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Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease initially involving anogenital areas. Noninvasive assessment is essential for precise management in VLS. We aim to analyze high-frequency ultrasound (HFUS) features and correlate HFUS with histopathological changes.Forty patients with histopathologically confirmed VLS lesions were retrospectively identified from August 2020 to September 2021. The clinical manifestations, dermoscopic images as well as both 20 and 50 MHz HFUS images were assessed. HFUS assessment included epidermal morphology, hypoechoic dermal band thickness, and hypoechoic dermal band internal echo. We compared HFUS images with histopathology, and Pearson's correlation coefficient was used to assess the relationship between hypoechoic dermal band thickness and histopathological depth.Hypoechoic dermal band was present in 100% (40/40) VLS lesions. There was a significant linear positive correlation between the histopathological depth and corresponding hypoechoic dermal band thickness, with a Pearson correlation coefficient of 0.685 (p 0.001). Besides, 95% (38/40) lesions revealed smooth epidermis, and the internal echo of hypoechoic dermal band was assessed as homogeneous in 60% (24/40) and inhomogeneous in 40% (16/40) lesions.HFUS characteristics, as well as measurable hypoechoic dermal band thickness, may provide valuable information in the precise diagnosis and the treatment monitoring of VLS.

Pseudoepitheliomatous keratotic and micaceous balanitis (PEKMB) is a clinicopathological entity characterized clinically by micaceous scale on the glans, and histologically by acanthosis, hyperkeratosis and pseudoepitheliomatous hyperplasia. We present a series of eight cases of this rare condition, the first series of more than two cases to be reported.To determine the clinical and histological characteristics of cases of PEKMB, and evaluate treatments used and clinical course.This monocentric case series was conducted at the University College London Hospitals tertiary male genital dermatology clinic between April 2018 and August 2020. Eight patients with PEKMB were evaluated. Data were collected on demographics, clinical presentation, histological features, presence of human papilloma virus (HPV), history of lichen sclerosus, treatment of PEKMB and subsequent response, and presence or development of squamous cell carcinoma (SCC) or penile intraepithelial neoplasia (PeIN) during follow-up.Eight Caucasian males presented with clinical and histological evidence of PEKMB. Seven had a background of lichen sclerosus; two had failed treatment with superpotent topical steroids and four had symptoms for three or more years prior to circumcision. There was no clinical or histological relationship with HPV infection, and p16 staining was negative. HPV PCR, performed in two cases, was negative. Basal atypia, insufficient to amount to PeIN, was present in six patients. One patient progressed to PeIN during follow-up, and no patient progressed to invasive malignancy. Five patients were treated successfully with glans resurfacing and split skin graft reconstruction.Our observations demonstrate that PEKMB represents a form of chronic, undiagnosed or misdiagnosed, inadequately treated or treatment refractory, unstable lichen sclerosus. The significant potential for squamous carcinogenesis (differentiated PeIN and verrucous carcinoma) can be mitigated by timely diagnosis and treatment. Glans resurfacing and split skin graft reconstruction appears to be a successful treatment modality in patients with refractory disease.

Eccrine squamous syringometaplasia (ESS) has been associated with several conditions including morphea, linear scleroderma, and burns. It is yet to be reported in lichen sclerosus et atrophicus (LSA). We describe a bullous LSA plaque on the forearm of a woman with pre-existing genital LSA and vitiligo. Besides the histopathological findings of bullous LSA, numerous small irregular squamoid structures were present in the mid and upper dermis always above the normal eccrine glands. The histopathology, periodic acid-Schiff stain, and positive immunostains for P63, low molecular weight keratins 818, epithelial membrane antigen, and carcinoembryonic antigen supported the diagnosis of ESS. The pathogenesis of ESS in LSA may be related to ischemia, inflammation, and fibrosis.

Vulvar dermatoses are often debilitating chronic skin conditions associated with pain and pruritus. In oncologic patients, cancer treatments can precipitate and exacerbate vulvar dermatoses. Cytotoxic chemotherapy, hormonal therapies, and local pelvic radiation therapy can lead to vulvar symptoms, and cancer treatment-induced vulvar conditions include graft-versus-host-disease and radiation dermatitis. There have also been reports of lichen sclerosus development or exacerbation secondary to hormonal therapy and immune checkpoint inhibitors, attributed to proposed hormonal and immunologic pathogenesis of lichen sclerosus. Early recognition and treatment of these conditions can significantly improve quality of life. In this review, we summarize the clinical features and management characteristics of six types of common vulvar dermatoses that may present in the oncologic patient.

