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Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.

Vulvar lichen sclerosus is an important skin disease that is common in women in their 50 s and beyond; however, it can also affect females of any age, including children. If not treated, it has the potential to cause significant and permanent scarring and deformity of the vulvar structure. In addition, if untreated, it is associated with a 2–6% lifetime risk of malignant squamous neoplasia of the vulva. Lichen sclerosus has been considered a difficult to manage condition; however, both serious complications can potentially be prevented with early intervention with topical corticosteroid, suggesting that the course of the disease can be treatment modified.

Lichen sclerosus is a chronic skin disease, mainly localised at the introitus and perineum. When the condition remains untreated, gradual atrophy of skin structures leads to permanent scarring, making early diagnosis and treatment crucial. We reviewed all patients diagnosed with lichen sclerosus presenting to a tertiary referral centre for paediatric and adolescent gynaecology between January 2011 and December 2015 to assess disease presentation and response to treatment. We identified 15 cases, with a mean age at diagnosis of 8.8 years. Their main presenting symptoms were vulvar pruritus and vulvar soreness. Seven girls had already atrophic changes, and in four girls, this amounted to clitoral phimosis, labial resorption or labial adhesion formation. The median delay in diagnosis was 7 months. Thirteen patients received local treatment with potent corticosteroids, responding well to treatment. However, 4 girls relapsed within 2 to 36 months. Two adolescents required surgical treatment, one because of urinary retention and the second because of dyspareunia caused by clitoral entrapment. Conclusions : There was a delay in diagnosis in most patients and this resulted in irreversible genital skin changes, which would have been preventable, had treatment been instituted promptly. The response to treatment with local corticosteroids was usually effective, leading to both symptom alleviation and prevention of disease progression. Atrophic changes and skin complications however were not reversed. What is Known: • Lichen sclerosus affects women of all ages, including girls, particularly prior to adolescence. • Lichen sclerosus responds well to local corticosteroid treatment . What is New: • In the majority of patients with lichen sclerosus there was a long delay between onset of symptoms and diagnosis . • Nearly half of the children diagnosed with lichen sclerosus had irreversible atrophic genital skin changes at the time of first presentation. These changes may have been prevented by a timely diagnosis and intervention .

Vulvar lichen sclerosus is a chronic, progressive inflammatory dermatosis that predominantly affects postmenopausal women but can also present in premenopausal individuals. It can cause significant scarring, leading to anatomical distortion, urinary dysfunction and impaired quality of life. The author describes a case of a woman in her late 30s with extensive vulvar involvement of lichen sclerosus, resulting in introital stenosis, dyspareunia and painful micturition. The patient was managed with a combination of topical steroids and systemic immunosuppressants, followed by surgical intervention using a Singapore flap for vaginal reconstruction. This case highlights the importance of early diagnosis, individualised treatment approaches and long-term follow-up in managing severe vulvar lichen sclerosus.

ObjectiveTo explore experience and prevalence of vulval lichen sclerosus (VLS) diagnosis in general practice using an anonymous patient survey.DesignQuantitative descriptive cross-sectional survey informed by previous qualitative interviews and developed with patient representatives, sent to people recorded in general practice as having a VLS diagnosis.SettingGeneral practices (n=24) in the UK (West Midlands).Participantsn=177 respondents.ResultsOne in five respondents reported that they had been misdiagnosed, and about a third reported that it was a struggle to get treatment. Only one third said they received regular check-ups, recommended in clinical guidelines. One-fifth reported they were not being treated with topical corticosteroids, the main first-line treatment for VLS. Less than one in 10 were members of a support group, and around four in 10 felt they had to hide their condition and did not speak to anyone else about it. Survey respondents prioritised improving education and awareness among healthcare professionals (HCPs).ConclusionGeneral practitioners and other primary care HCPs have a key role in recognising, diagnosing and managing VLS. Improving education and awareness among HCPs was a key priority for this patient group. Patients should be made aware of the need for ongoing treatment and yearly check-ups to prevent or manage disease progression. VLS is a highly stigmatised condition, and appointments with HCPs may be the only opportunity for people to talk about their experience.

