Search

Co-occurrence of blisters in patients with lichen sclerosus (LS) can raise the question of whether they represent a bullous variant of LS or a concomitant autoimmune disorder. We report a rare case of bullous pemphigoid (BP) occurring on previous LS lesions. To the best of our knowledge, this is also the first BP180-negative case reported in literature. Here, we propose alternative mechanisms, independent of BP autoantibodies, that may lead to development of BP on skin affected by LS. In addition, we provide a literature review that explores the underlying pathophysiology and offers practical treatment insights, equipping clinicians with valuable guidance for similar complex cases.

Urethroplasty for the management of long-segment urethral strictures associated with lichen sclerosus presents considerable clinical challenges. Oral mucosal grafts are commonly employed but are vulnerable to posttransplantation infection and recurrent stricture formation. Furthermore, the necessity for anesthesia and oral graft harvesting restricts their application in primary healthcare settings. The single layer of flattened epithelium of the tunica vaginalis can serve as a potential alternative to oral mucosa. Animal experiments have demonstrated that the tunica vaginalis can readily form a tight connection with the multilayered urothelium of the urethra. Utilizing the tunica vaginalis as a scaffold for urethral re-epithelialization may help reduce the risk of recurrence of urethral stricture after surgery. Over a 19 year period, pedicled tunica vaginalis urethroplasty has been used for successfully treating 86 cases. The surgical procedure involves dorsally incising the urethral stricture segment, then covering it with a pedicled tunica vaginalis patch followed by suturing. Postoperatively, the pedicled tunica vaginalis graft exhibits good vascularization and take rate, facilitating urethral re-epithelialization. The surgical procedure is conducted in a sterile environment to mitigate the potential for infectious complications. Moreover, the operation can be executed under spinal anesthesia, which facilitates its implementation in primary healthcare settings.

Introduction: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness. It may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are redness and oedema, typically followed by whitening of the genital skin; sometimes fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early., Objectives: The guideline intends to provide guidance on the diagnostic of LS, highlight important aspects in the care of LS patients (part 1), generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects., Materials and Methods: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives., Results: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases., Conclusions: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term., (© 2024 European Academy of Dermatology and Venereology.)

Introduction: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness; it may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are usually a whitening of the genital skin, sometimes preceded by redness and oedema; fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early., Objectives: The guideline intends to provide guidance on the diagnostic of LS (part 1), highlight important aspects in the care of LS patients, generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects., Materials and Methods: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives., Results: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases., Conclusions: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term., (© 2024 European Academy of Dermatology and Venereology.)

Competing Interests: Conflicts of interest None disclosed.

