Lichen Sclerosus Before Puberty Continues to Confound



MIAMI BEACH – High-dose topical steroids provide significant symptomatic relief for young girls with lichen sclerosus, a retrospective study has shown.

Less clear and more controversial, however, is whether this painful and itchy dermatologic condition spontaneously resolves after prepubertal girls reach menarche, Dr. Kathryn Squires** said. "We hypothesize that ... lichen sclerosus is a relapsing and remitting disease."

One important implication is a need for long-term follow-up, particularly because lichen sclerosus carries an increased risk of vulvar squamous cell carcinoma in adult women, Dr. Squires said.

With only a handful of published studies that assess the natural progression of lichen sclerosus in pediatric patients, Dr. Squires and her colleagues decided to find out more. They identified 97 premenarchal girls with lichen sclerosus treated at a gynecology or dermatology clinic at the Washington University in St. Louis* between 1995 and 2010. The mean patient age was 7 years at the time of diagnosis.

A total of 36 patients (or their parent) participated in a 10- to 15-minute, scripted telephone interview with at a mean follow-up of 5.3 years and were studied further. The main outcome was resolution of symptoms of pain and/or pruritus.

Thirty patients (83%) reported a period of remission after initial treatment. A total of 26 patients (72%) were in remission at the time of the telephone interview. Seven (78%) of the nine patients who continued to report symptoms at follow-up were still premenarchal.

"Most young women will experience symptomatic relief with high-dose topical steroid treatment, which in our study was clobetasol ointment," Dr. Squires said. Specifically, this therapeutic approach yielded significant improvement of symptoms within an average of 14 weeks in 33 girls.

Part of the treatment challenge stems from a lack of standardized protocol with regard to dose, frequency and duration of treatment, or need to taper, Dr. Squires said. In the current study, the duration of treatment varied, "but it appears that 3-4 months will be adequate for most patients," she added.

A total of 16 patients reported a relapse that required maintenance therapy. The average length of remission was 3.6 years (range, 1-10 years).

Because of these findings, the prognosis and long-term course of lichen sclerosus diagnosed prior to menarche remains unclear, Dr. Squires said at the annual meeting of the North American Society for Pediatric and Adolescent Gynecology.

"Lichen sclerosus is a common dermatologic condition. It is often found on the vulva, most commonly in postmenopausal women, [but] prepubertal girls account for about 5%-15% of cases," said Dr. Squires of the department of obstetrics and gynecology at Washington University in St. Louis. The condition is likely underrecognized and underreported in this pediatric population, she added.

Lichen sclerosus often presents as characteristic white, sharply demarcated plaques on the vulva and perianal skin. Some girls will have smooth and waxy skin; others present with crinkling or cellophane paper–type skin. Lichenification also can occur, in which case the skin becomes thick and leathery. The labia minora can adhere to adjacent structures in some cases. The precise etiology is unknown.

An unanswered question is whether lichen sclerosus in postmenopausal women is somehow distinct or on a continuum that includes the prepubertal presentations, Dr. Squires said.

A limitation of the telephone follow-up design of the study was the inability to verify physical findings.

Future research could compare the effectiveness and safety of treatment with calcineurin inhibitors versus topical steroids. In addition, it would be helpful to identify any factors that predict relapse versus achievement of complete remission.

Information for patients and families on pediatric vulvar lichen sclerosus is available online from the North American Society for Pediatric and Adolescent Gynecology.

Dr. Squires reported that she had no relevant financial disclosures.

* Correction: The university name was intially misstated as the University of Washington. 5/24/2012

**Correction: The investigator's name was initially misstated as Dr. Lauren C. Squires. 5/30/2012

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