Background Early inflammatory lesions of lichen sclerosus are histopathologically difficult to diagnose until the hallmark of the disease i.e., papillary sclerosis becomes visible in histological sections. Pre-sclerotic and late or resolved phases of the disease have not been extensively studied. Methods We retrospectively reviewed all cases diagnosed as genital lichen sclerosus over a ten-year period from 2006 to 2016, correlating the clinical findings with the histological features. Results A total of 133 cases of genital lichen sclerosus (90 males and 43 females) were identified. Both genders demonstrated a similar histological spectrum. Fifty eight (44%) cases were identified as having pre-sclerotic lichen sclerosus, 64 (48%) as having progressive disease and 11 (8%) cases were classified as fully resolved with atrophy. Asymptomatic vitiligoid lesions were identified in 19 (14%) cases of which 12 were male. Low-grade squamous cell carcinoma was seen within the areas affected by long-standing lichen sclerosus, in four patients (3%, 2 male). Limitations We studied only haematoxylin and eosin stained sections. The presence of basement membrane thickening could have been better illustrated with the periodic acid–Schiff stain. Conclusion The pathogenesis of lichen sclerosus probably involves an immune reaction to the basement membrane at the epidermal interface and around the adnexa. The initial band of inflammation shifts gradually downwards from the epidermal interface into the dermis destroying the vascular channels and appendages, resulting in excessive deposition of altered extracellular matrix. Basilar infiltration of lymphocytes along with a grossly vacuolated or thickened basement membrane is proposed as the characteristic diagnostic feature of the pre-sclerotic stage. Greater awareness of the clinicopathological spectrum of lichen sclerosus should enable early diagnosis and treatment, thereby preventing structural damage and possible malignant transformation in chronic cases.

Lichen sclerosus (LS) is a chronic inflammatory dermatosis that mostly affects the genital and anal skin areas. Symptoms may vary from pruritis and pain to sexual dysfunction; however, LS can also be asymptomatic. LS occurs at all ages and in both sexes. Approximately 5% of all women affected by vulvar LS will develop vulvar squamous cell carcinoma. Topical treatment is safe but less effective resulting in chronic course in most patients, who suffer from persistent itching and pain. In severe cases of therapy-resistant LS, there is no adequate treatment. Fat grafting is a novel regenerative therapy to reduce dermal fibrosis. The therapeutic effect of adipose tissue grafts for LS is already investigated in various pioneering studies. This review provides an overview of these studies and the putative mechanisms-of-action of fat grafting to treat LS.

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.

BACKGROUND: Lichen sclerosus (LS) affects the female anogenital area, causing anatomical changes. Reported symptoms include itching, soreness and dyspareunia.AIM: To evaluate the quality of life and sexual functioning in women with LS.METHODS: In total, 158 women aged over 18 years, diagnosed with LS, and referred to North Denmark Regional Hospital from January 2018 to November 2019, were included. Three questionnaires, the Female Sexual Function Index (FSFI), Dermatology Life Quality Index (DLQI) and the World Health Organization Five-Item Well-Being Index (WHO-5), were completed.RESULTS: The women (mean age 47 years, range 18-76) presented a low score on all FSFI scales, with a mean score of 13.83 (95% CI 12.46-15.20), indicating reduced sexual functioning. The subgroup evaluation scored for desire 2.32, arousal 2.23, lubrication 2.39, orgasm 2.28, satisfaction 3.02 and pain 1.59. The DLQI results revealed a mean score of 7.88 (95% CI 7.02-8.74), indicating a moderate effect on the women's everyday life. The mean subscores were treatment 0.32, sexual difficulties 1.56, relations 1.02, work/study 0.34, sport 0.45, social activities 0.54, clothing 0.89, shopping 0.22, embarrassment 0.99 and itching, soreness and pain 1.55. The mean score for the WHO-5 was 56.66 (95% CI 53.48-59.84) indicating that 40% of the women had signs of depression.CONCLUSION: LS has a considerable influence on the sexual functioning and quality of life of women. Healthcare professionals must not only consider the biological aspects but also the psychological and social aspects.

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.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).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.

Performing 1-stage urethroplasty in patients with urethral strictures caused by lichen sclerosus (LS) is hotly debated among reconstructive urologists due to conflicting reports of success. Therefore, the objective of this study was to determine the pooled incidence of stricture recurrence following 1-stage buccal mucosal graft (BMG) urethroplasty in patients with LS, to determine the impact of surgical technique on recurrence and to compare recurrence risk between patients with and without LS after 1-stage repairs.A systematic review was conducted in accordance with PRISMA criteria. The primary outcome was pooled incidence of recurrence, which was calculated using a Der-Simonian-Laird binary random effects model with a Freeman-Tukey arcsine transformation. A total of 21 studies were included, of which 15 provided data for comparative analyses.Pooled data from 625 LS patients revealed a stricture recurrence rate of 10% (95% CI 6-14). Among studies with longer followup (≥24 months), this increased to 18%. Among patients with penile urethral involvement, studies utilizing a penile skin incision had significantly higher pooled recurrence rates than those utilizing penile invagination (p=0.004). Across all studies, there was no evidence to suggest a difference in pooled recurrence rate between patients with and without LS (p=0.36). However, across only long-term studies, recurrence risk was significantly higher for patients with LS (OR 1.83, p=0.05).One-stage BMG urethroplasty is likely a viable surgical option for patients with LS-related strictures; however, high-quality data are limited. Future multi-institutional, long-term prospective studies are needed to assess durability of 1-stage repair.

