SAN FRANCISCO — If left untreated, lichen sclerosis can lead to scarring, destruction of normal vulvar architecture, and even skin cancer, but if caught early and treated properly, complete remission is possible.
“It's a wonderful disease to treat,” said Dr. Erika Klemperer, a dermatologist in private practice in Santa Barbara, Calif. “You can make these patients better.”
The first step is recognizing when the diagnosis is lichen sclerosis and when it is something else, she said at a meeting sponsored by Skin Disease Education Foundation (SDEF).
Patients will complain of pruritus, but they will avoid scratching because it is too painful. In contrast, vulvar lichen simplex chronicus results in an itch that is pleasurable to scratch.
They also will report dyspareunia, dysuria, and painful bowel movements. And patients will either be prepubertal or postmenopausal, she said.
“You've got to thoroughly look at the vulva to pick up the early signs of disease,” Dr. Klemperer said. “You have to be comfortable looking in this area so we can help these women. You've got to make sure you spread the lips. And not just pulling the labia apart. You really have to manipulate the folds, lift up the clitoral hood.”
If it is lichen sclerosis, pallor and edema can be seen, in addition to well-demarcated pearly white plaques that tend to be symmetric and arranged in a figure eight around the vulva, perineum, and perianal regions. The plaques will have a distinctive texture—either a dull, glazed, waxy texture; a very shiny texture; or a fine, crinkled texture.
Purpura, fissures, and fragility also are common. “Purpura is a key diagnostic sign,” Dr. Klemperer said. “If you see purpura, think lichen sclerosis.”
Edema around the clitoral hood is another early finding, and in more advanced cases there may be secondary lichenification and extensive scarring.
If unsure whether it is vulvar lichen sclerosis, look elsewhere on the patient's body. About 10%–15% of women with the condition will have extragenital disease, most often on the upper trunk. Lesions in that location can confirm an uncertain diagnosis, she said.
In addition to treating the secondary infections, “Ultrapotent topical steroid ointments are absolutely the treatment of choice for lichen sclerosis,” Dr. Klemperer said. “They've been proven not only to improve symptoms but also to actually affect both clinical and histologic disease. You can reverse all the changes that we talked about except for the scarring.” (See sidebar.)
Although treatment needs to be individualized, Dr. Klemperer tends to start with twice-daily treatment for the first month and once or twice daily thereafter. She said that she typically will treat for 12 weeks, but this can vary.
Relapses are common when treatment is stopped, so Dr. Klemperer now recommends maintenance treatment. “The studies have not been done yet to show what that ideal [maintenance] treatment is, but most of us in the vulvar derm world do 1–3 nights weekly of mild to potent topical steroid ointments,” she said. “And then I see them back in 3 months to see how they're doing.” Educating the patient on the need for these treatments also is critical. “I tell them why we're doing this,” Dr. Klemperer said. “We're trying to prevent further scarring. Lichen sclerosis definitely has an association with squamous cell carcinoma. My goal is to prevent that.”
Dr. Klemperer stated that she had no conflicts of interest related to her presentation. SDEF and this news organization are wholly owned subsidiaries of Elsevier.
Appropriate Use of Ultrapotents Is Key
Ultrapotent topical steroids can be used safely and effectively in the vulvar area, but appropriate use is key, Dr. Klemperer said.
She offered the following topical steroid safety tips:
▸ Ultrapotents should not be used for psoriasis or a mild contact dermatitis. Desonide ointment or other lower-potency topicals will do the trick.
▸ Use ointments rather than creams because they are more occlusive.
▸ Monitor patients monthly for side effects. Although mucous membranes are relatively resistant to steroid side effects, this is not true of skin in the groin or perineum, or around the buttocks.
▸ Stress to patients that ultrapotent steroids should be used once or twice daily, never more.
▸ Monitor the quantity that patients use. “They should not be going through a 30-g tube in less than 3 months. If they are, they're putting on much too much.”
▸ Perform a biopsy on a persistent hyperkeratotic, ulcerated, or nonresponsive area.