Long-Term Outcomes of Prepubertal-Onset Vulvar Lichen Sclerosus.

Abstract

Background: Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis affecting the vulvar and perianal skin. VLS has a bimodal age of onset, in pre-pubertal and post-menopausal age groups. Long-term outcomes of pre-pubertal VLS are unclear. This study aimed to establish the long-term disease activity, factors that influence disease progression, and the physical and psychological complications of pediatric VLS persisting into adulthood.

Methods: A cohort study was conducted in women diagnosed with VLS pre-menarche. A retrospective chart review of 135 case records was completed. The remaining data collection occurred prospectively through (i) study questionnaires, including quality of life as assessed by the Vulvar Quality of Life Index (VQLI), and (ii) dermatologist assessments.

Results: Of 135 patients screened, 68 were included. The mean age of diagnosis was 7.5 years, and the mean duration of follow-up was 12.8 years. 62% of cases achieved remission. Of patients who were adherent to treatment, 92.3% achieved remission, compared to 56.6% of patients who were non- or partially adherent. Adherence declined from menarche into early adolescence. Patients who were adherent had 60% fewer structural abnormalities. In addition, VQLI scores indicated significantly better quality of life with adherence (mean = 1.6/45) compared to partially or non-adherence (mean = 5.2/45).

Conclusions: This study informs clinicians of the long-term prognosis of VLS diagnosed pre-pubertally: 62% of patients achieved remission, and the rates of remission were higher in those who adhered to treatment. Structural changes and scarring were reduced in those who adhered to treatment.