Does compliance to topical corticosteroid therapy reduce the risk of development of permanent vulvar structural abnormalities in pediatric vulvar lichen sclerosus? Aretrospective cohort study.

Abstract

Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis of the genital skin, with up to 20% of cases in the pediatric age group. Limited data exist concerning the prognosis of pediatric VLS, particularly the likelihood of permanent architectural change and whether this can be prevented by compliance with topical corticosteroid treatment (TCS). To evaluate the extent to which compliance to TCS treatment influences the risk of developing vulvar structural abnormalities, including clitoral phimosis and diminutive or fused labia minora. A retrospective chart review of case records of pediatric-age females with VLS between January 31, 2004 and January 31, 2021. One hundred eighteen cases of VLS were identified, with a mean age at diagnosis of 7.25years and a mean follow-up period of 42.7months. Thirty-four girls were "partially compliant," whereas 84 were "compliant." The risk ratio (RR) of developing any vulvar structural abnormality or clitoral phimosis was 5.76 (95% CI 2.96-11.3) and 21.2 (95%CI 5.23-85.9) times higher, respectively, in partially compliant compared with compliant subjects. The RR of a partially compliant female with pre-pubertal onset VLS having a vulvar structural abnormality persisting beyond menarche was increased 3.54-fold relative to compliant females (95% CI 1.75-7.17). The retrospective nature of our data, lack of a control group, wide variability in follow-up duration, and nonstandardized method of stratifying compliance. Vulvar structural abnormalities are common in prepubertal onset VLS. Compliance to TCS appears to be critical in the prognosis of pediatric VLS although attitudes underpinning noncompliance to TCS treatment require further elucidation.