Lichen Sclerosus Research Articles

The Treatment of Lichen Sclerosus with Topical Roflumilast 0.3% Case Study

Lichen Sclerosus is a chronic inflammatory skin disease predominantly affecting post-menopausal women. Currently, the first line treatment approach for lichen sclerosus is ultra potent topical corticosteroids for 12 weeks. A treatment challenge to this approach is the risk of steroid atrophy due to the use of potent topical steroids on the genital skin. We describe reduction of erythema and symptomatic improvement following treatment with topical roflumilast 0.3% once daily in a patient with lichen sclerosus who refused topical corticosteroids.

Surgical treatment of hypospadias with lichen sclerosus: Long‐term outcomes from a single center

AbstractBackgroundHypospadias with lichen sclerosus (LS) poses surgical challenges due to lack of materials for urethral reconstruction and coverage and the limited number of reports on this clinical condition.ObjectivesTo report surgical strategies and outcomes for primary and redo hypospadias patients with LS.Materials and methodsWe conducted a retrospective observational descriptive study with 31 patients with primary/redo hypospadias and LS between 2013 and 2023. Of these patients, 9 had primary hypospadias with 7 distal types and 2 proximal types, and 22 were redo patients. Nineteen of the 31 patients underwent various surgical techniques, and 12 underwent the oral mucosa onlay procedure (OMOP). Postoperative uroflowmetry, hypospadias objective scoring evaluation (HOSE), and complications were evaluated.ResultsFor primary distal hypospadias with LS‐affected skin, five patients underwent circumcision; these patients had normal postoperative urinary flow, and only one patient had an inconspicuous penis after surgery. For the patients with primary proximal hypospadias with an LS‐affected urethra, one patient required perineal urethrostomy following a modified Koyanagi procedure for recurrent stricture; the other patient underwent two‐stage transverse preputial island flap urethroplasty with no postoperative lower urinary tract symptoms (LUTS) and satisfactory aesthetic scores. In two remaining cases of primary distal hypospadias with meatus/urethral LS, no LUTS or postoperative complications occurred following meatoplasty/circumcision and penile basal fixation. During reoperations, compared with those of patients who underwent other redo procedures, twelve patients who underwent OMOP had better Qmax and uroflowmetry (p = 0.002) and higher HOSE scores (p = 0.01).ConclusionsFor primary hypospadias patients with LS, enhanced exposure of the penile shaft and glans may benefit distal hypospadias, whereas staged surgery may be suitable for proximal hypospadias. The oral mucosa is favored for urethral augmentation in patients with redo hypospadias complicated with LS, and the OMOP can address neourethra coverage effectively.

The Influence of Genital Lichen Sclerosus on Sexual Health and Well-being: A Tripartite Comparative Analysis

The Influence of Genital Lichen Sclerosus on Sexual Health and Well-being: A Tripartite Comparative Analysis

Clinical Presentations and Treatment Outcomes in Extragenital Lichen Sclerosus: A 34-Year Multi-Institutional Retrospective Review.

Clinical Presentations and Treatment Outcomes in Extragenital Lichen Sclerosus: A 34-Year Multi-Institutional Retrospective Review.

Barriers to diagnosing and treating vulval lichen sclerosus: a survey study.

Background Vulval lichen sclerosus (VLS) is a chronic inflammatory condition that is frequently misdiagnosed and under-recognised. To date, qualitative research focuses on lived experience of VLS, with women attributing diagnostic delay to poor interactions with health care professionals (HCPs) often due to lack of knowledge. In the UK, women with VLS are most likely to present to primary care. Aim To establish HCPs perspectives on identification, management and education of vulval skin disease, with a focus on VLS. Design and Setting A survey was distributed to HCPs working in primary care. Method The survey was distributed via professional networks and at events. Analysis comprised of descriptive statistics, Spearman's rank correlations, and thematic analysis. Results Of 122 respondents, 53 were General Practitioners (GPs) and 59 were GP trainees. 37.7% of respondents had never participated in teaching nor learning on vulval skin disease. Confidence in the identification of vulval skin disease positively correlated with experience, exposure and female gender. The top identified barriers to diagnosis and treatment included lack of knowledge, embarrassment, and absence of VLS diagnostic criteria. Almost all participants (97.5%) felt VLS diagnostic criteria would be helpful in clinical practice. Conclusion This study provides insight into the barriers to diagnosing and treating VLS in primary care. HCPs recognise deficiencies in training, referral pathways and lack of tools to support VLS diagnosis. Training should include skills to address stigma and embarrassment. This study highlights the importance of developing interventions to overcome barriers, expediting diagnosis and treatment, such as reproducible diagnostic criteria.