Penile cancer is relatively rare in North America and Europe (1% of all malignant neoplasms); however, it remains a significant health concern with a higher propensity of cases in many African, South American, and Asian countries. It occurs primarily in older men with a peak incidence in the 6th decade of life. The etiology of penile cancer is multifactorial and there are many risk factors including lack of neonatal circumcision, chronic inflammation, lichen sclerosis, tobacco use, obesity, poor hygiene, exposure to ultraviolet radiation, history of sexually transmitted diseases, and human papillomavirus (HPV) infection. Pathogenesis of penile squamous cell carcinoma (PSCC) can be broadly dichotomized into HPV related and non-HPV-related pathways which will be discussed in detail in this review.

Genital lichen sclerosus decreases the quality of life of women; 10-15% of cases occur in prepubertal girls.Retrospective and descriptive study on the characteristics of girls diagnosed with genital lichen sclerosus at the Hospital Universitario de Navarra (Pamplona, Spain) between 2019 and 2022.Eleven girls aged between 4 and 14 year-old were diagnosed. Frequently, diagnostic delays were up to two years after the appearance of the lesions; the girl with a four-year delay showed a significant vulvar architectural alteration. All cases showed the typical sclerotic lesions on the genital area, and two of them also on the back. While six patients were asymptomatic, the rest reported pruritus and/or pain. Treatment with high/very high potency topical cortico-steroids achieved a good partial response, without complete remission of the lesions.Early diagnosis of genital lichen sclerosus is key to start early treatment, avoiding ireversible genital structural alteration.

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory disease. The clinical presentation is clustered distribution of porcelain white spots that can be fused together. Early diagnosis of LSA is difficult, especially in the vulva, where it is difficult to distinguish from vitiligo. Traditional diagnostic methods mainly rely on histopathology. In this study, we used reflectance confocal microscopy (RCM) to study the characteristics of LSA and vitiligo. We investigated the clinical and confocal features of 40 cases of histopathologically proven genital LSA or vitiligo, and correlated the features with histopathologic findings. Through the analysis of the results, by RCM, we can easily distinguish genital LSA from vitiligo, which are clinically indistinguishable. This can reduce the number of unnecessary biopsies, and help with early diagnosis and timely treatment.

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.

硬化萎缩性苔藓（lichen sclerosus et atrophicus，LSA）是一种原因不明的可能累及皮肤及黏膜的慢性疾病，好发于女性外阴部。本文报道1例罕见的仅累及口腔，同时还伴有唇部美观缺陷及牙周附着丧失的LSA病例，通过局部药物治疗并结合牙周手术治疗，缓解了患者的临床症状，随访2年病情稳定未复发。.

Acquired elastotic hemangioma (AEH) is a rare variant of hemangioma that usually presents as an asymptomatic, solitary, slow-growing red plaque on a sun-exposed site of an adult. Ultraviolet radiation can contribute to the pathogenesis of this hemangioma. Lichen sclerosus (LS), a scarring disease, may present a prominent accumulation of elastic fibers in the reticular dermis reflecting a reparative process. Vulvar elastosis, a novel diagnostic entity with fibers similar to solar elastosis, is more common in women 45 yr and older and is related to aging and/or hormonal changes. We herein report for the first time a case of AEH-like change located in the vulva, a sun-protected area, associated with genital LS. An 81-yr-old woman presented with a painful vulvar lesion of 1-yr duration. Clinical examination revealed vulvar LS and 1 cm-flat, erythematous, well-defined plaque with increased consistency located on the left labium minus. Histopathology showed a non-neoplastic proliferation of WT1-positive, small vascular channels surrounded and intertwined by intense elastosis in the reticular dermis. Alpha-smooth muscle actin positive pericytes encircled the vascular channels. The lesion can be understood as a reparative process within an LS with the appearance of epidermal hyperplasia, proliferation of small vascular channels, and hyperplastic elastosis. There is a close link between epidermal hyperplasia and angiogenesis in the formation of this reparative lesion. Recognition of this lesion is crucial to avoid confusion with other significant processes especially Kaposi sarcoma and well-differentiated angiosarcoma.

The benefits and harms of the available types of surgical management for lichen sclerosus-related (LS) strictures remain unclear and, thus, clear and robust clinical practice recommendations cannot be given. To assess the role of single-stage OMGU in the management of LS strictures and explore how its benefits and harms compare with the alternative management options. Medline, Embase and Cochrane controlled trial databases (CENTRAL, CDSR) were systematically searched. Randomized (RCTs) and nonrandomized studies (NRCSs) comparing single-stage OMGU with other surgical management options for LS strictures and single-arm studies on single-stage OMGU were included. Risk of bias (RoB) was assessed. Of the 1912 abstracts identified, 15 studies (1 NRCS and 14 single-arm studies) were included, recruiting in total 649 patients. All studies were at high RoB. In the only NRCS available, stricture-free rate (SFR) for single-stage and staged OMGU was 88% vs 60%, respectively (p = 0.05), at a mean follow-up of 66.5 months. SFR range for single-stage OMGU in single-arm studies was 65–100% (mean/median follow-up, 12–59 months). Single-stage OMGU had low complication rates and beneficial impact on LUTS and QoL. The present SR highlights the methodological limitations of the available literature. In the absence of adverse local tissue conditions, and taking into consideration benefit–harm balance and surgeon’s skills and expertise, single-stage OMGU can be justified in patients with LS strictures.