Introduction: Lichen sclerosus et atrophicus is an inflammatory, scarring dermatosis of the female anogenital area and may lead to pain and sexual dysfunction. In select cases which are refractory to conservative therapy, surgery may provide significant symptom improvement. The objective of this study was to expand the range of surgical treatment options for these patients by presenting the operative outcomes of a specialised reconstructive method using the anterior obturator artery perforator (aOAP) flap., Methods: A retrospective cohort study was conducted on sexual outcomes following the excision of affected vulvovestibular tissue by skinning vulvectomy and subsequent single-stage reconstruction using the aOAP flap. Additional procedures, such as the Omega-Domed (OD) flap, scar surgery and clitoral re-exposure, were performed when indicated., Results: Between 2014 and 2022, a total of 61 patients were surgically treated and retrospectively included in this study. Vulvectomy and subsequent reconstruction with bilateral aOAP flaps were performed in 53 (87%) cases. There was a significant reduction in the prevalence of dyspareunia and inability to have sexual intercourse at the 1-year follow-up compared to baseline (p < 0.001). There were several minor, reversible complications that required secondary intervention., Conclusions: The outcomes of this study indicate a substantial improvement in sexual function, evidenced by a significant reduction in dyspareunia and an increased ability to engage in sexual intercourse. Altered tissue quality in patients with lichen sclerosus et atrophicus and long-term cortisone application may predispose this patient population to a higher risk of minor post-operative complications., Clinical Trial Registration Number: DRKS00033261., (Copyright © 2024 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: Lichen sclerosus (LS) is a chronic inflammatory disorder, presenting with pruritis and hypopigmentation of the vulvar and anogenital skin. LS presenting as a peri-clitoral mass has not been previously described., Case: A 5-year-old patient with vulvar pruritis and ultrasound showing a homogenous mass was referred for suspected clitoromegaly with normal labs. Examination demonstrated a prepubertal patient with a mobile, soft, peri-clitoral mass and surrounding hypopigmentation consistent with LS. The cyst was excised surgically; pathology revealed an epidermal inclusion cyst. Postoperatively, she began using topical steroids for LS with symptom resolution., Conclusion: Thorough workup of clitoromegaly negative for hormonal causes requires further investigation to determine an alternative etiology of the mass. We suspect that inflammatory changes of LS and pruritus resulted in the peri-clitoral inclusion cyst., Competing Interests: Conflicts of Interest The authors have no conflicts of interest to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Abstract: Lichen sclerosus (LS) is a frequently encountered inflammatory skin disorder characterized by whitened, atrophic patches that can cause pain and pruritus. The underlying cause of this condition remains unknown. Primarily affecting the genital area, this condition carries an increased risk of developing cutaneous cancers and frequently co-occurs with autoimmune disorders. Our retrospective study aimed to explore histologic features of LS, with a particular focus on a newly established finding and its potential implications. We examined 53 histologic cases of LS collected over 2 years. Experienced pathologists evaluated and reached a consensus on the assignment of histologic features. Patient charts were manually reviewed to gather relevant demographic and clinical data. Statistical analysis was performed using IBM SPSS Statistics (2021). Of the 53 total patients identified as meeting criteria for inclusion in this study, only 8 (15%) were male. Eight cases (15%) demonstrated perineural inflammatory infiltrate. Notably, half of all samples from male patients exhibited perineural inflammatory infiltrate. A statistically significant increase ( P < 0.01) in the presence of dermal plasma cells was identified in cases with perineural inflammation versus cases without this feature. The findings of our study highlight the recurrent nature of perineural inflammation in LS, providing valuable insights into this condition. Furthermore, we observed a notable correlation between perineural inflammation, male patients, and the presence of dermal plasma cells. These discoveries contribute to a better understanding of the underlying mechanisms of LS and suggest avenues for future research into the condition., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: There is a paucity of reported long-term outcomes after contemporary urethroplasty. Our objective is to determine the long-term success of modern urethroplasty and identify factors associated with stricture recurrence in this context., Materials and Methods: Patients undergoing urethroplasty from July 2003 to May 2013 with at least 100 months of follow-up were identified. Long-term outcomes including stricture recurrence and patient satisfaction were evaluated by review of regional/provincial electronic records and telephone interview. Urethroplasty failure was defined as a recurrent stricture (<16F) confirmed on cystoscopy. Cox regression was used to evaluate variables associated with long-term stricture recurrence., Results: A total of 733 patients were identified with ≥ 100 months follow-up. Median patient age was 45 years, stricture length was 4.7 cm, and 85.8% failed prior endoscopic treatment. At a median follow-up of 12.3 years, 89 recurrences were observed. Cumulative incidence of stricture recurrence was 6%, 10%, and 12% after 1, 5, and 10 years, respectively. From a patient-reported perspective, 89% of patients reported being satisfied with the outcome of surgery. On multivariable analyses, increasing stricture length (HR 1.1, 95% CI 1.05-1.15; P < .001) and stricture etiology ( P < .001), in particular lichen sclerosus (HR 4.46, 95% CI 2.25-9.53), radiation (HR 4.25, 95% CI 1.65-10.9), and infectious strictures (HR 5.27, 95% CI 2.03-13.7), were independently associated with stricture recurrence., Conclusions: This study affirms the widely held belief that modern urethroplasty provides high long-term patency and patient-reported satisfaction. Patients with longer strictures as well as those with lichen sclerosus, radiation, and infectious etiologies have a higher hazard of stricture recurrence in the long term.

