Lichen Sclerosus Throughout Childhood and Adolescence: Not Only a Premenarchal Disease

Abstract

ABSTRACT Lichen sclerosus (LS) is a chronic dermatologic condition most commonly seen in prepubertal girls or postmenopausal women that causes inflammation and epidermal thinning in the anogenital region. Lichen sclerosus is suspected to have a genetic component due to a strong association with family history and is associated with various autoimmune diseases. Treatment involves medium- and high-potency topical corticosteroids, as well as calcineurin inhibitors; however, the optimal treatment regimen is unknown. LS follows a chronic and recurring pattern that causes significant discomfort and impaired quality of life over time; however, little data exist on the disease course in adolescent patients. This retrospective cohort study aimed to characterize and compare symptoms and physical examination findings of a cohort of pediatric, adolescent, and young adult females with vulvar LS. Patients 21 years or younger presenting to the University of Michigan between January 2006 and September 2020 with vulvar LS were included. Demographic data, personal and family history of autoimmune disease, specialty of provider, age of onset, age at menarche, presenting symptoms, and physical examination findings were abstracted from the chart. Patients were grouped for analysis based on symptom onset, first clinical visit, and the most recent clinical visit. Group 1 was patients who were premenarchal at their first clinic visit, and group 1a included those within group 1 who were postmenarchal at the most recent clinical visit. Group 2 included patients who were postmenarchal at the first clinic visit, and group 2a were those within group 2 who reported first onset of symptoms postmenarche. A total of 196 patients met inclusion criteria, with 141 in group 1, 26 in group 1a, 55 in group 2, and 36 in group 2a. The average age in group 1 was 7.02 years and in group 2 was 19.08 years. Patients in group 2 were more likely to have a personal history of autoimmune disease compared with group 1 (P = 0.04). Of the 26 patients in group 1a, 10 (38.5%; 95% CI, 19.8–57.2) continued to have symptoms of LS after undergoing menarche. Compared with patients with symptom onset postmenarche (group 2a), the premenarchal cohort (group 1) was significantly more likely to present with vulvar itching (70.2% vs 52.8%), vulvar bleeding (26.2% vs 5.6%), and bowel symptoms (16.3% vs 0%). The premenarchal cohort (group 1) was significantly more likely than the postmenarchal cohort (group 2a) to have subepithelial hemorrhages (24.8% vs 5.6%), whereas the postmenarchal group (group 2a) was significantly more likely to have architectural changes of the vulva including clitoral adhesions (25.0% vs 4.3%) and loss of labia minora (47.2% vs 2.1%). Of the 55 postmenarchal patients (group 2), 39 had at least 1 architectural change of the vulva, and among the 36 with first onset of symptoms postmenarche (group 2a), 20 (55.6%) had at least 1 vulvar skin change. This study of the largest cohort to date of young patients with vulvar LS demonstrates approximately 40% of patients with LS that went through puberty continued to have symptoms, and that symptoms and examination findings were different and often more severe among those who developed symptoms postmenarche versus premenarche.