title: Prepubertal-Onset Vulvar Lichen Sclerosus: The Importance of Maintenance Therapy in Long-Term Outcomes.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Dermatology, Women's and Children's Hospital, North Adelaide, South Australia, Australia.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Ellis
authors.affiliation: Department of Dermatology, Women's and Children's Hospital, North Adelaide, South Australia, Australia.|Department of Dermatology, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Gayle
authors.initials: G
authors.lastname: Fischer
mesh_terms: D000287:Administration, Topical; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D012074:Remission Induction; D012189:Retrospective Studies; D016896:Treatment Outcome; D007724:Vulvar Lichen Sclerosus / Q000188:drug therapy* / Q000473:pathology
chemical_list: D005938:Glucocorticoids