Lichen sclerosus of the vulva (LSV) is seen frequently enough to warrant knowing how to diagnose it and institute appropriate treatment strategies. LSV is a chronic skin disorder, very likely of autoimmune origin, which may affect various areas of the perineum, although some women may be affected in extragenital areas. The disease has significant adverse impact on quality of life and sexual activity and may undergo malignant transformation. History of symptomatology and clinical examination is sufficient to make the diagnosis. Skin biopsy is only necessary in specific scenarios. Topical corticosteroids are still the mainstay of therapy, initially to institute remission and then for maintenance. Long-term therapy and surveillance are invariably necessary. Surgery does have a role, but only in specific associated conditions. A number of alternative treatment options have been mooted, especially if the disease is resistant to topical corticosteroids, but these options are still being assessed and studied.

Objective The aim of the study was to identify whether erosive lichen sclerosus (LS) is a distinct clinicopathologic subtype. Materials and methods The pathology database was searched for "erosion," "erosive," "ulcer," and "lichen sclerosus." Inclusion criteria were histopathologic diagnosis of LS and erosion or ulcer overlying a band of hyalinization and/or fibrosis. Exclusions were concurrent neoplasia and insufficient tissue. Histopathologic review documented site, epithelial thickness, adjacent epidermal characteristics, infiltrate, and dermal collagen abnormality. Clinical data included demographics, comorbidities, examination findings, microbiologic results, treatment, and response. Results Ten examples of erosive LS and 15 of ulcerated LS occurred in 24 women with a mean age of 67 years. Ulcerated LS was associated with diabetes and nontreatment at time of biopsy. Clinicians identified red patches in all but 1 case of erosive LS. Ulcerated LS was documented as fissure, ulcer, or white plaque, with 8 (53%) described as lichenified LS with epidermal breaches. Erosive LS favored hairless skin with normal adjacent stratum corneum sloping gently into erosion, whereas most ulcers in LS had an abrupt slope from hair-bearing skin. All cases were treated with topical steroids; 2 patients with erosive LS and 10 with ulcerated LS also had oral antifungals, topical estrogen, antibiotics, and/or lesional excision. Treatment yielded complete resolution in 50%. Conclusions Erosive LS is an unusual clinicopathologic subtype characterized by red patches on hairless skin seen microscopically as eroded epithelium overlying a band of hyalinized or fibrotic collagen. In contrast, ulcerated LS is usually a traumatic secondary effect in an uncontrolled dermatosis.

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.

Lichen sclerosus (LS) is defined as a chronic mucocutaneous inflammatory disease with a localization predominantly to the anus and genitals (vulvar sclerosus (VLS)). Pediatric lichen sclerosus (LS) is a chronic inflammatory skin condition with predilection for the anogenital area that if untreated can lead to scarring. Vulvar LS is characterized by two peaks in incidence: it occurs in prepubertal girls and in postmenopausal women. To date, several mechanisms and risk factors have been proposed in the pathogenesis of pediatric vulvar LS; however, the etiology of this condition is still not fully understood and constitutes a challenge for scientists and clinicians. The presented research aimed to systematically review the existing literature on the pathogenesis of pediatric LS and to identify possible underlying autoimmune mechanisms and molecular networks. The clinical presentation of pediatric lichen sclerosus and available treatment modalities are also presented to acquaint a broader audience with this underdiagnosed and undertreated condition. As a result of our review, we discuss several potential mechanisms, molecules, and pathways that have been recognized in this disease. The purpose of our review was also to summarize what we can induce in further studies, which will ultimately help to identify the mechanism responsible for the disease and aid in the development of new, more effective treatment strategies for diagnosis and treatment by clinicians and researchers.

Linked Article: Arnold et al. Br J Dermatol 2022; 187:909–918.

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.