Purpose: We evaluate the efficacy and safety profiles of currently available conservative management options for penile and urethral lichen sclerosus., Materials and Methods: A systematic review of existing literature on lichen sclerosus was conducted utilizing the PubMed, Embase, and Web of Science databases. References were assessed for relevance to nonsurgical management of male genital lichen sclerosus by title and abstract by 3 independent reviewers, then reviewed in full and in duplicate by 5 independent reviewers., Results: Seventeen studies describing conservative management of histologically confirmed penile and urethral lichen sclerosus in male patients were included in the final review. We present available evidence supporting the use of 4 major treatment modalities represented in the existing literature: topical corticosteroids, tacrolimus, platelet-rich plasma, and CO 2 laser. We also briefly discuss the limited studies on the use of oral acitretin and polydeoxyribonucleotide injections. Outcomes assessed include symptoms, clinical appearance, quality of life, sexual satisfaction, adverse effects, and long-term efficacy of treatment., Conclusions: Topical corticosteroids remain the mainstay of conservative management of penile and urethral lichen sclerosus, with current literature supporting the use of other therapies such as tacrolimus and platelet-rich plasma as alternatives or adjuvant treatments when escalation of treatment is necessary. Future research should further explore the efficacy and safety of newer therapies through additional controlled clinical trials in the targeted population.

Lichen sclerosus (LS) and lichen planus (LP) are chronic inflammatory dermatoses of unknown aetiology. They pose the most important differential diagnoses of inflammatory dermatoses in the genital area. There is often a delay in diagnosing LS and LP and subsequently treatment is initiated late in the course of the disease, which will lead to scarring and a decreased quality of life. There is an increased risk of the development of malignancies in the genital area in both diseases; however, early and continuous treatment with potent topical steroids will decrease this risk., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Introduction: Vulvar lichen sclerosus (VLS) occurs in at least one in 900 girls. There is limited knowledge as to what extent the disease persists in adulthood and what the repercussions in adulthood may be. The aim of this study is to evaluate the long-term consequences of VLS diagnosed in childhood or adolescence., Material and Methods: The population of females histologically diagnosed with VLS in childhood or adolescence in the Netherlands between 1991 and 2015 was identified through the national pathology database. Histological specimens were retrieved and re-evaluated. Potential participants for whom the diagnosis was reconfirmed and who are now adults, were then traced and surveyed. Descriptive statistics were calculated and compared with the literature. Main outcome measures are the demographics of the cohort, their scores on standardized quality of life (QoL) and sexuality questionnaires and answers to additional questions regarding patients' experience with the disease. The questionnaires used were the Dermatology Life Quality Index (DLQI), the Skindex-29, the Female Sexual Function Index (FSFI) and the Female Sexual Distress Scale-Revised (FSDS-R). Secondary outcome measures include obstetric history and histological features found in the original tissue specimens., Results: A total of 81 women participated, median age 29.0 years, median follow-up from childhood diagnosis 19.5 years. Both QoL and sexuality were somewhat affected in 51.9% of cases. Less than half (45%) reported having regular check-ups. Forty-five (56%) reported symptoms within the past year; of those with symptoms, 14 (31%) were not under surveillance. Cesarean section rate (14.5%) was comparable to the general population, and there were more high-grade obstetric anal sphincter injuries with vaginal deliveries than expected. Sixteen respondents (20%) were not aware of the childhood diagnosis prior to this study., Conclusions: Symptoms due to VLS are reported by most adults diagnosed as juveniles. QoL and sexuality are affected and correlate to recent symptoms. VLS as a juvenile does not preclude a vaginal delivery. Women diagnosed with VLS in childhood or adolescence are often lost to follow-up., (© 2023 The Authors. Acta Obstetricia et Gynecologica Scandinavica published by John Wiley & Sons Ltd on behalf of Nordic Federation of Societies of Obstetrics and Gynecology (NFOG).)