The relationship between the vaginal and vulvar microbiomes and lichen sclerosus symptoms in post-menopausal women

Lichen sclerosus is a chronic inflammatory condition of unknown etiology that affects the genital and extragenital skin, which can lead to sexual dysfunction and has been associated with vulvar cancer. The vaginal microbiome has a critical role in gynecologic health, but little is known about the microbiome in lichen sclerosus. This study investigated the vaginal and vulvar microbiomes of 27 post-menopausal women with lichen sclerosus. The most abundant genera detected in the vaginal microbiome were Lactobacillus, Gardnerella, and Anaerococcus, while Lactobacillus, Anaerococcus, and Staphylococcus were the most abundant in the vulvar microbiome. The vaginal samples clustered into two main groups, Lactobacillus dominant (n = 6, > 50% microbiome Lactobacillus) and polymicrobial (n = 21) with no dominant genus. The vulvar samples were mainly polymicrobial (n = 25). Actinomyces, Anaerococcus, and Ezakiella in the vaginal microbiome and Actinomyces and Ezakiella in the vulvar microbiome were significantly associated with lichen sclerosus symptoms (adjusted p < 0.05). In this population of post-menopausal women with lichen sclerosus the majority have diverse, non-Lactobacillus dominant microbiomes, which is considered less optimal for gynecologic health based on studies of pre-menopausal women. Actinomyces, Ezakiella, and Anaerococcus were associated with lichen sclerosus symptoms. Understanding the role of these bacteria in lichen sclerosus pathogenesis will be an essential future investigation.

Precancerous Lesions of HPV-independent Vulvar Squamous Cell Carcinoma: Clinicopathologic Consideration of an Evolving Spectrum.

HPV-independent squamous cell carcinomas of the vulva comprise the majority of vulvar cancers, but their putative precancers represent only a small proportion of the vulvar squamous intraepithelial lesions that are encountered in routine practice. The precancerous lesions of HPV-independent vulvar squamous cell carcinoma encompass a spectrum of lesions that, collectively, may pose significant diagnostic challenges. Included in this spectrum are differentiated vulvar intraepithelial neoplasia [dVIN], the prototypical lesion of the group, which is characterized by a high propensity for progression, a relatively short duration to progression, frequent association with lichen sclerosus, and according to our review of the recent literature, TP53/p53 aberration in 50% to 95% (mean 77.4%) of cases. Regarding the latter, some authors consider TP53/p53 aberration to be a diagnostic requirement for dVIN, although this is controversial, as discussed further herein. Also included in the spectrum of lesions that are considered in this review are possibly related HPV-independent, p53-wild type lesions that have historically been reported as "vulvar acanthosis with altered differentiation" (VAAD), "differentiated exophytic vulvar intraepithelial lesion" (DEVIL), "verruciform lichen simplex chronicus" (vLSC), and which more recently, have collectively been described as "verruciform acanthotic vulvar intraepithelial neoplasia (vaVIN)" or "vulvar aberrant maturation (VAM)." In this review, we perform a comprehensive clinicopathologic review of putative precancerous lesions of HPV-independent squamous cell carcinomas of the vulva, with an emphasis on recent developments in terminology, practical diagnostic issues, biomarkers, and pathogenesis.

Vulvar lichen sclerosus in girls and adult females: A single-center retrospective study of 744 patients in China.

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

Zoon balanitis: not a distinct clinicopathological entity?

Zoon balanitis (ZB) was originally described in the 1950s in patients with clinical features resembling erythroplasia of Queyrat, but with histology that demonstrated a plasma cell infiltrate without evidence of dysplasia. Subsequently, ZB has been extensively reported in the literature, reflecting widespread acknowledgement as an established distinct clinicopathological entity. However, its existence as such has been questioned and there have been suggestions in the literature that ZB represents either a non-specific irritant reaction pattern, or a part of the heterogenous clinicopathological complex of male genital lichen sclerosus (MGLSc). In this study, the entire historical literature of published cases of ZB was subject to review, to determine respectively whether clinical or histological features of MGLSc had been present and been overlooked. One hundred and one papers were reviewed. Seventy-five contained images purporting to be of ZB. Images that were deemed to be of inadequate quality or to present uninformative anatomical views were excluded. The images in 66 manuscripts were amenable to scrutiny; 98.5% of these images demonstrated clinical signs of MGLSc, such as adhesions, effacement of the coronal sulcus and the frenulum, tightening of the prepuce, constrictive posthitis, etiolation of the glans, lichenoid inflammation and sclerosis. These findings strongly suggest that ZB occurs in the vast majority of cases as a manifestation of MGLSc and that its recognition as a distinct clinicopathological entity should be questioned, if not discontinued.

