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Background: Vulvar lichen sclerosus (VLS) is a chronic inflammatory skin condition associated with significant impairment of quality of life and potential risk of malignant transformation. However, diagnosis of VLS is often delayed due to its variable clinical presentation and shame-related late consultation. Machine learning (ML)-trained image recognition software could potentially facilitate early diagnosis of VLS., Objective: To develop a ML-trained image-based model for the detection of VLS., Methods: Images of both VLS and non-VLS anogenital skin were collected, anonymized, and selected. In the VLS images, 10 typical skin signs (whitening, hyperkeratosis, purpura/ecchymosis, erosion/ulcers/excoriation, erythema, labial fusion, narrowing of the introitus, labia minora resorption, posterior commissure (fourchette) band formation and atrophic shiny skin) were manually labelled. A deep convolutional neural network was built using the training set as input data and then evaluated using the test set, where the developed algorithm was run three times and the results were then averaged., Results: A total of 684 VLS images and 403 non-VLS images (70% healthy vulva and 30% with other vulvar diseases) were included after the selection process. A deep learning algorithm was developed by training on 775 images (469 VLS and 306 non-VLS) and testing on 312 images (215 VLS and 97 non-VLS). This algorithm performed accurately in discriminating between VLS and non-VLS cases (including healthy individuals and non-VLS dermatoses), with mean values of 0.94, 0.99 and 0.95 for recall, precision and accuracy, respectively., Conclusions: This pilot project demonstrated that our image-based deep learning model can effectively discriminate between VLS and non-VLS skin, representing a promising tool for future use by clinicians and possibly patients. However, prospective studies are needed to validate the applicability and accuracy of our model in a real-world setting., (© 2024 European Academy of Dermatology and Venereology.)

Vulvar lichen sclerosus (VLS) is a chronic, inflammatory disease which is accompanied by itching and pain, affecting the patient's daily life and sexual activity. However, the disease characteristics of children and adults are not completely the same. Currently, there are few studies in China that compare the characteristics of VLS between girls and adult female patients. The aim of this study was to compare the epidemiology, clinical features, and combined autoimmune diseases of VLS patients between girls and adult females, and to help clinicians better understand VLS in different age groups. We enrolled 744 female patients for analysis, divided by age into a child group (<18 years) and an adult group (≥18 years). Among girl patients, 94.6% had preadolescent onset, while among adult female patients, only 4.6% had preadolescent onset, which was a statistically significant difference. The highest percentage of adult female patients had onset during their child-bearing period (75.4%), while 20% had postmenopausal onset, with a significant difference when the three onset states were compared. White patches were equally common in both girl and adult female patients' external genital area, while mossy lesions and labia minora atrophy were more common in adult female patients. Involvement of the clitoris, labia minora, and vaginal opening area were more common in adult patients. The perianal area was more commonly involved in girl patients. We found eight cases (1.2%) of secondary squamous cell carcinoma in adult female patients. We also found that 13 patients had concurrent lichen sclerosus lesions on the vulva and extragenital region, including two girls and 11 adult females. Extragenital lichen sclerosus (EGLS) occurred mostly in the torso. Clinicians should be aware of these differences so that early diagnosis and treatment of the disease can be achieved, to avoid irreversible anatomical alterations and the risk of cancer., (© 2024 Japanese Dermatological Association.)

Abstract: Differentiated vulvar intraepithelial neoplasia (d-VIN) is an HPV-independent precursor to vulvar squamous cell carcinoma. The histology of d-VIN lesions is difficult to differentiate from that of non-neoplastic epithelial disorders, especially lichen sclerosus (LS). The authors present a case of LS, where relying on histopathology alone could have led to misdiagnosis. The patient was a 17-year-old female patient with clinical features of vulvar dermatitis and LS for 2 years. She was counseled to apply clobetasol 0.05% to the affected area daily but reported no improvement after 6 months. A biopsy of the right labia majora revealed histologic findings typical of d-VIN and near-contiguous p53 expression. These features are characteristic of d-VIN. However, d-VIN is exceedingly rare in young patients. The case was reviewed by 6 dermatopathologists and gynecologic pathologists, who observed that the degree of inflammation would be unusual postclobetasol therapy and could be due to noncompliance. A review of the patient's chart revealed that she "does not always remember to apply" clobetasol. The patient's clinician confirmed that there were compliance issues, and the follow-up biopsy was negative for d-VIN. The case was signed out as LS, with a note describing the above, and to rebiopsy if concern persisted. The authors conjecture that inflammatory infiltrates in the biopsied area caused reactive atypia due to lack of adherence to treatment. Although the patient's age helped rule out d-VIN, similar cases in elderly patients may be occurring. Pathologists must be aware that reactive forms of untreated LS can mimic d-VIN, to avoid misdiagnosis., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

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.)

