Long-term follow-up in anogenital lichen sclerosus needed to prevent morbidities

Key takeaways:

• 92% of patients diagnosed prepuberty exhibited signs of active disease long-term.
• Nine patients were asymptomatic.
• 88% of patients had clinical signs of permanent anatomical changes from disease progression.

Anogenital lichen sclerosus can lead to permanent anatomical changes and sexual discomfort in women, highlighting a need for long-term follow-up and high treatment adherence, the latter of which is lacking, according to a study.

Although anogenital lichen sclerosus (ALS), an inflammatory dermatosis that affects the anogenital region, mainly affects postmenopausal women, 15% of cases are diagnosed before puberty.

“The prevalence of prepubertal ALS is estimated to be 1:900,” Sheila Vallée, MD, FRCPC, of the pediatric dermatology division at Sainte-Justine University Hospital Center at the University of Montreal, and colleagues wrote. “It is one of the most common conditions associated with anogenital complaints in girls.”

Since long-term studies on the clinical course of these patients is lacking, the authors conducted a 35-year retrospective study of the evolution of 65 girls (mean age at diagnosis, 6.5 years) with ALS diagnosed in the prepubertal stage. Of these participants, 54 took part in a phone assessment at presentation, and 30 took part in a phone questionnaire and/or physical examination at follow-up.

The authors found that 14 of 30 (47%) patients were asymptomatic after their menarche. The most common symptom at both presentation (52%) and follow-up (28%) was pruritus. The second most common symptom differed, with constipation the most common at presentation (52%) and vulvar discomfort the most common at follow-up (23%). Pain during intercourse was also reported in 40% of the 20 patients who were sexually active at follow-up.

Investigators found signs of active disease after puberty in 92% of the 25 patients who agreed to a physical exam at follow-up, with the most common symptoms including atrophy (92%) and hypopigmentation/white plaques (92%). However, nine of these patients were asymptomatic and six had stopped their treatment. Notably, two patients who did not have extragenital lichen sclerosis prepuberty had it at follow-up.

“This is in accordance with other studies where clinical signs of LS have been found in asymptomatic patients,” the authors said. “Using symptoms as an indicator of disease activity is insufficient and long-term follow-up with physical examination should be recommended for patients with a diagnostic of ALS before puberty.”

Clinical changes to look out for include alteration in vulvar anatomy such as the resorption and/or adherence of the labia, covering of the clitoris, narrowing of the vaginal vestibule and fissuring of the perineum. In this cohort, 88% of the patients that underwent a physical examination presented with such changes.

The continual application of topical steroids is the best way to combat this type of progression; however, 17 patients in the cohort limited treatment to symptomatic flares and nine only used topical steroids as a maintenance therapy.

Limitations included being a single-center, small study, with only about half of the patients included in follow-up data.

“Physical examination post puberty is essential to identify clinical progression that may lead to permanent anatomical changes and sexual discomfort,” the authors concluded. “Long-term follow-up in patients with prepubertal-onset of ALS is important to prevent morbidity associated with ALS.”