Prepubertal-Onset Vulvar Lichen Sclerosus: The Importance of Maintenance Therapy in Long-Term Outcomes.

BACKGROUND

There is sufficient published data on induction treatment with potent topical corticosteroid (TCS) in childhood vulvar lichen sclerosus (VLS) but limited data on long-term management. VLS has been shown to extend beyond menarche and repercussions of suboptimal long-term control may be devastating and permanent. This single-center retrospective study reviewed outcomes of long-term treatment using individualized regimens with target outcome of complete objective normality.

METHODS

Forty-six girls with prepubertal-onset VLS were studied for demographic data, previous treatment, induction and maintenance treatment, clinical response and compliance. Photographic records were available for all patients. The cohort was divided into two groups: adherent (using treatment all or most of the time) and non-adherent (using treatment some or less of the time).

RESULTS

Twenty-six patients (56%) had received prior treatment however only three had achieved disease control using daily potent TCS. Twelve patients (26%) had scarring on presentation. All achieved initial objective disease suppression with induction treatment using potent TCS. Thirty-one of 33 adherent patients (93.93%) sustained complete disease remission with no progression or scarring. In contrast, 1 of the 13 nonadherent patients (8%) achieved complete disease remission (p < 0.001), 9 of the 13 (69.23%) experienced disease progression (p < 0.001) and 3 of the 13 (23%) developed scarring during follow-up. Those with established scarring on presentation did not recover with treatment.

CONCLUSION

Our data support previous studies regarding initial treatment with potent TCS but additionally suggest maintenance treatment with individualized regimens that achieve objective normality in addition to symptom control provide optimal outcomes.