Lichen sclerosus (LS) can affect sexuality and quality of life (QoL).The aim of the study was to evaluate the impact of psychosexual counseling in women with LS.One hundred fifty-eight women 18 years or older, newly diagnosed with LS, and referred to North Denmark Regional Hospital from January 2018 to November 2019 were included. The women were randomized in a 1:1 ratio to usual care or an intervention group receiving usual care and up to 8 individual consultations with a specialist in sexual counseling. Spouses or partners were encouraged to participate. The women filled out the questionnaires Female Sexual Function Index (FSFI), Dermatology Life Quality Index, and the WHO-5 Well-Being Index at baseline and after 6 months.The controls presented a mean score of 14.8 ± 8.7 and the intervention group presented a mean score of 12.8 ± 8.9 at FSFI. At follow-up, the controls had an FSFI score of 15.2 ± 9.2 and the intervention group revealed an FSFI score of 18.3 ± 9.5. Both groups experienced improved sexual functioning and for the intervention group the increase was significant ( p.001).At baseline, the Dermatology Life Quality Index mean score was 8.9 ± 5.6 for the control group and 9.3 ± 6.1 for the intervention group. At follow-up, the controls revealed a score of 8.6 ± 5.5 and the intervention group a score of 6.8 ± 5.8. The intervention group reached a significantly higher degree of QoL than the controls ( p = .008).Psychosexual counseling has a significant impact on sexual functioning and QoL in women with LS.Lichen sclerosus (LS) can affect sexuality and quality of life (QoL).The aim of the study was to evaluate the impact of psychosexual counseling in women with LS.One hundred fifty-eight women 18 years or older, newly diagnosed with LS, and referred to North Denmark Regional Hospital from January 2018 to November 2019 were included. The women were randomized in a 1:1 ratio to usual care or an intervention group receiving usual care and up to 8 individual consultations with a specialist in sexual counseling. Spouses or partners were encouraged to participate. The women filled out the questionnaires Female Sexual Function Index (FSFI), Dermatology Life Quality Index, and the WHO-5 Well-Being Index at baseline and after 6 months.The controls presented a mean score of 14.8 ± 8.7 and the intervention group presented a mean score of 12.8 ± 8.9 at FSFI. At follow-up, the controls had an FSFI score of 15.2 ± 9.2 and the intervention group revealed an FSFI score of 18.3 ± 9.5. Both groups experienced improved sexual functioning and for the intervention group the increase was significant ( p.001).At baseline, the Dermatology Life Quality Index mean score was 8.9 ± 5.6 for the control group and 9.3 ± 6.1 for the intervention group. At follow-up, the controls revealed a score of 8.6 ± 5.5 and the intervention group a score of 6.8 ± 5.8. The intervention group reached a significantly higher degree of QoL than the controls ( p = .008).Psychosexual counseling has a significant impact on sexual functioning and QoL in women with LS.

Vulval Lichen Sclerosus (VLS) is a chronic inflammatory dermatosis that significantly impacts the quality of life. We report 2 cases of severe treatment-resistant VLS treated with subcutaneous adalimumab. One patient showed modest clinical improvement with no significant improvement in quality-of-life measures. The second patient showed a significant improvement in both clinical and quality-of-life measures. Both patients achieved optimal control with a maintenance dosing of 40 mg subcutaneously weekly. Adalimumab may be a treatment option for recalcitrant VLS; however, clinical trials are required to confirm efficacy for this indication.

To compare the quality of life in patients with vulval lichen sclerosus (VLS), vulval lichen planus (VLP) and chronic vulvovaginal candidiasis (CVVC), as measured by the Vulval Quality of Life Index (VQLI).A retrospective, single-centre cohort study was conducted at a combined dermatology and gynaecology practice from March 2018 to November 2021. VQLI scores and patient data were systematically collected and recorded in an online patient database. Treatment regimens were individualised and titrated to clinical response.Over 3 years, a total of 200 women were recruited: 59 with CVVC, 79 with VLP and 62 with VLS. The median duration of follow-up for all patients was 45.43 (16.25-80.89) weeks. At baseline, the median (interquartile range [IQR]) VQLI score was 24.00 (19.00-31.00), 21.00 (12.00-26.00) and 14.00 (7.00-26.00) for CVVC, VLP and VLS, respectively. At follow-up, the median (IQR) VQLI score for CVVC, VLP and VLS was 9.00 (3.00-15.00), 9.00 (3.00-16.00) and 5.00 (2.00-10.00), respectively. All three groups showed a significant improvement in VQLI score (p 0.0001). At baseline, the highest scoring domains were 'Sexual Function' for CVVC and 'Future Health Concerns' for VLP and VLS. At follow-up, the highest scoring domains were 'Sexual Function' for CVVC and VLP, and 'Future Health Concerns' for VLS.Vulval disease has an immense impact on QOL, especially in patients with CVVC. The VQLI is useful to clinicians in identifying the unique impact of each vulval condition on a patient's QOL in order to provide better patient-focussed care.

Folliculosebaceous units (FSU) has been considered an early target of inflammation in vulvar lichen sclerosus (VLS). This diagnostic clue is not reported in lichen sclerosus (LS) of the foreskin (FLS) that is normally hairless. We evaluated the presence and inflammation of FSU and sebaceous glands (SG) in LS of the foreskin.Histological specimens from therapeutic circumcision were assessed in order to evaluate the frequency and inflammation of FSU and SG in LS.Ninety-eight cases, grouped into 46 early (group 1) and 52 overt (group 2) FLS were included in the study. SG-FSU were found in 95.7% of group 1, and 65.4% of group 2 cases. Their density was inversely correlated with patient age (P=0.0014). We observed perifollicular inflammation in all cases with visible SG-FSU and frequent FSU abnormalities.SG and FSU were frequent in early FLS and decreased in advanced disease and adults. We hypothesize that SG and FSU are involved in the inflammatory process leading to FLS. These data, which need further investigation, could help to better understand the pathogenesis of FLS.

