Vulvar lichen sclerosus (LS) is a chronic dermatosis for which the true prevalence is not well established. LS remains an underrecognized and undertreated disease, and treatment patterns and differences among various specialties that care for patients with LS are largely unknown.This study sought to determine the prevalence of diagnosed vulvar LS, demographic characteristics, and diagnostic and treatment patterns in the United States.A database comprising >21.7 million women with commercial insurance in the United States was analyzed, and the largest cohort of patients with LS in the United States to date was identified.We found that obstetricians/gynecologists are diagnosing and managing approximately half of women with LS, and regardless of specialty, the majority of providers are appropriately prescribing high-potency topical steroids.Our results suggest that LS may truly be underrecognized and underdiagnosed, especially in younger women.© 2020 Published by Elsevier Inc. on behalf of Women's Dermatologic Society.

Lichen sclerosus (LS) of the vulva in children is presenting more commonly, but its origin is unknown.The purpose of this study was to assess the prevalence of childhood vulvar LS, to examine factors that may play a role in its cause, and to attempt to define the current optimal treatment.We surveyed 70 cases of LS currently being treated in the pediatric vulvar clinic to collect information on presentation, family history, associated disease, and typical course of the disorder. We looked retrospectively and prospectively at treatments for LS.We found a prevalence of premenarchal LS of 1 in 900 and confirmed an increasing incidence. In a high proportion of cases (77%), the question of sexual abuse had been raised. A family history of LS was found in a minimum of 17% of cases. Associated autoimmune disease occurred in 14%, but 64% told of a family history, and atopy occurred in 59%. Potent topical steroid ointments appear to alleviate symptoms most effectively, with minimal side effects.Although the cause of LS remains uncertain, it is not a rare disease in prepubertal girls. Current optimal management includes prompt diagnosis and institution of treatment with a potent topical steroid.

Vulvar lichen sclerosus (VLS) and possibly vulvar lichen planus (VLP) are associated with an increased vulvar cancer (VC) risk. We analyzed the risk of VC and its precursors after a diagnosis of VLS or VLP.A search was performed to identify articles describing the development of vulvar neoplasia in women with VLS or VLP. This systematic review was registered with the PROSPERO database.Fourteen studies on VLS included 14,030 women without a history of vulvar neoplasia. Vulvar cancer, differentiated vulvar intraepithelial neoplasia (dVIN), and vulvar high-grade squamous intraepithelial lesion occurred in 2.2% (314/14,030), 1.2% (50/4,175), and 0.4% (2/460), respectively. Considering women with previous or current VC, the rate was 4.0% (580/14,372). In one study, dVIN preceded VC in 52.0% of the cases. Progression of dVIN to VC was 18.1% (2/11).The risk was significantly higher in the first 1-3 years after a biopsy of VLS and with advancing age; it significantly decreased with ultrapotent topical steroid use.For the 14,268 women with VLP (8 studies), the rates of VC, dVIN, and vulvar high-grade squamous intraepithelial lesion were 0.3% (38/14,268), 2.5% (17/689), and 1.4% (10/711), respectively.Vulvar lichen sclerosus is associated with an increased risk of VC, especially in the presence of dVIN and with advancing age. Ultrapotent topical steroids seem to reduce this risk. An increased risk of developing VC has been suggested for VLP. Hence, treatment and regular life-long follow-up should be offered to women with VLS or VLP.Copyright © 2022 by the American Society for Colposcopy and Cervical Pathology.

The aim of the study was to describe the demographic, clinical, and histopathologic features of differentiated vulvar intraepithelial neoplasia (dVIN) and vulvar aberrant maturation (VAM).Specimens from 2010 to 2020 reported as dVIN or VAM were reviewed. Clinical data included age, rurality, symptoms, and evidence of lichen sclerosus (LS). Histopathologic data included epithelial thickness, keratinization, architectural and dyskeratotic features, stroma, p16, and p53. Differentiated vulvar intraepithelial neoplasia and VAM were distinguished by assessment of basal nuclear chromatin, enlargement, pleomorphism, and mitoses.One hundred twenty women with a median age of 71 years had 179 examples of dVIN and VAM. Squamous cell carcinoma was concurrent in 66% and associated with rurality. Ten percent were asymptomatic, and all but 3 had evidence of LS. Differentiated vulvar intraepithelial neoplasia showed a range of thickness, architecture, and dyskeratosis; its unifying !feature was basal atypia. Differentiated vulvar intraepithelial neoplasia displayed hyperchromasia in 83% and easily observed mitoses in 70%. Nonkeratinizing morphology, subcategorized into basaloid and intermediate, occurred in 24% of women with dVIN. Traditional dVIN represented 62% of keratinizing cases; the remainder were atrophic (13%), hypertrophic (13%), acantholytic (8%), or subtle (5%). Vulvar aberrant maturation had abnormal stratum corneum, acanthosis, premature maturation, and enlarged vesicular nuclei. Null p53 helped distinguish dVIN from VAM and dermatoses.The morphology of dVIN encompasses nonkeratinizing and keratinizing types, the latter subdivided into traditional, acantholytic, atrophic, hypertrophic, and subtle. Diagnosis relies on basal atypia with supportive p16 and p53. Atypia exists on a biologic spectrum with mild abnormalities of VAM and reactive change. Identification of dVIN and VAM requires collaboration between clinicians and pathologists experienced in vulvar disorders.