Objectives: Studies on the consequences of juvenile vulvar lichen sclerosus (JVLS) in adulthood are limited. A number of measuring tools are available for analyzing adult vulvar lichen sclerosus (VLS), but these have not been applied in studies on JVLS. The aim is to study physical findings, quality of life, sexual well-being, and self-image in adult women with a history of juvenile VLS., Materials and Methods: Adult women with a biopsy proven history of JVLS were recruited to be examined and surveyed using available standardized measurement tools. This took place in an outpatient setting by physicians who were not involved in the treatment of participants., Results: Twenty-seven women (median age 29 years) with a history of JVLS and median time since biopsy of 19.5 years were recruited. Of these women, 59% currently had symptoms, 63% had signs of active disease, and 85% had moderate to severe architectural changes. Despite these residual signs, vulvar specific-quality of life and vulvar self-image scored favorably while generic health-related quality of life was somewhat effected., Conclusions: JVLS has consequences in adulthood involving physical findings and vulvar quality of life. The use of standardized outcome measures for clinical practice and research purposes facilitates a better understanding of the sequelae to JVLS., Competing Interests: Conflicts of interest: None to declare., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.)

Objective: The main outcome of this study was the evaluation of clinical characteristics, comorbidities, and therapeutic approaches in patients with vulvar lichen sclerosus (VLS) aged from childhood to perimenopause. Secondly, it was intended to compare these characteristics according to the menarchal status., Methods: Patients less than 45 years of age with a diagnosis of VLS from January 2002 to June 2022 in 10 referral centers were included in this retrospective longitudinal study. The univariate analysis compared the dependent variables according to menarchal status., Results: One hundred eighty-six patients met the inclusion criteria. At diagnosis, between 25% and 40% of premenarchal patients reported signs related to subepithelial hemorrhage. A significantly greater presence of bleeding ( p < .005), easy bruising ( p = .028), fissures ( p = .008), petechiae/splinter hemorrhages ( p < .001), and bleeding/blistering or open sores ( p = .011) was observed in premenarchal patients with respect to the postmenarchal group. The perineum ( p = .013) and the perianal region ( p < .001) were significantly more involved in the premenarchal group. Topical calcineurin inhibitors were more used in the premenarchal population ( p = .004), whereas vitamin E oil and moisturizers were more used in the postmenarchal population ( p = .047)., Conclusions: Vulvar lichen sclerosus is a chronic condition that can cause vulvar changes that result in severe morbidity and affects sexual function and quality of life, even before menopause. Vulvar lichen sclerosus continues to be misdiagnosed in this population. This may lead to an average delay from symptom onset to diagnosis. Evaluating clinical manifestations of VLS in premenarchal and postmenarchal age allowed us to find different clinical characteristics between the 2 periods suggestive of the diagnosis., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2024, ASCCP.)

Background: Vulval lichen sclerosus (VLS) is a skin condition which causes pain, itching, anatomical changes, and increases vulval cancer risk by up to 22 times. VLS is treatable, with use of topical steroids allowing symptom resolution and complication prevention. Diagnostic delay and misdiagnosis of VLS is common. Previous studies found that women attribute this to poor interactions with and knowledge of clinicians. Most VLS is diagnosed in primary care., Aim: To investigate primary care clinicians' views on confidence in identifying and managing vulval skin disease and VLS, barriers to diagnosis, education on vulval skin disease, and diagnostic criteria for VLS., Method: A mixed methods survey was distributed via professional events and networks. Qualitative data was analysed in NVivo using inductive thematic analysis. Spearman's rank correlation was used to correlate participant characteristics and confidence., Results: There were 122 responses, the majority from GP trainees (48%) and GPs (43%). Confidence in diagnosing, identifying, and treating VLS positively correlated with female gender, time in role, and examination frequency. Themes identified include patients not knowing normal, male clinicians: deskilling and reluctance, lack of clinician knowledge, and uncertainty around diagnosis and treatment. 38% of participants never participated in teaching or learning on vulval skin disease. Almost all (98%) participants feel diagnostic criteria would be helpful., Conclusion: This study illuminates the multiple barriers to diagnosis and treatment of VLS. The results confirm that clinicians share patient concerns that knowledge is poor, highlight the importance of developing clear diagnostic criteria, and will allow us to target training to those lacking in confidence., (© British Journal of General Practice 2024.)

Background/objectives: Lichen sclerosus (LS) is a chronic condition that warrants close follow-up due to the risk of scarring. The optimal long-term management of pediatric vulvar and perianal lichen sclerosus (PVPLS) is unknown. This study aimed to identify diagnostic, treatment, and maintenance regimens among pediatric dermatologists and pediatric/adolescent gynecologists, as well as assess provider confidence and desire for guidance on long-term PVPLS management., Methods: A cross-sectional 35-question survey was administered through the Pediatric Dermatology Research Alliance (PeDRA) and the North American Society for Pediatric and Adolescent Gynecology (NASPAG) between 7/13/2021 and 8/30/2021 to ascertain PVPLS diagnostic and management regimens., Results: Most responders were attending-level pediatric/adolescent gynecologists (46%) and pediatric dermatologists (41%). Although 85% of participants felt completely or very confident in diagnosing PVPLS, the majority (86%) desired further management guidelines. While the initial treatment was similar among providers, maintenance regimens and follow-up varied considerably, with only 42% recommending lifelong monitoring despite potential persistence into adulthood., Conclusions: While initial treatment was similar among practitioners, there was variation by specialty in subsequent management and a lack of uniformity in long-term follow-up. Additional studies are needed to clarify the optimal management of PVPLS and to provide evidence-based guidelines regarding long-term follow-up.&nbsp; J Drugs Dermatol. 2024;23(6):450-455.&nbsp; &nbsp;&nbsp; doi:10.36849/JDD.8084.