Management of Lichen Sclerosus and Related Comorbidities at a Tertiary Referral Center: Beyond Topical Steroids

Introduction: Lichen sclerosus (LS) is a chronic inflammatory dermatosis predominantly affecting the anogenital area, with potential systemic implications. Objective: Our aim is to elucidate its clinical characteristics and management strategies. Methods: We conducted a retrospective observational study on adult patients with LS referred to our center between January 2022 and December 2023. Results: Forty-six adult subjects affected by LS were enrolled in the present study. The majority of patients presented with genital involvement, but a significant subset exhibited extragenital LS and concomitant immune-mediated disorders. Common symptoms included itching, soreness, and dyspareunia, with characteristic skin findings of erythema and atrophy. Topical corticosteroids were the mainstay of therapy, complemented by alternative treatments in refractory cases. Alternative therapeutic approaches, such as platelet rich plasma or adipose-tissue derived stromal/stem cell-based strategies, were effectively employed in refractory cases, in the absence of major adverse events. Conclusions: Our findings underscore the importance of alternative approaches in the management of LS and highlight the need for further research to elucidate its pathogenesis and optimize therapeutic interventions.

Associations vulvar lichen sclerosus with autoimmune thyroid diseases.

Vulvar lichen sclerosus (VLS) is defined as a chronic inflammatory skin disease that most often involves lesions on the mucous membranes of the vulva with a tendency to progress to the anal skin. The etiopathogenesis of VLS remains unknown and is likely multifactorial. Data emphasize the role of immunological factors - more than 25% of VLS cases coexist with autoimmune diseases. The purpose of the present study was to determine the correlation of the prevalence of anti-thyroid antibodies - IgG class antibodies against thyroid peroxidase and IgG class antibodies against thyroglobulin in women with vulvar lichen sclerosus, and the appropriateness of screening tests for autoimmune thyroid diseases in women with vulvar lichen sclerosus. Fifty women with vulvar lichen sclerosus were enrolled in the study. The control group consisted of 41 healthy women. A detailed medical history was taken with all patients, followed by laboratory determinations - anti-thyroid antibodies - IgG class antibodies against thyroid peroxidase and IgG class antibodies against thyroglobulin. Antibodies to thyroid peroxidase were present in 12% of the study group with vulvar lichen sclerosus and 4.88% of the control group, and this difference was not statistically significant (p = 0.41). Anti-thyroglobulin antibodies were detected in 4% of the patients with vulvar lichen sclerosus and 4.88% of the control group, and this difference was not statistically significant either (p = 0.76). The study did not confirm the association of VLS with autoimmune thyroid diseases. Undoubtedly, based on the data available in the literature, further studies are needed to determine the mechanisms behind the association between vulvar lichen sclerosus and autoimmune thyroid diseases.

Association between vulvar lichen sclerosus and celiac disease in woman.

Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition involving mainly the genital area with an undetermined aetiology. Recent studies show that in up to 34% of cases in adult women, VLS coexists with allergies or autoimmune diseases like celiac disease (CD), among others. However, literature data relating strictly to the co-occurrence of celiac disease and Duhring's disease (DH) in patients with VLS are very limited. In our study, we sought to clarify the possible relationship between vulvar lichen sclerosus in adult women and celiac disease in its cutaneous form. The aim of the study was to demonstrate the presence of celiac disease-specific antibodies in women with VLS. The control group consisted of 41 healthy women, and the study group consisted of 50 women aged 24-83 years with diagnosed vulvar lichen sclerosus who were hospitalized in the Department of Gynaecology, Obstetrics and Gynaecologic Oncology of the Bonifraters Medical Centre in Katowice. There were no significant differences in blood serum levels of CD-specific antibodies between both groups. The study conducted did not confirm the association between vulvar lichen sclerosus and celiac disease or Duhring's disease. The main limitation of the research was the small size of the study and control groups. Further studies on a larger group of patients are needed. They could clarify the possible mechanisms behind the co-occurrence of these two conditions. Earlier diagnostic will help prevent the development of severe and irreversible complications.

Tiktok as a Source of Education and Misinformation in Lichen Sclerosus.