Topical pharmacotherapy is often practiced in the treatment of adults with phimosis or Lichen Sclerosus (LS). However, it is unclear if it is effective.The authors review pharmacological treatments in adults with phimosis or LS using the Preferred Reporting of Items for Systematic Reviews and Meta-analysis (PRISMA) methodology. They searched seven databases using key words phimosis, drug treatment, medical, treatment, lichen sclerosis, pharmacological treatment, medications, comparison study for all English language articles from 1962 to 2021. Inclusion criteria were patients18 yr., and the use of topical medical treatment with outcome data. The article includes the authors' expert opinion and their perspectives for the future.There is limited research, with significant heterogeneity, which suggests the probable safety of topical steroids with some potential to reduce the signs and symptoms of LS. However, good quality research, with patient reported outcome data, is required to establish its true role and effectiveness.

Lichen sclerosus (LS) is a chronic inflammatory mucocutaneous disease of unknown aetiology. About 85% of total cases of LS are genital cases, while extragenital form is seen in only 15-20% of cases. Extragenital LS (EGLS) can occur simultaneously with genital form; however, in 6% of the cases, only extragenital form has been described. Genetic, autoimmune, infectious, environmental and hormonal factors are implicated in its aetiology. Extragenital LS presents as asymptomatic white opalescent papules, which cluster in plaques and slowly progress over time resulting in parchment-like skin usually involving upper trunk, neck and shoulders. Lesions are frequently accompanied by purpura/haemorrhagic spots. The relationship with morphoea has been a topic of debate. Association with several autoimmune diseases has been observed. Diagnosis is usually based on clinical and dermoscopic examination and further supported by histopathological findings. LS needs to be differentiated from several other dermatological conditions such as discoid lupus erythematosus, vitiligo, mycosis fungoides (hypopigmented variant), lichen planus, graft-versus-host disease and morphoea depending upon the stage of the disease. Generally, extragenital LS is believed to lack carcinogenic potential. However, case reports with possible malignant transformation have been described. In this article, the authors have described a concise review of the extragenital form of LS.

Objective: To review the short-term effects and safety of vulvovaginal fractional microablative CO2 laser therapy on atrophy symptoms using validated questionnaires pre- and posttreatment. Methods: This is a retrospective chart review from January, 2016 to December, 2019 on 139 women with vulvovaginal atrophy symptoms, who completed three treatments about 6 weeks apart. All were >18 years old and nonpregnant. As is the practice in our clinic for all women receiving treatment, they were surveyed prior to the 1st and 3rd treatments with validated questionnaires, Female Sexual Function Index (FSFI) and Vulvovaginal Symptoms Questionnaire (VSQ), as well as a visual analog scale (VAS). Paired t test was completed on the pre- and post FSFI and VAS scores. Pre- and postproportions of the VSQ were evaluated by the Fisher's exact test. Means were presented for each study variable. Multivariable regression analysis was completed on continuous and binomial variables for scores predictors. Results: Mean age was 62 years with a mean follow-up of 13.8 weeks. Concomitant topical estrogen was reported in 53% (n = 74). Breast cancer diagnosis was documented in 27% (n = 38), and lichen sclerosus in 22% (n = 31). All FSFI scores improved (pre: 12.7, post: 19.0, P < 0.001). The VSQ showed 18 of 21 questions significantly improved (P < 0.05). The VAS showed significant improvement in painful intercourse (pre: 6.6, post: 2.4, P < 0.001), and vulvar dryness (pre: 4.6, post: 1.5, P < 0.001). Posttreatments, 17 additional women became sexually active. No major adverse events were reported. Conclusions: Fractional CO2 laser may be effective and safe for the treatment vulvovaginal atrophy. Video Summary:http://links.lww.com/MENO/A700.

The diagnosis of balanitis xerotica obliterans (BXO) in children may be challenging, since clinical examination only could lead to an underestimation of its incidence. The aim of this retrospective and single-centre study is to assess the diagnostic performance of clinical examination, together with clinical history, in identifying BXO. Ninety-seven children underwent circumcision for phimosis from 2015 to 2019. Histology was routinely performed. Cohen’s kappa coefficient, sensitivity, specificity, predictive values, likelihood ratios and accuracy of macroscopic appearance of the foreskin, steroid administration and past medical history were estimated. Forty-eight patients (50%) were affected by BXO; 31 of them (69%) presented with suggestive clinical signs. A strictured or whitish urethral meatus was detected during surgery in nine cases (19%); this was associated to allergic or immune diseases (p = 0.046). Foreskin appearance alone mildly correlated with histology (k = 0.494; p Conclusion: Foreskin appearance together with clinical history could predict BXO with certainty. However, since the absence of a positive medical history could not exclude the diagnosis, foreskin histology is still highly recommended. What is Known:• Occurrence of balanitis xerotica obliterans may be underestimated in children and it could lead to long-term complications.• The diagnostic accuracy of clinical examination is controversial. What is New:• Clinical signs together with patients’ medical history present high specificity and positive predictive values but low sensitivity.• When suggestive clinical aspects are present, patient should be referred to surgery avoiding prolonged conservative treatment; and preputial histology is highly recommended.