Objectives: Lichen planus (LP) and lichen sclerosus (LS) are the most common vulvar lichenoid dermatoses. The diagnostic challenges are due to site-specific variation in microscopic appearance and small-sized biopsies. Authentication of diagnostic criteria to distinguish LS and LP to uncover any resemblance or divergence in presentation of these conditions is attempted., Methods: Cases of vulvar LP and LS diagnosed between January 2012 to December 2022 were included. The clinical details included age, presenting symptoms, examination findings, and other organ involvement. Histopathological analysis of epidermal, dermal, and adnexal findings was done., Results: There were 28 cases of vulvar LP and 72 cases of LS, with a median age of 51 and 60 years, respectively. Depigmentation and atrophy were the major clinical features in LS, whereas ulcers/erosions and erythema were more prevalent in LP with a significantly higher incidence of oral involvement. The most diagnostic feature in LS was diffuse dermal sclerosis (76.8%) and interstitial pattern of inflammation (81.4%), whereas the characteristic features in LP cases was a lichenoid pattern of inflammation (85.7%), necrotic keratinocytes, and lymphocytic exocytosis. In 44.4% of LS, unconventional features like compact orthokeratosis, parakeratosis, thickened/wedge-shaped hypergranulosis, and sawtooth rete pegs were noted. Lichen sclerosus with lichenoid inflammation (21.4%) mimicked LP, from which it was distinguished by presence of thickened or diminished granular layer with basal melanin absence (60%) and dermal homogenization (80%)., Conclusion: Although the classical, well-established variant of LS poses no diagnostic difficulty, the unconventional variant may mimic LP. Identification of the subtle histological clues demonstrated in this study can help to arrive at the correct diagnosis., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2024, ASCCP.)

Introduction: Vulvar lichen sclerosus (VLS) is characterized by progressive anatomical changes which become increasingly severe and irreversible. The objective of this study was to investigate if a "window of opportunity" exists in VLS, i.e., to assess if an early treatment may prevent disease progression and facilitate clearance of symptoms and/or signs., Methods: This retrospective, cohort study included VLS patients treated for the first time with a topical corticosteroid, namely with mometasone furoate 0.1% ointment, for 12 weeks (2016-2021). Scoring of subjective symptoms (global subjective score, GSS, and dyspareunia) and clinical features (global objective score [GOS] and sclerosis-scarring-atrophy) was performed at baseline (T0) and at the control visit (T1). We assessed if the achievement of clearance in GSS, GOS, sclerosis-scarring-atrophy, or dyspareunia depended on the time elapsed between VLS onset and treatment initiation., Results: Among the 168 patients (59.2 ± 13.2 years) included, the median time between VLS onset and first treatment was 14.0 months. At T1, 48.8% of patients achieved clearance of GSS, 28% of GOS and 11.9% of both GSS and GOS, 57.9% of dyspareunia, and 19.2% of sclerosis-scarring-atrophy. The logistic regression model showed that each 10-month increase in treatment initiation adversely affected the clearance of GSS while starting treatment within 6 months of disease onset was significantly associated with clearance of GOS and sclerosis-scarring-atrophy., Conclusion: Early treatment is crucial in determining a complete healing of VLS-related symptoms and signs, especially of tissue sclerosis-scarring-atrophy, which appear poorly responsive, or even unresponsive, after the earliest stages of the disease. Thus our findings provide evidence for a "window of opportunity" in VLS treatment., (© 2024 S. Karger AG, Basel.)

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., (© 2023 The Authors. Acta Obstetricia et Gynecologica Scandinavica published by John Wiley & Sons Ltd on behalf of Nordic Federation of Societies of Obstetrics and Gynecology (NFOG).)

Competing Interests: Conflicts of interest None disclosed.

Competing Interests: Conflicts of interest The authors declare no conflicts of interest.

Background and Objectives: Although data regarding the rates of remission and progression of the disease are still scarce, it is generally now acknowledged that pediatric vulvar lichen sclerosus (pVLS) can persist beyond puberty. Recent studies reveal that this condition may persist in as many as 75% of cases. The present study aims to answer the following query: how does pVLS evolve after menarche?, Methods: This observational retrospective study conducted on premenarchal girls diagnosed with pVLS in our institution between 1990 and 2011 describes 31 patients who returned for multidisciplinary clinical evaluation following menarche., Results: The mean follow-up time was 14 years. At the post-menarche clinical examination, patients were classified as follows: 58% were still affected by VLS, 16% presented with a complete remission of disease, and 26% were completely asymptomatic although with persistent clinical signs of VLS., Conclusions: In our series, pVLS persists following menarche in the majority of patients. These findings suggest the importance of a long-term follow-up even among patients who report resolution of symptoms following menarche., (© 2023 Wiley Periodicals LLC.)

Competing Interests: Conflicts of interest the authors have no potential conflicts of interest to disclose.