Demographics and clinical characteristics Characteristics Sampled patients with L90.0 code (N = 245) Age at first diagnosis  Mean (SD) 62 (14)  Median [Q1, Q3] 63 [55, 71] Race  African American/Black 14 (6.2%)  Asian 8 (3.5%)  Caucasian/White 186 (82%)  Other/Multiracial 19 (8.4%)  Missing 18 (7.3%) Body Mass Index  Mean (SD) 29 (6.1)  Median [Q1, Q3] 28 [25, 32]  Missing 13 (5.3%) Specialty of first diagnosing physician  OBGYN 141 (58%) Dermatology 36 (15%)  Primary care 38 (16%)  Other 30 (12%) Biopsy available  Yes 74 (30%)  No 171 (70%) Biopsy confirmed lichen sclerosus  Yes 63 (85%)  No 11 (15%) Symptoms  Itch 95 (39%)  Hypopigmentation 74 (30%)  Thinning 70 (29%)  Burn 22 (9.0%)  Pain 25 (10%)  Dyspareunia 24 (9.9%)  Dysuria 15 (6.1%)  Anatomic changes 41 (17%)  Plaques 17 (7.0%)  Lichenification 12 (4.9%)  Fissuring 4 (1.6%)  Follicular plugging 0 (0%) Medical history  Autoimmune comorbidity 70 (29%)  Fungal infection 17 (7.0%)  Bacterial infection 17 (7.0%)  Gynecologic malignancy 16 (6.6%)  HPV 11 (4.5%)  Viral infection 4 (1.6%) Abbreviations OBGYN, obstetrics-gynecology; HPV, human papilloma virus Table 2. Positive predictive values of diagnosis code case definitions for lichen sclerosus of the vulva from electronic medical records Case definition Number of sampled patients satisfying case definition Reference definition: chart note diagnosis by treating physician, or biopsy confirmation Number meeting reference definition for lichen sclerosus Positive predictive value, % (95% CI)a At least 1 diagnosis, any provider 245 200 85.2 (80.3, 90.0) By Specialty, First diagnosis  OB/GYN 141 122 86.5 (81.0, 92.0)  Dermatology 36 33 91.7 (81.1, 97.5)  Primary Care 38 24 63.2 (51.0, 75.4)  Other 30 21 70.0 (55.4, 84.6) By Specialty, Any diagnosis  OB/GYN 160 140 86.8 (81.4, 92.1)  Dermatology 40 37 94.1 (86.2, 98.3)  Primary Care 46 32 78.3 (68.1, 88.6)  Other 32 23 74.0 (60.1, 87.9) Other case definitions  At least 1 by non-  dermatologist and non-  OB/GYN 51 29 56.9 (44.9, 68.9)  At least 2 by non-  dermatologist and non-  OB/GYN 10 7 71.0 (46.6, 95.4) Abbreviations OB/GYN, obstetrics-gynecology a – The positive predictive values and confidence intervals are estimated for the entire population from which the stratified random sample was drawn, and account for the unequal probabilities of being sampled across strata To our knowledge, PPVs of case finding algorithms for LS of the vulva have not been established. Limitations of this analysis include relatively low sample size for specialties other than OB/GYN, variation in provider documentation practices, and restriction of data to a single healthcare system.

IntroductionPaediatric vulval lichen sclerosus (VLS) is a chronic disease with distressing symptoms and severe consequences when left untreated. Majority of existing data on pathophysiology and treatment is based on studies conducted among adult patients. Whereas the course of VLS, its symptomatology and prognosis are distinct to some extent in paediatric and adolescent patients as compared with adults. The purpose of this scoping review is to systematically examine what symptoms of VLS are typical of paediatric and adolescent patients, how often specific signs and symptoms are reported in the literature, if there are differences between paediatric and adolescent patients and what could be the implication of such differences.Methods and analysisThis scoping review will adopt the methodology for Joanna Briggs Institute scoping reviews and will consider studies that include female patients aged 1–18, with VLS symptoms and signs with no exclusion based on ethnicity, comorbidity or previous history of treatment. Studies on any aspect of paediatric VLS, including pathogenesis, diagnosis and treatment, which included patients and reported patients’ symptoms and signs, will be considered eligible. There will be no geographical or cultural limitation applied in relation to this scoping review. The search will include Embase, Academic Search Premier, CINAHL, Cochrane Library, Google Scholar, Health Source, Ovid Embase, Ovid Medline, PubMed, Scopus and Web of Science Principal Collection. A critical synthesis and results will be presented in the final review as tables and accompanying narrative summary.Ethics and disseminationEthical approval is not required for this review. To date, no systematic approaches were undertaken to classify symptoms of the VLS that would aid in formulating disease severity criteria adequate for the paediatric population. We believe that the results of this review will facilitate the development of disease severity scales that could aid in intraindividual and interindividual comparability, both in real-life settings and clinical trials.Trial registration numberhttps://doi.org/10.17605/OSF.IO/FB9EG