Lichen sclerosus (LS) may cause the glans and prepuce to become fused, making a standard circumcision impossible. Most authorities recommend excision of the fused area with glans resurfacing, although partial circumcision is often performed.To evaluate an alternative technique that preserves the fused area and allows a complete circumcision without grafting.Over 3 yr (January 2016-March 2018), 28 men (age 28-93 yr; mean 62 yr) underwent the restoration of lost obscured coronal sulcus (ROLOCS) procedure with over 1 yr of follow-up. Complications were reviewed retrospectively with an additional survey.The shaft skin is incised at the corona. Dartos is divided, which allows antegrade dissection just outside the fused glans membrane. The foreskin is removed and shaft skin sutured to dartos below the corona.Postoperative pain, aesthetic satisfaction, sexual enjoyment, glans sensation, and urinary symptoms were measured.There were no major complications. In all cases, the coronal sulcus was restored and the glans skin became soft without skin grafting. All were satisfied with the aesthetics. Of the patients,70% experienced mild to low-moderate pain; 55% and 25% had, respectively, improved or reduced glans sensation; and 40% reported improved enjoyment of sex. Histology showed LS in all cases with squamous cell carcinoma in four, including three out of five patients who had previously undergone partial circumcision. Although this is the largest series reported yet, the numbers were too small for a meaningful statistical analysis.The ROLOCS operation offers an aesthetically superior alternative to partial circumcision and is easier to perform with less morbidity than skin grafting.The restoration of lost obscured coronal sulcus (ROLOCS) procedure provides an alternative to partial circumcision or circumcision with skin grafting when the foreskin is welded to the head of the penis (glans) due to lichen sclerosus. It produces a good cosmetic result, but the glans can be sore until it heals.

Background Lichen sclerosus (LS) is an acquired inflammatory mucocutaneous disease affecting the anogenital area, characterized histologically by hyalinosis and thickened vessel walls in the dermis. The presence of serum autoantibodies against extracellular matrix protein 1 (ECM1) in LS patients may suggest its involvement in disease pathogenesis. Objective To examine if reduced ECM1 production by dermal fibroblasts contributes to the pathogenic features of LS. Methods Gene expression in ECM1 knockdown human dermal fibroblasts was analyzed by cDNA microarray. Functional enrichment for genes involved in cellular functions was conducted. Protein expression was analyzed by ELISA and confocal laser scanning microscopy using LS skin. Results Microarray analysis identified 3,035 differentially expressed genes in ECM1 knockdown cells, wherein 1,471 were upregulated genes related exclusively to cell adhesion, proliferation, apoptosis, intracellular signaling, and extracellular matrix organization. Further narrowing with criteria specific for localization and function of ECM1 identified 48 upregulated genes identified to have structural, fibrogenic, and carcinogenic properties. Of these, laminin-332 and collagen-IV displayed altered immunolabeling within the basement membrane zone (BMZ) and dermal vessels in LS skin, similar to that of collagen-VII, which exhibited unchanged transcription levels in ECM1-knockdown fibroblasts. Collagen-VII bound to recombinant ECM1 in a solid-phase immunoassay and colocalized with ECM1 in the skin BMZ. Further, ECM1-knockdown fibroblasts exhibited a marked delay in cell migration and gel contraction. Conclusion In the absence of ECM1 expression in fibroblasts there is selective dysregulation and disassembly of structural and extracellular matrix molecules, which may result in microstructural abnormalities reminiscent of LS.

Background Differentiated vulvar intraepithelial neoplasia (dVIN), the precursor lesion to human papillomavirus (HPV)-independent vulvar squamous cell carcinoma (VSCC), can be difficult to distinguish from vulvar inflammatory dermatoses. p53 has been suggested to be a useful biomarker for dVIN, but the percentage, intensity and patterns of staining have not been well characterized in dVIN and its histologic mimics. Method We studied p53 immunohistochemical staining patterns in 16 dVIN cases and 46 vulvar non-neoplastic squamous lesions (12 lichen sclerosus (LS); 7 lichen simplex chronicus (LSC); 3 lichen planus (LP); 6 psoriasis (PSO); 13 spongiotic dermatitis (SPO); 5 candidiasis (CAN)). dVIN cases were adjacent to a p16-negative invasive VSCC in resection specimens. Results All dVIN cases demonstrated null-type or moderate-strong uniform p53 staining in over 70% of basal cells, with moderate to strong continuous parabasal staining extending to two-thirds of the epidermis. This was in contrast to weak or weak-moderate patchy p53 staining in the majority of other lesions. Moderate-strong and increased basal p53 staining (≥70%) was also observed in a subset of lichen sclerosus (5/12, 42%), lichen planus (1/3, 33%) and spongiotic dermatitis (36%, 4/11), however in all categories, this was limited to the basal layer and any staining in the parabasal layers was patchy. Conclusion Strong and uniform p53 staining of basal cells, extending into the parabasal layers, and a complete absence of staining (null-type) is useful in distinguishing dVIN from other mimics in the vulva. p53 staining of lesser intensity or quantity, particularly basal-overexpression only, overlaps with vulvar inflammatory lesions.