Background: Vulvar lichen sclerosus (VLS) is a chronic and recurrent dermatosis of an inflammatory nature with severe focal atrophy of the skin. Connective tissue changes are polymorphic and are still not taken into account in histological diagnostics due to the difficulty of interpreting routine histological methods. In this work, we use multiphoton microscopy (MPM) as a new imaging technique that provides detailed information about the organization of collagen fibers in the dermis based on a non-linear second harmonic generation (SHG) process., Objective: To determine the degree of connective tissue damage in lichen sclerosus using standard histological techniques and to reveal the diagnostic capabilities of multiphoton microscopy., Material and Methods: We studied 42 biopsies with a histopathological diagnosis of VLS and 10 biopsies of normal vulvar skin. Histological, histochemical and immunohistochemical evaluation was used in comparison with MPM data. Quantitative analysis included the determination of the thickness, length of collagen fibers and the average intensity of the SHG signal., Results: A comprehensive study of the skin showed 4 groups of changes that can be regarded as the degree of the dermis damage: initial, mild, moderate, severe. The affected area at the initial and mild degree has subtle changes, however, it is reliably identified by quantitative analysis of the SHG signal. So, the initial degree is characterized by thin (1.3-1.8 µm) long (56-69 µm) collagen fibers, with a moderate degree, the fibers are thickened (3.4-4.3 µm) and fragmented (22-37 µm). The affected area in moderate and severe cases undergoes homogenization, which is associated with the deposition of extremely thin (0.6-0.9 μm) short (16-28 μm) collagen fibers and the expression of type V collagen., Conclusion: Multiphoton microscopy in the second harmonic generation mode is a reliable method for identifying collagen fibers in tissues. The study made it possible to identify 4 degrees of the dermis damage in vulvar lichen sclerosus.

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.)

Background: Vulvar diseases are common in the general population and have a negative impact on the quality of life., Objectives: To describe our experience as dermatologists in the management of vulvar dermatosis consultations., Methods: A retrospective observational study was conducted with patients who attended monographic vulvar consultations over a 5-year period. Clinical information was obtained from the patient's charts., Results: 148 women were studied. Their mean age was 43.24 years (standard deviation: 15.15 years), with ages ranging from 4 months to 80 years. 53.4% of patients took between 2 and 5 years to seek medical attention for the first time. The most frequent diagnosis was lichen sclerosus (41.9%), irritative eczema of the vulva (14.9%), and lichen simplex chronicus (10.1%). 83.8% reported anogenital itching, 66.2% pain, and 45.9% dyspareunia. The most frequently prescribed treatment was ultra-potent topical corticosteroids (clobetasol propionate; 41.2%). Patients with lichen sclerosus were significantly older than those who presented with any of the other diseases. No differences were found in terms of either the time of disease evolution or in symptom presentation., Study Limitations: Retrospective study. Vulvar diseases with an infectious cause are usually managed in primary care, therefore, were not included. All patients were recruited from a single private hospital which limits the comparisons with the public health system., Conclusions: Vulvar diseases frequently occur and are associated with high morbidity. It is essential to promote the development of specific vulvar consultations in hospitals. Specialties such as dermatology, gynecology, urology, or physiotherapy must be part of these units., (Copyright © 2022 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. All rights reserved.)

Background: The first-line treatment for vulvar lichen sclerosus (VLS) is 3 months of topical corticosteroid therapy. However, limited evidence is available concerning the use of fat grafting and platelet-rich plasma as a second-line treatment for patients who do not respond to first-line treatment., Methods: This prospective single-center randomized pilot trial included 20 patients with a clinical and histological diagnosis of moderate to severe VLS. The patients in the treatment group (TG) received two infiltrations (at 3-month intervals) of nanofat mixed with platelet-rich plasma (PRP) into the vulvar area, while the control group (CG) received standard topical corticosteroid therapy. Fat was aspirated from the medial thigh or lower abdomen regions. Microfat was obtained after centrifugation and was emulsified to obtain a nanofat suspension. Treatment efficacy was determined by measuring changes in the vulvar skin elasticity, histopathology, and clinical signs, symptoms, and patient quality of life at after 1 year., Results: A total of 19 patients were finally assessed (9 TG and 10 CG). At the end of the study (1 year), there had been no significant improvement in vulvar skin elasticity. However, patients in the TG showed a significant improvement in their symptoms (itching, pain, burning, and dyspareunia) and clinical signs (cervical erosions, fissures, stenosis, and leukoderma). Analysis of skin biopsies revealed a significant decrease in all inflammatory cell types in the TG. No adverse events related to the autologous treatment were recorded., Conclusions: Compared with topical corticosteroids, two infiltrations delivered 3 months apart decreased the inflammation of the vulva and improved most of the clinical signs and symptoms associated with VLS. Nonetheless, no improvement in vulvar skin elasticity was derived from the autologous treatment., Level of Evidence I: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 ., (© 2021. Springer Science+Business Media, LLC, part of Springer Nature and International Society of Aesthetic Plastic Surgery.)

This review investigates women suffering from chronic vulval pruritus who may suffer from lichen sclerosus (LS). LS is a chronic inflammatory skin condition involving the vulva and anogenital regions. Treatment may be initiated upon suspicion, although the diagnosis is predominantly clinical and at times a skin biopsy is needed to confirm the diagnosis. LS is treated with potent to very potent topical steroids initially for three months. Prophylactic lifelong treatment is recommended to avoid long term scarring, sexual dysfunction, improve quality of life and to prevent cancer. Mild cases can be managed in general practice.