The aim of the study was to describe the features required for diagnosis of differentiated vulvar intraepithelial neoplasia (dVIN) and vulvar aberrant maturation (VAM).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.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.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.Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.

        女性外阴硬化性苔藓（vulvar lichen sclerosus，VLS）是一种常见的外阴慢性炎症性非瘤样皮肤病变。本病以外阴及肛周的皮肤和黏膜萎缩变薄为主要特征，呈慢性进展伴反复发作。不及时规范的治疗可导致外阴萎缩、粘连、瘢痕形成，甚至外阴丧失正常解剖及功能，局部发生癌变的风险亦有所升高。浏览更多请关注本刊微信公众号及当期杂志。

Women with lichen sclerosus (LS) may suffer sexually because of dyspareunia, fissures, and introital narrowing. However, the literature remains limited on the biopsychosocial aspects of LS and its impact on sexual health.

Lichen sclerosus (LS) is a chronic inflammatory condition that is known to arise on the vulva. Many women with LS report vulvar pain, often affecting a patient's quality of life. In this study, the sexual function of LS patients, with and without pain, was compared to control populations.A case-control study to examine the relationship between LS and sexual dysfunction was conducted. A total of 335 women presenting to the gynecology clinic were included in the study: 197 women with biopsy confirmed LS were compared to two control groups (95 asymptomatic women were "healthy" controls and 43 women had vulvovaginal candidiasis) on self-reported current health complaints, medical and surgical history and current symptoms such as pain and itching, type and frequency of sexual activity, and satisfaction with sexual activity.Women with LS reported less frequent sexual activity than healthy controls (p=0.007) and Candida controls (p=0.04). Currently sexually active women with LS were significantly less likely to report vaginal intercourse (71.6%) than healthy controls (89.0%, p=0.003) or Candida controls (100%, p=0.0003), even though similar proportions of all three groups reported that vaginal intercourse was important. Satisfaction towards the quality of current sexual activity was significantly lower among women with LS compared with both the healthy and Candida control groups. 23.7% of women with LS reported that sexual activity was rarely or never satisfactory as compared with 0% of healthy controls (p<0.0001) and 6.5% of Candida controls (p=0.03).Women with LS have less frequent sexual activity and less satisfying sexual activity when compared with controls.

The vulva is composed of aesthetic units that can be affected differently by vulvar conditions. A reliable, comprehensive, and quick-to-use clinical scoring system is required to assess the disease extent in the vulvar area.The aim of this study was to develop and validate a grading scale based on the aesthetic unit principle to evaluate the extent of vulvar lichen sclerosus (VLS).After reviewing photographs of 100 patients affected by VLS, the authors targeted the aesthetic units most frequently affected. The disease signs were recorded and graded in 4 levels of severity (none, mild, moderate, severe) taking into account the vulvar architecture and skin involvement. To validate the scale, 14 observers were asked to apply it to photographs of 25 VLS patients on 2 different occasions. Intra- and inter-observer reliabilities were determined employing Pearson's and intraclass correlation coefficients.A 6-region, 4-point grading system was designed and identified as the Vulvar Architecture Severity Scale (VASS). In all 6 areas, the Pearson's r was greater than 0.9 (mean, 0.994; 95% confidence interval [CI] = 0.992), indicating that the intra-observer reliability of the VASS was consistent over time (P < 0.001). Intraclass correlation at time 1 was 0.928 (95% CI = 0.910, 0.943) and at time 2 was 0.944 (95% CI = 0.931, 0.996), indicating a high reliability level among different observers.The VASS is a reliable scale to assess the severity of VLS, and it might be considered as an outcome measure in future VLS trials.© 2020 The Aesthetic Society. Reprints and permission: journals.permissions@oup.com.