Differentiated vulvar intraepithelial neoplasia (dVIN) is the precursor lesion of HPV-negative vulvar squamous cell carcinoma (VSCC). The histopathological diagnosis of dVIN can be challenging, as it often resembles vulvar non-neoplastic epithelial disorders (NNED), especially lichen sclerosus (LS). We aimed to establish the most specific and reproducible histological features of dVIN and assessed cytokeratin 13 (CK13) and cytokeratin 17 (CK17) immunohistochemistry as a diagnostic aid. Consecutive cases of dVIN (n = 180) and LS (n = 105) from the period 2010 to 2013 were reviewed using a checklist of histological features. Each feature was recorded as ‘present’ or ‘absent’ and statistical comparison (dVIN vs LS) was made. Interobserver agreement between two pairs of pathologists was assessed for a subset of cases of dVIN (n = 31) and LS and other NNED (n = 23). Immunohistochemistry with CK13, CK17, MIB1 and p53 was performed on dVIN, LS, and other NNED cases. Macronucleoli, features of disturbed maturation and angulated nuclei were significantly more common in dVIN than LS (p < 0.001). We found ‘substantial agreement’ for the diagnosis of dVIN (κ = 0.71). Macronucleoli and deep keratinisation had the highest agreement. In dVIN, the mean percentage of cells staining with CK13 was 15 and with CK17, this was 74. For LS, the mean percentage of cells staining with CK13 was 31, and with CK17, this was 41. By plotting receiver operating characteristic curves (ROC), an area under the curve (AUC) of 0.52 was obtained for CK13, and an AUC of 0.87 was obtained for CK17. The most helpful histological features for diagnosing dVIN were macronucleoli, features of disturbed maturation, and angulated nuclei. Increased CK17 expression may have promise for supporting dVIN diagnosis.

The classic clinical features of vulvar lichen sclerosus are shiny white atrophic plaques in a \"figure of 8\" pattern that encircle the vulva, perineum and perianal areas, although more focal areas can be affected. Advanced disease can result in severe scarring, leading to deformed anatomy and stenosis. Other findings may include petechia, ecchymosis, erosions, fissures, hypertrophic plaques and hyperpigmentation. Fifteen percent of patients will also have extragenital involvement. Vulvar lichen sclerosus is rarely asymptomatic. More than 90% of patients will present with severe pruritus, but they may also have dysuria, dyspareunia and clitoral hyperesthesia. Differential diagnoses include lichen planus, dermatitis or lichen simplex chronicus. Here, Cleminson and Baxter examine the case of a 71-year-old woman with vulvar lichen sclerosus.

This case study presents the clinical journey of an elderly woman in her late 70s seeking medical attention for vulvar discomfort. Upon observing a red area on the vulvar region, initial management involved the use of clobetasol due to suspicion of lichen sclerosus, a condition linked to vulvar discomfort and erythematous patches.After 1 year, the patient reported symptom improvement, but a persistent red area on the labia minora prompted a biopsy. Surprisingly, the biopsy revealed Zoon’s Vulvitis, a diagnosis not anticipated due to the unusual clinical presentation. This case underscores the significance of considering rare conditions, such as Zoon’s Vulvitis, even in vulvar abnormalities with atypical features. Further research is warranted to enhance our understanding of the varied clinical presentations of Zoon’s Vulvitis.