Objectives/purpose: Presenting symptoms of vulvar lichen sclerosus (LS) specific to premenopausal women are not well reported in the literature and may differ from those in postmenopausal women. This study aimed to characterize the presentation of vulvar LS among premenopausal women., Materials and Methods: An observational web-based study was conducted in premenopausal women with biopsy-confirmed vulvar LS between the ages of 18-50 years. Participants completed a 28-question survey evaluating characteristics of symptoms, timing of diagnosis, alternate diagnoses, and presence of concomitant autoimmune conditions., Results: Of the 956 responses received, 503 met inclusion criteria of biopsy-confirmed LS and premenopausal status. Average age of symptom onset was 27 years, and average age of diagnosis was 32 years, with a 4-year delay in diagnosis. Symptoms most present were dyspareunia (68%) and tearing with intercourse or vaginal insertion (63%). Symptoms that affect the individual most were also dyspareunia (44%) and tearing with intercourse or vaginal insertion (39%). Symptoms that most frequently prompted patients to seek medical attention were dyspareunia (35%), pruritus (31%) and tearing with intercourse or vaginal insertion (26%). Most common skin changes included hypopigmentation (81%), vulvar fissures (72%), and labial resorption (60%), with fissures affecting the individual the most (48%). Sixty-six percent of the respondents initially received an alternative diagnosis, most commonly vulvovaginal yeast infection (49%). Hypothyroidism was the most common concurrent autoimmune condition (10%)., Conclusions: Vulvar LS affects premenopausal women, commonly presenting with dyspareunia and tearing with intercourse. This condition should be considered and evaluated in premenopausal women presenting with vulvar symptoms and sexual pain., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2022, ASCCP.)

Lichen Sclerosus is a chronic-relapsing inflammatory skin disease usually involving the anogenital region lacking a resolutive therapy. Potent to high-potent topical corticosteroids are considered to be the standard first-line treatment. The objective of this preliminary study is to evaluate the efficacy of injecting Hybrid Cooperative Complexes of Hyaluronic Acid (HCC) in the treatment of Vulvar Lichen Sclerosus (VLS). Twenty-female patients (range 21-78 years) with histopathologic diagnosis of lichen sclerosus and good general conditions were enrolled. Patients underwent HCC infiltration every month, for three times. Patients had been assessed at baseline (T0) and after 1 and 6 months from treatment (T1 and T2, respectively). Clinical evaluation was executed in every visit. Itching, burning sensation, pain and dyspareunia were reported by a patient at T0, T1, and at T2. The effectiveness of the treatment on patients' quality life and sexual life was assessed using the Dermatology Life Quality Index (DLQI) at T0 and at T2. During or after the treatment no complications or side-effects were observed. All patients had a significant reduction in most symptoms after 1 and 6 months of HCC treatment. The reduction of patients with itching (p ≤ 0.001), pain (p = 0.031), burning sensation (p = 0.004) at 6 months is significant. The analysis of DLQI scores revealed a significant improvement in patients' quality of life. The DLQI mean score (±SD) at baseline and at follow-up was 5.89 ± 3.68 and 3.42 ± 2.36 (p = 0.002), respectively. Our preliminary study has demonstrated the safety and tolerability of HCC infiltrations in patients with VLS, and the effectiveness of HCC in reducing symptoms and, thus, to improve patient Quality of Life., (© 2022 Wiley Periodicals LLC.)

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.)

Objectives: Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease of unclear etiology. Recent studies show that 15-34% of cases in adult women and 14% in girls coexist with allergies or autoimmune diseases, among others - celiac disease (CD). Most of the research on the coexistence of VLS and autoimmune diseases has been carried out on a group of adult women. Literature data on this issue are very scarce., Material and Methods: The presented work is a pioneering project in which we tried to elucidate a possible relationship between celiac disease and lichen sclerosus in girls. The aim of the research was to study the antibodies characteristic of celiac disease in girls with VLS. The control group consisted of 35 heathy adolescent girls and the study group consisted of 20 girls aged 2-18 years old diagnosed with vulvar lichen sclerosus recruited at the Gynecological Clinic for Girls at the Women's Health Center in Katowice., Results: There were no significant differences in the concentrations of antibodies characteristic for CD in the blood serum between the studied groups., Conclusions: The main limitation of our study was the small size of the study group. It is therefore legitimate to conduct further research on a larger study group to find themutual correlations between the analyzed antibodies and the onset and the course of VLS in girls. The finding of a positive correlation between the coexistence of VLS and CD may prevent potentially serious, long-term complications.

Competing Interests: Competing interests: None declared.

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.)