Long-segment lichen sclerosus (LS) urethral stricture is a challenge for urologists. Limited data are available for surgeons to make a surgical decision between Kulkarni and Asopa urethroplasty. In this retrospective study, we investigated the outcomes of these two procedures in patients with LS urethral stricture. Between January 2015 and December 2020, 77 patients with LS urethral stricture underwent Kulkarni and Asopa procedures for urethroplasty in the Department of Urology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine (Shanghai, China). Of the 77 patients, 42 (54.5%) underwent the Asopa procedure and 35 (45.5%) underwent the Kulkarni procedure. The overall complication rate was 34.2% in the Kulkarni group and 19.0% in the Asopa group, and no difference was observed ( P = 0.105). Among the complications, no statistical difference was observed in the incidence of urethral stricture recurrence ( P = 0.724) or glans dehiscence ( P = 0.246) except for postoperative meatus stenosis ( P = 0.020). However, the recurrence-free survival rate between the two procedures was significantly different ( P = 0.016). Cox survival analysis showed that antiplatelet/anticoagulant therapy use ( P = 0.020), diabetes ( P = 0.003), current/former smoking ( P = 0.019), coronary heart disease ( P < 0.001), and stricture length ( P = 0.028) may lead to a higher hazard ratio of complications. Even so, these two techniques can still provide acceptable results with their own advantages in the surgical treatment of LS urethral strictures. The surgical alternative should be considered comprehensively according to the patient characteristics and surgeon preferences. Moreover, our results showed that antiplatelet/anticoagulant therapy use, diabetes, coronary heart disease, current/former smoking, and stricture length may be contributing factors of complications. Therefore, patients with LS are advised to undergo early interventions for better therapeutic effects., (Copyright © 2023 Copyright: © The Author(s)(2023).)

Competing Interests: Conflicts of interest None disclosed.

Objective: Lichen sclerosus (LS) is a chronic inflammatory skin disease. In male patients, it usually involves the glans penis and foreskin and can cause phimosis or meatal stenosis. The aim of this cross-sectional case-control study was to identify clinically important comorbidities in male patients with LS., Materials and Methods: By searching Turku University Hospital electronic health records, the authors identified 630 male patients diagnosed with LS between 2004 and 2020. To investigate possible comorbidities, the authors compared this patient group to a 10-fold larger control group., Results: The incidence of LS increased during the study period, from 5 to 27.5 per 100,000 men. Patients were most often diagnosed at 21 to 25 years of age. Patients with LS exhibited markedly increased risks of penile carcinoma (odds ratio [OR], 81.0; 95% CI = 10.82-3516.7; p < .001) and carcinoma in situ of the penis (OR = 60.5; 95% CI = 7.32-2738.9; p < .001). Patients also more commonly exhibited lichen planus (OR = 16.8; 95% CI = 8.97-32.39; p < .001), psoriasis (OR = 3.3; 95% CI = 1.80-5.70; p = .004), angina pectoris (OR = 1.8; 95% CI = 1.10-2.81; p = .013), obesity (OR = 2.6; 95% CI = 1.72-3.77; p < .001), type 2 diabetes (OR = 2.3; 95% CI = 1.74-3.09; p < .001), and hypertension (OR = 1.9; 95% CI = 1.53-2.37; p < .001). The most commonly performed urological procedures were operation for phimosis, uroflowmetry, and ultrasound measurement of residual urine., Conclusions: Genital malignancies, other dermatological conditions, and diseases related to metabolic syndrome should be considered when treating patients with LS., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.)