Supplemental digital content is available in the text., Objective 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). Methods 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. Results 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. Conclusions 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.

Morphea and lichen sclerosis et atrophicus (LSA) are two distinct immune-mediated diseases with a dominant presentation of dermal fibrosis and sclerosis. The two diseases have many similar clinical and histological features and tend to co-occur. Both diseases are thought to result from a derailment of the normal response to environmental triggers. Positive family history is more common in LSA than morphea but individuals with morphea have a higher frequency of concomitant and familial autoimmunity. These findings hint at the involvement of inheritance in susceptibility to LSA and morphea and thus provide a rationale for exploring the disease genetics. This chapter contains a comprehensive review of the pathogenesis of the two diseases and their known genetic associations including HLA class I and II genes.

In recent years, LASER has been introduced as a minimally invasive treatment for a broad range of vaginal and vulvar symptoms and diseases. However, the efficacy and safety of vaginal and vulvar LASER has continuously been questioned. The aim of this study is to create an overview of the current literature and discuss the controversies within the use of LASER for genitourinary syndrome of menopause, vulvovaginal atrophy, urinary incontinence and lichen sclerosus. A search string was built in PubMed. The search was commenced on August 25, 2021 and closed on October 27, 2021. Two authors screened the studies in Covidence for inclusion according to the eligibility criteria in the protocol. The data were extracted from the studies and are reported in both text and tables. This review included 114 papers, of which 15 were randomized controlled trials (RCTs). The effect of LASER as a vaginal treatment was investigated for genitourinary syndrome of menopause in 36 studies (six RCTs), vulvovaginal atrophy in 34 studies (four RCTs) and urinary incontinence in 30 studies (two RCTs). Ten studies (three RCTs) investigated the effect of vulvar treatment for lichen sclerosus. Half of the included RCTs, irrespective of indication, did not find a significant difference in improvement in women treated with vaginal CO2 or Er:YAG LASER compared with their respective controls. However, most non-comparative studies reported significant improvement after exposure to vaginal or vulvar LASER across all indications. Included studies generally had a short follow-up period and only a single RCT followed their participants for more than 6 months post treatment. Adverse events were reported as mild and transient and 99 studies including 51 094 patients provided information of no serious adverse events. In conclusion, this review found that the effect of vaginal and vulvar LASER decreases with higher study quality where potential biases have been eliminated. We therefore stress that all patients who are treated with vaginal or vulvar LASER should be carefully monitored and that LASER for those indications as a treatment should be kept on a research level until further high-quality evidence is available.

Background: Studies concerning pediatric lichen sclerosus are limited, and, to date, there have been no studies comparing the course of lichen sclerosus in boys and girls. We sought to examine all publications on boys and girls with lichen sclerosus and assess and compare epidemiology, symptoms and signs, genetic background, risk factors, treatment, and prognosis. Methods: A systematic search was performed in the Embase, Medline, Cochrane, and Web of Science databases. Inclusion criteria were information on children ages 0–18 years and a clinical or histologic diagnosis of lichen sclerosus. Literature from 1985 to 2021 was reviewed. Results: A total of 1780 articles were retrieved from the search, of which 90 articles were eligible for inclusion. Boys and girls present similarly on many aspects; nonetheless, treatment and follow-up are approached differently. Conclusions: Though the clinical approach is often different, lichen sclerosus in boys and girls demonstrates many similarities. More research is needed, especially on follow-up, to gain a better understanding of the course of lichen sclerosus and establish an advanced management plan for children.

Male genital lichen sclerosus (MGLSc) is a chronic inflammatory dermatosis that predominantly affects uncircumcised males. Several aetiological factors have been described, with growing evidence supporting chronic urine exposure of susceptible epithelium being crucial to its pathogenesis. We describe a 45-year-old man with a 2-year history of MGLSc at the site of a urethrocutaneous fistula secondary to a penile piercing. To our knowledge, this is the first documented case of MGLSc occurring at the site of a genital piercing.

Balanitis Xerotica Obliterans is a chronic, progressive, sclerosing inflammation of unclear etiology. It involves the external genitalia of males and more specifically the prepuce and its frenulum, the glans, and the external urethral meatus while it may extend to the peripheral part of the urethra. Recent studies have noted an increasing incidence in the paediatric population. It is the most common cause of secondary (pathologic) phimosis. Even more, in boys with physiologic phimosis that does not respond to conservative treatment, Balanitis Xerotica Obliterans should be considered as the underlying condition. In this study, we present all the latest data and attempt to create a diagnostic and curative algorithm regarding this condition.