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

• Published in: Acta obstetricia et gynecologica Scandinavica Vol. 102; no. 11; pp. 1469 - 1478
• Main Authors: Morrel, Beth, Avoort, Irene A. M., Ewing‐Graham, Patricia C., Damman, Jeffrey, Schappin, Renske, Zeijl, Kelly N., Voorham, Quirinus J. M., Esajas, Martha D., Oom, Daniella M. J., Vleeschouwer, Marloes L. H. M., Mous, Marieke, Salvatore, Concetta M., Leeuwen, Jeanette, Bais, Joke M. J., Bassie‐Minderhoud, Willy, Dijkstra, Jeroen R., Geukes, Marije, Grinsven‐Dmitrieva, Nadja N., Janssen, Ineke C. A. H., Joostens, Mieke A. M., Kagie, Marjolein J., Klompmaker, Esther R., Lammerink, Ellen A. G., Lardenoije, Celine M. J. G., Lenselink, Charlotte H., Louwers, Jacqueline A., Maassen, Marloes S., Mierlo, Kim M. C., Nelissen, Ewka C. M., Piscaer‐van Eijsden, Claire J. A., Polderman, Marloes C. A., Salm, Paulien C. M., Santegoets, Lindy A. M., Traas‐Hofmans, Maaike A. F., Wassen, Martine M. L. H., Wijk, Heidy F. H., Kate‐Booij, Marianne J., Burger, Curt W., Pasmans, Suzanne G. M. A.
• Format: Journal Article
• Language: English
• Published: Reykjavik John Wiley & Sons, Inc 01-11-2023; John Wiley and Sons Inc
• Subjects: adolescent lichen sclerosus; Adults; Child development; Cohort analysis; DQLI; Females; FSDS‐R; FSFI; Gynecololgy; juvenile vulvar lichen sclerosus; Medical diagnosis; Obstetrics; Original; pediatric lichen sclerosus; Pediatrics; quality of life; Questionnaires; sexual well‐being; Sexuality; Skindex‐29; Teenagers; Vagina; vulvar lichen sclerosus
• ISSN: 0001-6349; 1600-0412
• DOI: 10.1111/aogs.14668

Introduction
Vulvar 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 methods
The 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.
Results
A 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.
Conclusions
Symptoms 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.
Vulvar lichen sclerosus (VLS), a chronic vulvar condition with symptoms including itching, pain and bleeding and often leading to permanent loss of vulvar skin and shape, is one of the most frequent diagnosis in girls who have vulvar complaints, affecting at least one in every 900 girls. VLS in children usually does not resolve at puberty, contrary to earlier beliefs. This study demonstrates what patients following a diagnosis of VLS in childhood and adolescence may experience as they reach adulthood.