psychosexual support, as these patients are usually sexually dysfunctional, she said.

Topical corticosteroids to suppress inflammation are the cornerstone of treatment. Dr. Margesson prefers clobetasol or halobetasol 0.05% ointment used very sparingly daily in a thin film for 8–12 weeks for the mucous membranes of the vulva, and 1–3 times a week for maintenance. Topical tacrolimus (Protopic) 0.1% ointment can be helpful, but this is an off-label use, and it should be applied sparingly as it may burn, she said.

Clobetasol or halobetasol ointment, or tacrolimus compounded in a 0.1% cream, can be used nightly intravaginally. Hydrocortisone acetate is available as a 25-mg suppository. A compounded hydrocortisone acetate suppository, 100–500 mg, used at bedtime for 2 weeks, can be given for severe vaginal disease, but adrenal suppression is possible. After 2 weeks, the dose is decreased, depending on the response.

For severe lichen planus, Dr. Margesson finds intramuscular triamcinolone acetonide 1 mg/kg every 4 weeks for 3–4 months is a better tolerated alternative to prednisone. Other effective drugs include methotrexate 5–10 mg per week or cyclosporine 4–5 mg/kg, but the latter should be limited to 2–3 months of treatment. Mycophenolate mofetil (CellCept) has been used up to 3 g per day, and recent studies suggest the use of etanercept (Enbrel) 50 mg subcutaneously twice weekly.

Surgery may be needed for significant scarring or vaginal adhesions, but these can recur. Dilators used nightly are important in these cases, and for vaginal stenosis, she said.

Many unanswered questions remain regarding lichen planus, Dr. Margesson said. They include the precise length of therapy, remission rate, best maintenance therapy, and long-term surveillance plan, whether treatment is needed in asymptomatic patients, which patients will go on to get squamous cell carcinoma, and what factors trigger lichen planus.

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