Objectives: The purpose of this cross-sectional study was to prepare a reliable and easy-to-use architectural classification for vulvar lichen sclerosus (VLS) aimed at defining the morphological patterns of this condition., Materials and Methods: An expert panel composed by 7 physicians with expertise in clinical care of vulvar conditions outlined the architectural criteria for the definition of VLS severity (phimosis of the clitoris, involvement of the interlabial sulci, narrowing of the vulvar introitus), identifying 5 grades to build up a classification. Thirteen physicians with 2-30 years expertise in vulvar diseases (nonexpert group) were asked to evaluate 3-5 pictures from 137 patients. Each physician individually assigned a grade to each case, according to the previously mentioned criteria. Interrater reliability was analyzed by means of intraclass correlation coefficient (ICC). The reliability concerning the 2 classifications of each rater was analyzed by means of κ statistic. Intraobserver and interobserver reliability in vivo was analyzed by means of κ index., Results: This study provides a new classification of VLS, based on defined anatomical criteria and graded into mutually exclusive progressive classes.The ICC analysis showed a substantial interrater reliability of the classification, ICC = 0.89 (0.87-0.91), both in the expert panel and in the nonexpert group (ICC = 0.92 and 0.87, respectively). An "almost perfect" intraobserver and interobserver reliability was achieved among physicians in vivo (κ = 0.93)., Conclusions: Our classification showed a high reliability. It is easy to use, and it can be applied in clinical practice and eventually, in the evaluation of regenerative and cosmetic surgery., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2021, ASCCP.)

Vulvar vitiligo (VV) and vulvar lichen sclerosus (VLS), both feature skin and mucosal hypo-/depigmentation. The aim of this study was to describe the clinical and dermoscopic features of VV and VLS in the pediatric population, providing diagnostic clues, and to define their association. We performed a systematic literature review of the clinical and dermoscopic features of pediatric VV and VLS. An observational study was conducted on children affected by VLS associated with VV, referred to the Dermatology Unit of the Sant'Orsola Polyclinic in Bologna, Italy. Medical history, age at diagnosis, ethnicity, clinical and dermoscopic features, and symptoms were recorded for all patients. 124 cases of VLS and 10 cases of VV were reviewed. Clinical manifestations included hypo-/depigmented patches in both conditions, while ecchymosis/purpura and fissures/erosion were observed in VLS. Symptoms including pruritus, pain, or burning were reported only by VLS patients. In our study five patients with VLS associated with VV were retrieved. Clinical features included well-demarcated depigmented patches in VV and translucent areas, erythema, ecchymoses/purpura, and labial fusion in VLS. Dermoscopy showed white structureless areas with a whipped cream-like appearance, linear or dotted vessels, white chrysalis-like structures, erosion and red-purpuric blotches in VLS and reduced pigment network or pigment absence, intralesional spots of residual pigmentation and telangiectasias in VV. Symptoms were present in all patients. Both VV and VLS show hypo-/depigmented patches. In the presence of associated symptoms, possible VLS should be investigated with clinical and dermoscopic examination to achieve a prompt diagnosis., (© 2021 Wiley Periodicals LLC.)

Competing Interests: Financial Disclosure The authors did not report any potential conflicts of interest.

A complete clearance of vulvar lichen sclerosus (VLS) is achieved in a minority of patients treated with a standard 12-week duration corticosteroid treatment. The aim of this pragmatic, retrospective, open label, comparative trial was to assess the effectiveness, in terms of complete clearance, of a 24-week treatment with mometasone furoate 0.1% ointment (MMF) and to compare it with a 12-week therapy. We included VLS patients treated with MMF administered for five consecutive days/week for 24 weeks (group A). The following were assessed: (a) clearance in Global Subjective Score (GSS), Global Objective Score (GOS) or both, (b) changes of these parameters and dyspareunia at treatment completion compared to baseline, (c) safety profile. All these assessments were compared with the same outcomes recorded among VLS patients who had previously undergone a 12-week MMF treatment (group B). Twenty-nine patients were included in group A and 32 in group B. The rates of patients who achieved the clearance of GSS, GOS or both parameters did not significantly differ between groups A and B. The groups did not differ in any of the effectiveness outcomes assessed. A 24-week duration corticosteroid treatment does not seem to provide significant therapeutic benefits in comparison with standard 12-week courses, especially considering the occurrence of complete clearance., (© 2021 The Authors. Dermatologic Therapy published by Wiley Periodicals LLC.)

Squamous cell carcinoma of the vulva can arise through 2 pathways: human papillomavirus (HPV)-dependent high-grade squamous intraepithelial lesions (previously termed usual vulvar intraepithelial neoplasia) or HPV-independent (differentiated vulvar intraepithelial neoplasia, dVIN). Distinguishing between the 2 types can be clinically and histologically difficult. A subset of high-grade squamous intraepithelial lesions with superimposed chronic inflammation mimicking dVIN has recently been reported; p53 shows characteristic mid-epithelial staining (with basal sparing) in such cases. The pathology databases of 2 academic institutions were searched for vulva specimens with corresponding p53 and p16 immunohistochemical stains, yielding 38 specimens (from 27 patients). In situ hybridization and multiplex polymerase chain reaction-MassArray for high-risk HPV were performed on at least 1 block from each patient. All cases resembled dVIN or lichen sclerosus morphologically, but with a higher degree of atypia. All but 1 case demonstrated mid-epithelial p53 staining with basal sparing by immunohistochemistry. All cases showed block positivity for p16 and at least patchy positivity by HPV in situ hybridization. Of the 23 cases with valid HPV DNA polymerase chain reaction results, 15 were positive and 8 were negative. Of the positive cases, HPV16 was identified in 10 cases, with other high-risk types in the remaining 5. To our knowledge, this is the largest cohort of high-grade squamous intraepithelial lesions mimicking dVIN reported to date. Prior studies reported positivity for HPV16 in all cases tested, however, we found HPV16 in only 67% of HPV positive cases. This case series highlights the importance of immunohistochemistry, and occasionally HPV in situ hybridization, for accurate diagnosis, and expands the spectrum of associated HPV types., Competing Interests: R.W.L. is on the advisory board for Invio. The remaining authors declare no conflict of interest., (Copyright © 2020 by the International Society of Gynecological Pathologists.)

