The long-term clinical consequences of juvenile vulvar lichen sclerosus: A systematic review.

Background: Vulvar lichen sclerosus (VLS) occurring in children and adolescents may have repercussions throughout life.

Objective: We sought to assess the evidence available on the long-term consequences of juvenile VLS.

Methods: Multiple databases were searched for studies containing long-term follow-up information on children or adolescents up to age 18 years with VLS. Articles were classified by level of evidence and the specific aspects of VLS studied.

Results: In all, 37 studies met the inclusion criteria, giving information on the long-term consequences of VLS, of which 13 were cohort studies and 24 were case reports or series. These publications show that signs and symptoms persist after puberty and beyond, scarring and permanent architectural changes occur, treatment is effective with regard to symptoms, and long-term quality of life is affected. Findings suggest a possible relationship with risk of malignancy. The included publications had low-level evidence.

Limitations: Meta-analysis was not possible because the studies had different focuses. Very few patients were followed into adulthood.

Conclusions: There is low-level evidence suggesting long-term repercussions of juvenile VLS. Studies following children and adolescents with VLS into adulthood are needed to better understand the course of this disease and its repercussions on adult vulvar health.