The purpose of this study is to evaluate the content, delivery, and quality of medical information for vulvar lichen sclerosus on the social media platform TikTok. This is a descriptive, cross-sectional study. Using the third-party data scraping tool Apify, TikTok videos tagged with #lichensclerosus or "lichen sclerosus" were identified and sorted by view count. A sample of 100 videos was reviewed by 2 independent reviewers, excluding those not discussing lichen sclerosus. Videos were assessed using a coding document, the Patient Educational Materials Assessment Tool, and the DISCERN instrument. Interrater reliability was measured, and statistical analyses included Fleiss' kappa, intraclass correlation coefficient, t tests, and Wilcoxon rank sum test with Holm-Bonferroni correction. Content creators included patients (46%), health care professionals (30%), and others. Topics focused on clinical disease (52%) and treatment (48%). Evidence-based medicine was discussed in 71.7% of treatment-related videos, while 51.7% included nonevidence-based approaches, with a neutral or positive sentiment. Videos discussing topical steroids often had negative sentiments. Quality assessment revealed 61% of videos were understandable, 27% actionable, and 46% contained misinformation. Videos by health care professionals had less misinformation and higher quality scores compared to patient-generated content. Commercially biased videos were more understandable but contained more misinformation. TikTok serves as a significant platform for sharing information on lichen sclerosus, but nearly half of the content contains misinformation. Health care professionals need to engage in social media to provide accurate information and counteract misinformation. Enhanced collaboration with patient advocates and careful resource sharing can improve the quality and reliability of medical information available online.

A cross‐sectional study of the vulval dermatology clinic population over a 4‐year period and development of a national vulval disease database

AbstractBackgroundVulval disease significantly impacts quality of life but is historically under‐researched. The epidemiology and aetiology of many vulval conditions is unclear. Data to optimise patient care are lacking.ObjectivesTo describe the population attending a specialist vulval dermatology clinic and achieve consensus amongst vulval experts on data items to be collected for a future national vulval database.MethodsThis descriptive cross‐sectional study analysed data that was prospectively collected during clinical contact with consecutive new patients at a vulval dermatology clinic over 4 years.A two‐stage electronic‐Delphi survey was performed with British vulval experts. Consensus was defined as ≥75% agreement on items for inclusion.ResultsThe database included 424 (including 29 paediatric) patients. Most patients were White British (71%), with a significant Asian population (13%). Long symptom duration (9.5% &gt; 10 years) and multiple diagnoses, up to 4, were common. Exploratory associations were identified between irritant contact dermatitis and urinary and faecal incontinence, frequent vulval washing and lichen simplex, urinary incontinence and lichen sclerosus and a negative association between candidiasis and age.Following two rounds of the electronic‐Delphi survey, consensus was achieved for 18 items that 28 participants agreed were important for a future database.ConclusionsWe report disease incidence, patient pathways, outcome measures and potential associations. Though not generalisable, this large UK‐based study could inform future projects to improve patient care and support ongoing research, such as a national vulval disease database, for which we also achieve expert consensus on the most valuable items to include.

Lack of oral involvement in a large cohort of women with vulvar lichen sclerosus - a multicenter prospective study.

We assessed the prevalence of oral lichen sclerosus in a cohort of women affected with vulvar lichen sclerosus (VLS). This prospective, observational study included adult female patients with a histologically proven VLS who attended the Vulva Clinics of three Dermatology Units from January 2020 to July 2023. During this period, all VLS patients were asked to be examined in their oral cavities in order to detect any possible sign of oral diseases, which were then biopsied. Three hundred women (mean age 64.5 ±13.0years) were included, of whom 21 (7%) had a concurrent extragenital LS. In six (2%) patients, white, non-removable lesions were found at oral inspection and biopsied. All cases were histologically lichen planus. No other clinically relevant oral lesions were found. The six women with histologically proven oral lichen planus had more frequent periodontal and autoimmune diseases than the other included subjects. Our findings confirm the absolute rarity of oral involvement in patients with VLS. They suggest that the oral mucosa, unlike the genitals, is a setting which weakly leads to development of LS, also in predisposed subjects.