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.)

Vulvar lichen sclerosus (VLS) is a chronic inflammatory skin disease of the anogenitalarea leading to itch, burning, sexual dysfunction and impaired quality of life. An unmet need in the context of LS is a practical, easily assessable grading scale to classify disease severity and to allow intra- and interindividual comparisons. The objectives of this study were i) to assess the prevalence and severity of 23 items proposed by a recent Delphi consensus group in patients with adult VLS. ii) to develop a clinical severity scale and, iii) to test the interrater reliability of this novel severity scale. A retrospective assessment of the prevalence and severity of 23 items in 143 patients was performed by using patient records and photo documentation to develop a novel clinical severity scale (i.e. the "Clinical Lichen Sclerosus Score" = CLISSCO) for VLS. Thereafter, the CLISSCO was validated by 16 raters. We found that the items proposed by the consensus group vary markedly in frequency and severity. Following selection of the most relevant items, the CLISSCO was developed consisting of 3 "Symptoms", 3 "Signs" and 6 "Architectural changes" rated on a 0-4 point Likert-scale. The intraclass correlation coefficient was excellent for each item, the applicability of the CLISSCO considered user-friendly by the raters. We conclude that the CLISSCO proved to be a user-friendly, reliable tool to assess disease severity in VLS. However, further studies are needed to validate its applicability and value in daily practice and clinical research., (© 2021 Wiley Periodicals LLC.)

Objective: The aim of the study was to describe the features required for diagnosis of differentiated vulvar intraepithelial neoplasia (dVIN) and vulvar aberrant maturation (VAM)., Materials and Methods: The International Society of the Study of Vulvovaginal Diseases tasked the difficult pathologic diagnoses committee to develop consensus recommendations for clinicopathologic diagnosis of vulvar lichen planus, lichen sclerosus, and dVIN. The dVIN subgroup reviewed the literature and formulated diagnostic criteria that were reviewed by the committee and then approved by the International Society of the Study of Vulvovaginal Diseases membership., Results: Differentiated vulvar intraepithelial neoplasia is the immediate precursor of human papillomavirus (HPV)-independent vulvar squamous cell carcinoma and shows a spectrum of clinical and microscopic appearances, some overlapping with HPV-related neoplasia. The histopathologic definition of dVIN is basal atypia combined with negative or nonblock-positive p16 and basal overexpressed, aberrant negative, or wild-type p53. The most common pattern of dVIN is keratinizing with acanthosis, aberrant rete ridge pattern, and premature maturation. The morphologic spectrum of keratinizing dVIN includes hypertrophic, atrophic, acantholytic, and subtle forms. A few dVIN cases are nonkeratinizing, with basaloid cells replacing more than 60% of epithelium. Vulvar aberrant maturation is an umbrella term for lesions with aberrant maturation that arise out of lichenoid dermatitis and lack the basal atypia required for dVIN., Conclusions: Evaluation of women at risk for dVIN and VAM requires a collaborative approach by clinicians and pathologists experienced in vulvar disorders. Close surveillance of women with lichen sclerosus and use of these recommendations may assist in prevention of HPV-independent squamous cell carcinoma through detection and treatment of dVIN and VAM., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.)

Improvement in suffering after treatment has been poorly investigated in women affected with vulvar lichen sclerosus (VLS). We performed an observational study on a cohort of VLS patients for assessing the effect of a 12-week topical corticosteroid treatment on their VLS-related burden, as measured with Pictorial Representation of Illness and Self-Measure (PRISM) and Dermatology Life Quality Index (DLQI). Demographics and disease-related subjective and objective scores (at baseline, T0, and at the control visit, T1) were recorded. The PRISM and DLQI were administered at T0 and T1. We assessed the variation of PRISM and DLQI at T1 compared to baseline and the relevance of several variables on these changes. Sixty-three patients were included. A significant improvement was found in both PRISM and DLQI after treatment. A higher coefficient of variations was observed for PRISM and DLQI as compared to subjective and objective scores. Improvement of global subjective score after treatment was the sole variable associated with PRISM and DLQI variations. The corticosteroid treatment led to a significant decrease in the impact of VLS on patients' well-being, in terms of suffering and quality of life impairment. PRISM seems a reliable instrument for integrating clinicians' and patients' perspectives for a comprehensive VLS management., (© 2020 Wiley Periodicals LLC.)

