Search

Background: Lichen sclerosus (LS) is a chronic inflammatory disorder, presenting with pruritis and hypopigmentation of the vulvar and anogenital skin. LS presenting as a peri-clitoral mass has not been previously described., Case: A 5-year-old patient with vulvar pruritis and ultrasound showing a homogenous mass was referred for suspected clitoromegaly with normal labs. Examination demonstrated a prepubertal patient with a mobile, soft, peri-clitoral mass and surrounding hypopigmentation consistent with LS. The cyst was excised surgically; pathology revealed an epidermal inclusion cyst. Postoperatively, she began using topical steroids for LS with symptom resolution., Conclusion: Thorough workup of clitoromegaly negative for hormonal causes requires further investigation to determine an alternative etiology of the mass. We suspect that inflammatory changes of LS and pruritus resulted in the peri-clitoral inclusion cyst., Competing Interests: Conflicts of Interest The authors have no conflicts of interest to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Study Objective: To determine the frequency of persistence of vulvar lichen sclerosus (LS) through the pubertal transition and assess if the symptomatology and exam findings differ by menarchal status at onset of symptoms DESIGN: A retrospective cohort study SETTING: Academic tertiary care hospital PARTICIPANTS: Females aged 21 years or younger with a diagnosis of vulvar LS INTERVENTION: None MAIN OUTCOME MEASURES: Menarchal status at symptom onset, presenting symptoms, exam findings, persistence after menarche RESULTS AND CONCLUSIONS: Of the 196 patients who met criteria, 141 were premenarchal and 55 postmenarchal. Of these 55, 36 had postmenarchal symptom onset, and the others had premenarchal symptom onset or LS diagnosis. Over the data review period, 26 patients were followed through the pubertal transition, and 10 (38.5%) had continued symptoms of LS. The premenarchal group (n = 141) was significantly more likely than the symptom-onset postmenarchal group (n = 36) to present with vulvar itching (70.2% vs 52.8%; P = .048), vulvar bleeding (26.2% vs 5.6%; P = .008), and bowel symptoms (16.3% vs 0%; P = .009). The premenarchal group was significantly more likely on exam to have subepithelial hemorrhages (24.8% vs 5.6%; P = .01). The postmenarchal group had more clitoral adhesions (25.0% vs 4.3%; P < .0001) and loss of labia minora (47.2% vs 2.1%; P < .0001). Thirteen postmenarchal patients presented with dyspareunia. This study suggests that premenarchal LS can persist after menarche in about 40% of adolescents and can initially develop in postmenarchal adolescents. Initial symptoms and exam findings differ on the basis of menarchal status. Continued surveillance is recommended., (Copyright © 2022. Published by Elsevier Inc.)

This Clinical Opinion replaces the NASPAG Clinical Recommendation: Pediatric Lichen Sclerosus published in 2014. The objective of this document is to provide guidance in the diagnosis and management of vulvar lichen sclerosus (LS) in the pediatric and adolescent patient in order to treat patient symptoms and reduce long-term sequelae. LS is a chronic inflammatory condition affecting the anogenital region that may present in the prepubertal or adolescent patient. Clinical presentations include significant pruritus, loss of pigmentation and vulvar adhesions with loss of normal vulvar architecture. Management includes topical agents for induction and maintenance therapy, as well as long-term follow-up for identification and treatment of recurrence and sequelae. This document is intended for use by both primary and specialty pediatric and adolescent gynecology (PAG) providers, including specialists in pediatrics, gynecology, adolescent medicine, and dermatology., (Copyright © 2021. Published by Elsevier Inc.)