Treatment Modalities for Genital Lichen Sclerosus: A Systematic Review

Background: Lichen sclerosus (LS) is a chronic, inflammatory dermatosis that affects both genital and extragenital sites. It is often difficult to treat and may lead to a variety of complications if not adequately treated. The mainstay of therapy involves topical corticosteroids, topical calcineurin inhibitors, and systemic immunomodulators. Although a variety of topical, oral, and procedural therapies are available, a review comparing relative efficacy is lacking. To this end, this systematic review aimed to summarize the literature regarding treatment modalities and their respective response rates in patients with genital LS. Methods: A literature search was conducted in accordance with PRISMA guidelines. Results: This review qualitatively summarizes information from 31 randomized controlled trials, encapsulating a total of 1507 patients with LS, the majority of which were female (n = 1374, 91%). Topical corticosteroids, the mainstay of therapy for LS, were discussed throughout the literature, and proved to be more efficient than topical calcineurin inhibitors, topical hormonal therapy, topical vitamin E oil and cold cream. However, other treatment modalities proved to be more efficient than topical corticosteroids, including CO2 and Nd:YAG laser therapies, and the addition of polydeoxyribonucleotide intradermal injections, to steroid therapy. Finally, other modalities that proved to be efficient in the treatment of LS included silk undergarments, human fibroblast lysate cream, platelet-rich plasma, acitretin, and surgical intervention. The risk of bias was assessed using the Cochrane risk-of-bias tool for randomized trials. Limitations included the inclusion of only randomized controlled trials, moderate or high risk of bias, and heterogeneity in treatment regimens, among others. Conclusion: Although high-potency topical corticosteroids have validated efficacy in the management of LS, other treatment modalities, including steroid-sparing agents and/or procedural adjuncts, have been demonstrated to have a beneficial role in the treatment of LS.

'A hundred paper cuts over my vulva': A patient's experience of vulval lichen sclerosus.

This patient viewpoint illustrates the difficulties experienced by women in seeking diagnosis and treatment for vulval lichen sclerosus. The author vividly describes anatomical changes, the impact on mental health and on quality of life. In addition, this viewpoint highlights the importance of patient involvement in research, education and support.

Urethroplasty with Pedicled Tunica Vaginalis for the Treatment of Long-segment Anterior Urethral Stricture Caused by Lichen Sclerosus of Glans Penis

Urethroplasty with Pedicled Tunica Vaginalis for the Treatment of Long-segment Anterior Urethral Stricture Caused by Lichen Sclerosus of Glans Penis

From Compression to Itch: Exploring the Link Between Nerve Compression and Neuropathic Pruritus.

Neuropathic itch is a type of chronic pruritus resulting from neural dysfunction along the afferent pathway. It is often accompanied by abnormal sensations such as paresthesia, hyperesthesia, or hypoesthesia. This condition, which may involve motor or autonomic neural damage, significantly impacts patients' quality of life, causing severe itch and associated comorbidities such as depression, disrupted sleep, and social strain. Neuropathic itch accounts for 8% of chronic pruritus cases, though this may be underestimated. This comprehensive review focuses on nerve impingement as the primary pathophysiological mechanism for various forms of neuropathic itch including brachioradial pruritus (BRP), notalgia paresthetica (NP), and anogenital itch. BRP, often seen in middle-aged white women, manifests as pruritus in the dorsolateral forearms typically exacerbated by ultraviolet (UV) exposure and related to cervical spine pathology. NP, prevalent in middle-aged women, presents as pruritus in the upper back due to thoracic spine nerve compression. Anogenital pruritus, affecting 1-5% of adults, is often linked to lumbosacral spine issues after ruling out dermatologic conditions such as lichen sclerosus or lichen simplex chronicus. The pathophysiology of neuropathic itch involves both peripheral and central mechanisms, with nerve damage being a key factor. Diagnosis requires a thorough history, physical examination, and potentially imaging studies. Topical agents such as menthol, capsaicin, and lidocaine are used for mild cases, while systemic medications such as gabapentin, pregabalin, and antidepressants are prescribed for moderate to severe cases; however, no USFood and Drug Administration (FDA)-approved therapies currently exist specifically for neuropathic itch. Understanding the underlying neural dysfunction and appropriate therapeutic strategies is crucial for managing neuropathic itch effectively.

Squamoid eccrine ductal carcinoma of the vulva: A case report

Abstract Introduction/Objective A 53-year-old female sought medical attention for left vulvar pain and a vulvar lesion. Methods/Case Report Physical examination revealed a 1.0 x 1.0 cm lesion within a background of lichen sclerosus. Biopsy demonstrated a carcinoma with tubular and glandular architecture, areas of squamoid differentiation and widespread perineural invasion. Ancillary immunohistochemistry testing with keratin 7 highlighted foci of ductal differentiation. Tumor cells were also positive for p53, p63, pankeratin, and keratin 5/6. The Ki-67 proliferation index was approximately 15%. Tumor cells were negative for Keratin 20, PAX 8, p16, and CD 34. We diagnosed the lesion as a vulvar sweat gland adenocarcinoma of the squamoid eccrine ductal subtype. Results (if a Case Study enter NA) N/A Conclusion Carcinomas of vulvar sweat gland origin are extremely rare, comprising less than 1% of all vulvar cancers. To the best of our knowledge, this is the first case report of squamoid eccrine ductal carcinoma afflicting the vulva.