Objective: The objective of this study was to test the severity rating of the signs and architectural changes for interrater reliability among world experts via analysis of lichen sclerosus (LS) photographs., Methods: A recent Delphi consensus exercise established a list of symptoms, signs, and architectural changes, which experts feel are important to include in a severity scale. Photographs of vulvar LS were manually extracted from patient charts and 50 photographs with a range of severity of signs and architectural changes were chosen. Lichen sclerosus experts were invited to take part in the study and 3 dermatologists and 3 gynecologists were selected for their expertise and geographic variety. Raters assessed the photographs for multiple signs and architectural changes as well as an overall impression of disease severity on a 4-point Likert scale. Intraclass correlation coefficients were calculated., Results: The intraclass correlation coefficients were very poor for individual signs and architectural changes as well as for overall disease severity when analyzed for all 6 raters as well as when analyzed with dermatologists' and gynecologists' responses grouped separately. There were no statistically significant correlations found., Conclusions: Global experts were unable to agree on any signs, architectural changes, or an overall global impression to assess vulvar LS disease severity based on analysis of vulvar photographs. Standardized descriptions regarding what constitutes mild, moderate, and severe signs and anatomical changes are required before further scale development can occur.

Objective: The aim of the study was to describe the clinical and histopathologic features required for a clinicopathologic diagnosis of vulvar lichen planus (LP), which is divided into 3 types: erosive, classic, and hypertrophic., Materials and Methods: The International Society of the Study of Vulvovaginal Diseases tasked the Difficult Pathologic Diagnoses committee with development of a consensus document for the clinicopathologic diagnosis of vulvar LP, lichen sclerosus, and differentiated vulvar intraepithelial neoplasia. The LP subgroup reviewed the literature and formulated diagnostic criteria, then approved by the International Society of the Study of Vulvovaginal Diseases membership., Results: The clinicopathologic diagnosis of erosive LP incorporates 5 criteria: (a) a well-demarcated, glazed red macule or patch at labia minora, vestibule, and/or vagina, (b) disease affects hairless skin, mucocutaneous junction, and/or nonkeratinized squamous epithelium, (c) evidence of basal layer damage, categorized as degenerative or regenerative, (d) a closely applied band-like lymphocytic infiltrate, and (e) absent subepithelial sclerosis. The clinicopathologic diagnoses of classic and hypertrophic LP each require a characteristic clinical appearance accompanied by hyperkeratosis, hypergranulosis, acanthosis, basal layer degeneration, a closely applied lymphocytic infiltrate, and absent dermal sclerosis, with hypertrophic LP showing marked epithelial abnormality compared with classic LP., Conclusions: Clinicopathological correlation yields the most reliable diagnosis of vulvar LP. Disease appearance overlaps with other physiologic, dermatologic, infectious, and neoplastic entities; a low threshold for biopsy at all morphologically distinct areas is recommended. Use of the histopathologic criteria described in this document may reduce the nondiagnostic biopsy rate for clinically diagnosed LP.

Background/objectives: The Vulval disease Quality of Life Index (VQLI) is a new tool that assesses the burden of vulval disease on quality of life (QoL). Our objective was to assess the correlation between VQLI score and clinician-rated severity scores, overall patient itch/discomfort, disease duration, sexual activity, and age, in vulval lichen sclerosus (VLS) at a vulval disorders clinic., Methods: A retrospective case note review, including consecutive women with VLS who attended the clinic between April and October 2018. Outcome measures include the VQLI score, clinician-rated severity score, and patient symptom score., Results: A total of 109 women with VLS were included. On multivariable analysis, there was evidence of a positive relationship between VQLI scores and the total clinician-rated score (mean increase in VQLI score per unit increase in clinician score 1.34, 95% confidence interval [CI] 0.31, 2.38; P = 0.01); the relationship was stronger for the cutaneous component. There was little evidence for relationships of the VQLI with the patient's age, sexual activity or time since onset of symptoms. There was strong evidence for a positive relationship between VQLI score and overall itch/discomfort score (mean increase 2.38, 95% CI 1.88, 2.88; P < 0.001). New and follow-up data were obtained on sequential visits for 12 women, among whom the VQLI score dropped a mean -2.75 points between visits (95% CI -6.05, 0.55; P = 0.094)., Conclusion: The clinician-rated severity correlates with the impact of VLS on QoL. The VQLI captures information included in a patient itch/discomfort score, which can be easily incorporated into routine assessment., (© 2019 The Australasian College of Dermatologists.)

Background: Vulvar lichen sclerosus (VLS) occurring in children and adolescents may have repercussions throughout life., Objective: We sought to assess the evidence available on the long-term consequences of juvenile VLS., Methods: Multiple databases were searched for studies containing long-term follow-up information on children or adolescents up to age 18 years with VLS. Articles were classified by level of evidence and the specific aspects of VLS studied., Results: In all, 37 studies met the inclusion criteria, giving information on the long-term consequences of VLS, of which 13 were cohort studies and 24 were case reports or series. These publications show that signs and symptoms persist after puberty and beyond, scarring and permanent architectural changes occur, treatment is effective with regard to symptoms, and long-term quality of life is affected. Findings suggest a possible relationship with risk of malignancy. The included publications had low-level evidence., Limitations: Meta-analysis was not possible because the studies had different focuses. Very few patients were followed into adulthood., Conclusions: There is low-level evidence suggesting long-term repercussions of juvenile VLS. Studies following children and adolescents with VLS into adulthood are needed to better understand the course of this disease and its repercussions on adult vulvar health., (Copyright © 2019 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)