Long‐term consequences of juvenile vulvar lichen sclerosus: A cohort study of adults with a histologically confirmed diagnosis in childhood or adolescence

Abstract

IntroductionVulvar lichen sclerosus (VLS) occurs in at least one in 900 girls. There is limited knowledge as to what extent the disease persists in adulthood and what the repercussions in adulthood may be. The aim of this study is to evaluate the long‐term consequences of VLS diagnosed in childhood or adolescence.Material and methodsThe population of females histologically diagnosed with VLS in childhood or adolescence in the Netherlands between 1991 and 2015 was identified through the national pathology database. Histological specimens were retrieved and re‐evaluated. Potential participants for whom the diagnosis was reconfirmed and who are now adults, were then traced and surveyed. Descriptive statistics were calculated and compared with the literature. Main outcome measures are the demographics of the cohort, their scores on standardized quality of life (QoL) and sexuality questionnaires and answers to additional questions regarding patients’ experience with the disease. The questionnaires used were the Dermatology Life Quality Index (DLQI), the Skindex‐29, the Female Sexual Function Index (FSFI) and the Female Sexual Distress Scale‐Revised (FSDS‐R). Secondary outcome measures include obstetric history and histological features found in the original tissue specimens.ResultsA total of 81 women participated, median age 29.0 years, median follow‐up from childhood diagnosis 19.5 years. Both QoL and sexuality were somewhat affected in 51.9% of cases. Less than half (45%) reported having regular check‐ups. Forty‐five (56%) reported symptoms within the past year; of those with symptoms, 14 (31%) were not under surveillance. Cesarean section rate (14.5%) was comparable to the general population, and there were more high‐grade obstetric anal sphincter injuries with vaginal deliveries than expected. Sixteen respondents (20%) were not aware of the childhood diagnosis prior to this study.ConclusionsSymptoms due to VLS are reported by most adults diagnosed as juveniles. QoL and sexuality are affected and correlate to recent symptoms. VLS as a juvenile does not preclude a vaginal delivery. Women diagnosed with VLS in childhood or adolescence are often lost to follow‐up.

Outcome Measures in Adult Vulvar Lichen Sclerosus: A Systematic Review

“…Table 1 shows the included publications listed chronologically, describing the study, the questionnaires applied and outcome measures with the corresponding (C)OD 14 . Of the 35 studies included, 5–10,16–44 14 16–21,25–28,33–36 developed a new scoring system or questionnaire. Twenty-six different tools were found, and 4 different ODs identified: QoL, symptoms, clinical (visible) signs, emotional impact, and sexual functioning.…”

“…Six questionnaires that had been applied just once in VLS research were found, the EQ-5D VAS † , 5 Short Form-12 (SF-12), 5 Short-Form-36 (SF-36), 24 WHO-5 d, 9 WPAI:GH, d and PHQ-9 d, 42 The subtypes QoL-dermatologic and QoL-vulvar cover the QoL-LS specific COD. Within this domain 4 questionnaires, the Skindex-29, 5,6,43 DLQI, 7,9,30,41,43 DSQL-V d , 16 and Vulvar Quality of Life Index (VQLI) 10,31,33,37,38,44 have been applied to VLS. Of these questionnaires the DLQI, which is a QoL-dermatological specific questionnaire, and the VQLI, a QoL-vulvar specific questionnaire, were used most often respectively 5 and 6 times.…”

“…Within this scale type, 5 questionnaires, the Female Sexual Function Index (FSFI), Female Sexual Distress Scale (FSDS), Patient Reported Outcomes Measurement Information System Sexual Function (PROMIS) SexFs-v2.0, PROMIS SexFS v2-Vulvar discomfort scales, and Sexual Quality of Life-Female (SQOL-F) 42 were found in the literature. The FSFI 7,9,19,39,40,43 was used most frequently in studies on VLS ( n = 6). The FSDS, 7,40,43 was used 3 times and PROMIS SexFSv2.0 g 23 just once.…”

“…The FSFI 7,9,19,39,40,43 was used most frequently in studies on VLS ( n = 6). The FSDS, 7,40,43 was used 3 times and PROMIS SexFSv2.0 g 23 just once. The PROMIS-SexFS-v2-vulvar discomfort scale, developed for the potential application to various specific vulvar conditions, has not yet been applied in VLS research.…”

“…The effects of the disease on sexual functioning may be underestimated using generic or nonvulvar-specific dermatological QoL assessment in women in good general health. Regarding QoL and sexual wellness, there is a significant impairment of sexual function in subjects with VLS based on the FSFI and FSDS compared to healthy controls of similar age, 7,43 even despite adequate treatment 4 …”