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.

To record the clinical features, symptomatic response to topical steroids, and resolution of clinical signs in a large cohort of female patients with vulvar lichen sclerosus.Descriptive cohort study with a mean follow-up of 66 months.The vulvar clinics of a teaching hospital and of a district general hospital in Oxfordshire, England.Three hundred twenty-seven patients (74 girls and 253 women) with a definite clinical diagnosis of vulvar lichen sclerosus. Interventions The patients received topical steroids as part of their normal care.Symptomatic response to treatment (good, partial, or poor); response of the vulvar signs (total, partial, minor, or poor); and the presence or absence of moderate or severe scarring.The mean age at onset was 5.4 years in girls and 55.1 years in women and first-choice therapy was an ultrapotent topical steroid for 50% of the girls and 89% of the women. Response to treatment was recorded in 255 patients. In 244 patients (96%) symptoms improved with treatment, as 168 (66%) became symptom free and 76 (30%) showed partial response; 11 (4%) had poor response. Among the 253 patients in whom a response of the vulvar signs to topical steroid was recorded, 58 (23%) showed total response, with return to normal skin texture and color; and 173 (68%) showed partial, 18 (7%) showed minor, and 4 (2%) showed poor response. Moderate or severe scarring occurred less often in girls (P<.001). Squamous cell carcinoma developed in 6 women (2.4%).Topical ultrapotent steroid is an effective treatment for vulvar lichen sclerosus, giving relief of symptoms in most and completely reversing the skin changes in approximately one fifth of patients.

The purpose of the present systematic review is to evaluate the available medical treatments for vulvar lichen sclerosus, using an arm-based network meta-analysis protocol.We searched Medline (1966-2019), Scopus (2004-2019), Cochrane Central Register of Controlled Trials CENTRAL (1999-2019), Clinicaltrials.gov (2008-2019) databases, and Google Scholar (2004-2019) database along with the reference list of all included studies.All observational, randomized, and single-arm studies that evaluated medical treatments for vulvar lichen sclerosus were considered eligible for inclusion in the present systematic review.A network meta-analysis was carried out in R 3.4.3 using the pcnetmeta package, which uses a Bayesian hierarchical model (based in Markov chain Monte Carlo convergence simulation).Sixteen studies were included in this present meta-analysis, which recruited 954 women with vulvar lichen sclerosus. Their quality was evaluated with the JADAD, Cochrane risk of bias, and risk of bias in nonrandomised studies of interventions-I tools. Clobetasol treatment ranked as the best treatment for disease remission after evaluating rank probabilities (40% chance of ranking first compared with tacrolimus [38%]). However, the density plot revealed partial overlapping with tacrolimus. The lowest probability of experiencing a relapse was observed with pimecrolimus (15% [2-48%]); however, the density plot revealed significant overlapping with mometasone furoate, testosterone, and clobetasol.Robust evidence concerning the superiority of potent steroids at least over calcineurin inhibitors is still lacking in the field of vulvar lichen sclerosus. On the other hand, the gross heterogeneity in terms of selected population, duration of treatment, administered regimen, outcome reporting, and selection of outcome measures leaves several fields unanswered.Copyright © 2019 Elsevier Inc. All rights reserved.

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.

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.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.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).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.Copyright © 2024, ASCCP.