Study Objective: To assess long-term outcomes of lichen sclerosus (LS) in the female pediatric population, specifically in relation to patient age, treatment type and duration, and remission. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Retrospective chart review was conducted to identify female pediatric patients (0-18 years of age) who were diagnosed with LS between January 1, 2015 and January 1, 2020 at the University of North Carolina Dermatology and/or Obstetrics and Gynecology Departments. Patients were contacted via telephone for follow-up interviews consisting of a series of questions regarding patient age, symptom onset, time of diagnosis, treatment, and current symptoms., Results: Of the 128 patients identified, 61 patients consented and participated in follow-up interviews. At the time of study follow-up, 55/61 (90%) of participants reported their symptoms were improved. Patients reported using a variety of treatments, with medium- to high-potency topical steroids being the most common. At the time of follow-up, 53/61 (87%) of patients reported being asymptomatic, 37/53 (70%) of whom were not using any form of maintenance therapy. Those who achieved symptom resolution did so at an average of 8.4 years of age. There was no significant difference in age in asymptomatic patients receiving maintenance therapy and those receiving no maintenance therapy. There was a positive correlation for the duration of LS treatment and time in remission (P < .001). Increased patient age at time of follow-up also correlated positively with time in remission (P < .001)., Conclusion: In our cohort, the need for continued maintenance therapy was not correlated with age or, by proxy, pubertal status. Thus, LS remission might be determined more by early and successful pharmacological interventions., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Study Objective: To compare clinical characteristics, treatment histories, and microbiology of premenarchal girls who presented to a pediatric gynecology specialty clinic with short-duration and chronic vulvar symptoms., Design: Retrospective cohort study., Setting: Pediatric and adolescent gynecology clinic at a tertiary care children's hospital., Participants: One hundred eighty-two premenarchal patients ages 2-14 years who presented to a pediatric gynecology specialty clinic with vulvar complaints and who were evaluated with a yeast and/or bacterial culture., Interventions: None., Main Outcome Measures: Chronic and short-duration vulvar symptoms, microbiology, and diagnosis., Results: Patients with chronic symptoms were more likely to present with itching (59/102 (57.8%) vs 34/80 (42.5%); P = .04), redness or rash (53/102 (52.0%) vs 22/80 (27.5%); P = .0009), and discomfort (59/102 (57.8%) vs 30/80 (37.5%); P = .006), compared with patients with short-duration symptoms. Overall, 44.5% of patients had a history of antifungal treatment, with a greater proportion of patients with chronic symptoms having received antifungal treatment compared with those with short-duration symptoms (53/102 (52.0%) vs 28/80 (35.0%); P = .02). Despite a history of antifungal treatment in nearly half of the patients, Candida albicans was isolated in only 3/144 (2.1%) yeast cultures. Bacterial vulvar cultures were positive in 75/159 (47.2%), and there was no difference among the symptom duration groups (38/71 (53.5%) vs 37/88 (42.1%); P = .15)., Conclusion: Vulvovaginitis is a common gynecological diagnosis among premenarchal girls with short-duration and chronic vulvar symptoms. Regardless of symptom duration, yeast cultures are rarely positive. Antifungal treatment should be avoided in toilet-trained prepubertal girls., (Copyright © 2021 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Objective: To elucidate current understanding on the pathophysiological mechanism of genital lichen sclerosus (LS), urologic manifestations, and treatment options., Materials and Methods: The Medline/PubMed and Embase databases were systematically reviewed for publications pertaining to LS. After applying inclusion and exclusion criteria, references were assessed for relevance to the pathophysiology, presentation, and treatment of LS by title and abstract review by 2 independent reviewers, yielding 186 articles for assessment., Results: The contemporary understanding of the epidemiology and histology of LS is reviewed herein. Additionally, we explore in detail the 3 hypotheses regarding the pathophysiological mechanism contributing to disease presentation: infectious etiology, primary immune dysregulation, and the isotraumatopic response. We summarize the available biological evidence supporting each hypothesis. This discussion provides context for understanding LS morbidity and may spur new avenues of research. For the clinician, we review the clinical presentation of disease, including the risk of progression to squamous cell carcinoma. The current medical and surgical treatment options are also detailed., Conclusion: LS remains a potentially insidious disease which may lead to debilitating urinary and sexual dysfunction. Cross disciplinary research should aim for earlier detection, as well as more effective and durable treatment. The exact cause of LS remains unknown., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: We present an adolescent with multiple vulvar angiokeratomas within a background of lichen sclerosus., Case: A 13-year-old girl presented with vulvar pruritus and wart-like vulvar lesions. Four lesions were resected because of discomfort and uncertainty of the diagnosis. Pathology revealed angiokeratomas with chronic inflammation suggestive of lichen sclerosus. Postoperatively, pruritus continued in the largest excised lesion, which was associated with lichen sclerosus, and symptoms were treated successfully with topical steroids., Summary and Conclusion: Vulvar angiokeratomas are asymptomatic red papular lesions and are rare in the female adolescent population. In this case, the pathology revealed the rare co-occurrence of angiokeratomas and lichen sclerosus. Biopsies of vulvar vascular lesions in symptomatic adolescents are recommended. Vulvar angiokeratomas might manifest rare genetic disease in otherwise asymptomatic female patients and warrant further follow-up., (Copyright © 2019. Published by Elsevier Inc.)

Study Objective: To help determine the long-term course of girls diagnosed with lichen sclerosus before puberty., Design: Retrospective chart review and follow-up interview., Setting: Washington University pediatric gynecology and dermatology clinics., Participants: Premenarchal girls diagnosed with lichen sclerosus from 1989-2010., Interventions: Telephone interview., Main Outcome Measures: Resolution of symptoms, specifically pain and/or pruritus., Results: Follow-up was available for 36 premenarchal girls. The mean age at lichen sclerosus (LS) diagnosis was 7 years (range: 3-14 years). The mean duration of follow-up was 5.3 years (range: 2 months-15 years). Treatment with topical steroids (primarily 0.05% clobetasol propionate ointment) resulted in improvement in symptoms within an average of 14 weeks (range: 2 weeks-2 years) in 33 girls. Eighty-three percent of patients (n = 30) experienced remission after initial treatment. Sixteen patients reported relapses requiring an average of 3.1 years of intermittent maintenance therapy. The mean length of remission to date was 3.6 years (range 1 months-10 years). 72% of patients reported remission at the time of the phone interview. Of note, 7 out of 9 patients in our study who continue to report symptoms are still premenarchal. One postmenarchal patient was asymptomatic but had signs of LS on physical exam., Conclusion: The prognosis and long term course of LS diagnosed prior to puberty is unclear. Although remission may occur prior to menarche in some cases, once children reach menarche with active disease, complete remission may be less likely. Treatment duration of LS in our study had a wide range, but 3 months appears to be adequate for most patients to obtain remission., (Copyright © 2013. Published by Elsevier Inc.)