To identify reasons for ongoing poor quality of life (pQOL) in a subset of long-term topical corticosteroid-treated vulval lichen sclerosus (VLS) patients.A prospective cross-sectional study of patients attending a dermato-gynaecology practice in Sydney, Australia, comparing VLS patients with good quality of life (gQOL) and pQOL, in pre-treatment and long-term treatment groups, using the Vulval Quality of Life Index (VQLI). Demographics, VQLI scores and treatment characteristics were compared between gQOL and pQOL patients.A total of 255 biopsy-proven VLS patients, 67 in pre-treatment and 188 in long-term treated groups were considered. There were 33 (49.3%) pQOL patients in pre-treatment and 13 (6.9%) in treatment groups (p < 0.001). The highest-scoring domain in treated pQOL patients was sexuality (1.7 [interquartile range (IQR) 1.0-2.0]), followed by anxiety [1.3 (IQR 1.0-1.5]), symptoms (1.0 [IQR 0.5-1.5]) and activities of daily living (0.7 [IQR 0.3-1.0]). Compared to treated gQOL, treated pQOL had significantly higher proportions of patients with partial treatment adherence (8 [61.5%] vs 42 [24.0%], p = 0.006), suboptimal disease control (7 [53.8%] vs 20 [11.4%], p < 0.001), scarring progression (3 [23.1%] vs 7 [4.0%], p = 0.024) and urinary incontinence (5 [38.5%] vs 27 [15.4%], p = 0.049).Only a minority of long-term treated VLS patients reported ongoing pQOL. Of those who did, sexuality and anxiety domains were found to be the main sources of distress. Three major areas distinguishing gQOL from pQOL patients were (1) treatment adherence and disease control, (2) psychological factors and (3) urinary incontinence.© 2022 The Authors. Australasian Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Australasian College of Dermatologists.

Lichen sclerosus (LS) is a chronic inflammatory dermatosis mostly localized in the genital area, characterized by vulvar alterations that can severely impact a patient’s quality of life. Current treatment modalities often provide incomplete relief, and there is a need for innovative approaches to manage this condition effectively. Platelet-rich plasma (PRP) and adipose-derived stem cells (ADSCs) have emerged as potential regenerative therapies for LS, offering promising results in clinical practice. This comprehensive review explores the utilization of PRP and ADSC therapy in the treatment of genital LS, highlighting their mechanisms of action, safety profiles, and clinical outcomes. PRP is a blood product enriched in growth factors and cytokines, which promotes tissue regeneration, angiogenesis, and immune modulation. ADSC regenerative potential relies not only in their plasticity but also in the secretion of trophic factors, and modulation of the local immune response. Numerous studies have reported the safety of PRP and ADSC therapy for genital LS. Adverse events are minimal and typically involve mild, self-limiting symptoms, such as transient pain and swelling at the injection site. Long-term safety data are encouraging, with no significant concerns identified in the literature. PRP and ADSC therapy have demonstrated significant improvements in LS-related symptoms, including itching, burning, dyspareunia, and sexual function. Additionally, these therapies enable many patients to discontinue the routine use of topical corticosteroids. Several studies have explored the efficacy of combining PRP and ADSC therapy for LS. In combination, PRP and ADSCs seem to offer a synergistic approach to address the complex pathophysiology of LS, particularly in the early stages. The use of PRP and ADSC therapy for genital lichen sclerosus represents a promising and safe treatment modality. These regenerative approaches have shown significant improvements in LS-related symptoms, tissue trophism, and histological features. Combination therapy, which harnesses the synergistic effects of PRP and ADSCs, is emerging as a preferred option, especially in early-stage LS cases. Further research, including randomized controlled trials and long-term follow-up, is warranted to elucidate the full potential and mechanisms of PRP and ADSC therapy in the management of genital LS. These regenerative approaches hold great promise in enhancing the quality of life of individuals suffering from this challenging condition.

Vulvar lichen sclerosus (LS) is a chronic, progressive, autoimmune dermatologic condition that causes cutaneous changes accompanied by pruritus and pain. There remains a small population with vulvar LS refractory to topical corticosteroids. Injection of platelet-rich plasma (PRP) has been reported to have positive effects on tissue repair. The aim of this pilot study was to evaluate changes in symptom scores during and after PRP vulvar infiltration.

Lichen sclerosus (LS) is a disease affecting mostly genital and perianal areas. Photodynamic therapy (PDT) has gained interest during the past years. The present study accumulates current evidence on the efficacy of PDT in the management of vulvar LS.We used Medline (1966-2017), Scopus (2004-2017), ClinicalTrials.gov (2008-2017) and Cochrane Central Register of Controlled Trials CENTRAL (1999-2017) databases in our primary search along with the reference lists of electronically retrieved full-text papers.Eleven studies were finally included in our systematic review, which recruited 337 women. The existing evidence supports that PDT results in significant relief of symptoms related to LS, hence remains confusing in evaluating the progress in the clinical appearance of the lesion. No major adverse effects were reported during therapy and during the posttreatment period. Pathologic findings seem to be conflicting, as current data do not unanimously support a beneficial histological effect.According to the findings of our study, PDT seems to be promising in the treatment of patients with vulvar LS. Nonetheless, current knowledge is extremely limited, and further observational studies with large patient series are needed in the field to elucidate the efficacy of PDT.