Lichen sclerosus (LS) is a chronic lymphocyte mediated inflammatory mucocutaneous disease of unknown aetiology with a predilection for the anogenital region, and affecting both sexes. The disease is characterized by pain, intolerable itching and scarring. In late stages of LS, disfiguring scarring can drastically alter the structural anatomical architecture of the genitals. The association between genital LS and different malignant tumours is a concern that needs to be further investigated. An association between LS and several autoimmune diseases has been confirmed in recent studies. All registered citizens of Region Jönköping, Sweden were included in the present study. Patients diagnosed with LS (n = 5680) between 2001 and 2021 were identified using ICD-10 code L90.0 and selected as cases. All other individuals (n = 362 568) served as controls. Odds ratios (ORs) for the selected comorbidity were calculated and adjusted for age and sex. The cumulative incidence of LS for the entire population over a 20-year period was 1.54% (15.4 per 1000 people). The cumulative incidences over a 20-year period for females and males were 2.13% and 0.97%, respectively. This study confirmed the association between LS and vulvar cancer (OR = 17.4; 95% CI 12.1-25.3), penis cancer (OR = 9.1; 95% CI 4.3-18.9), prostate cancer (OR = 2.0; 95% CI 1.6-2.4) and breast cancer (OR = 1.6; 95% CI 1.4-1.8). LS was also associated with Crohn´s disease (OR = 2.0; 95% CI 1.6-2.6) and diabetes mellitus type 1 (OR = 1.9; 95% CI 1.6-2.1). The present study revealed novel important data regarding the association of LS with cancer and autoimmune diseases, emphasising the importance of sufficient treatment and follow-up of patients with LS. However, future studies are needed to confirm these results and the potential role of LS in the development of cancer., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Objective: To describe differences in the urinary microbiome of patients with pathologically confirmed lichen sclerosus (LS) urethral stricture disease (USD) vs non-lichen sclerosus (non-LS) USD pre- and post-operatively., Methods: Patients were pre-operatively identified and prospectively followed, all underwent surgical repair and had tissue samples obtained to make a pathological diagnosis of LS. Pre- and post-operative urine samples were collected. Bacterial genomic DNA was extracted. Alpha and beta diversity measurements were calculated and compared. A zero-inflated negative binomial model was utilized to compare taxa abundances between disease status and surgery status., Results: Urine samples were obtained from both cohorts, 69 samples in total: 36 samples were obtained pre-operatively and 33 samples were obtained post-operatively. Ten patients provided both a pre-operative and post-operative urine sample. Twenty-six patients had pathological evidence of LS and 33 patients did not. There was a statistically significant difference in alpha diversity between the pre-operative urine samples of patients with non-LS USD and LS USD, (p = 0.01). There was no significant difference in alpha diversity within post-operative urine samples between patients with non-LS USD and LS USD, (p = 0.1). A significant difference was observed in Weighed UniFrac distances with respect to disease and operative status, (p = 0.001 and 0.002)., Conclusions: LS USD have significant alterations in diversity and differential abundance of urine microbiota compared to non-LS USD controls. These findings could be used to guide further investigations into the role of the urinary microbiome in LS USD pathogenesis, severity of presentation, and stricture recurrence., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Vulvar lichen sclerosus is a chronic inflammatory disease involving vulvar skin. The risk of developing invasive vulvar cancer for women with LS is reported in the literature, but the risk of extra-vulvar tumors has been under-investigated. This multicentric study aims to estimate the risk of developing cancers in a cohort of women with a diagnosis of vulvar lichen sclerosus., Methods: A cohort of women diagnosed with and treated for vulvar lichen sclerosus in three Italian gynecological and dermatological clinics (Turin, Florence, and Ferrara) was retrospectively reviewed. Patient data were linked to cancer registries of the respective regions. The risk of subsequent cancer was estimated by dividing the number of observed and expected cases by the standardized incidence ratio., Results: Among 3414 women with a diagnosis of vulvar lichen sclerosus corresponding to 38,210 person-years of follow-up (mean 11.2 years) we identified 229 cancers (excluding skin cancers and tumors present at the time of diagnosis). We found an increased risk of vulvar cancer (standardized incidence ratio = 17.4; 95 % CL 13.4-22.7), vaginal cancer (standardized incidence ratio = 2.7; 95 % CL 0.32-9.771), and oropharyngeal cancer (standardized incidence ratio = 2.5; 95 % CL 1.1-5.0), and a reduced risk of other gynecological tumors (cervical, endometrial, ovarian) and breast cancer., Conclusions: Patients with vulvar lichen sclerosus should undergo annual gynecological check-up with careful evaluation of the vulva and vagina. The increased risk of oropharyngeal cancer also suggests the need to investigate oropharyngeal cavity symptoms and lesions in patients with vulvar lichen sclerosus., Competing Interests: Declaration of competing interest The authors declare that they have no competing interest., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Lichen sclerosus (LS) is an inflammatory disease mostly arising at the genital level. It is unclear whether human papillomaviruses (HPVs) have an etiological significance in LS, and data on their prevalence in patients with LS are controversial., Objectives: The authors assessed alpha, beta, and gamma HPV prevalence in patients with genital LS. The association of HPV positivity with demographic and clinical factors was also investigated., Methods: One hundred thirty-two formalin-fixed, paraffin-embedded LS samples (2016-2020) were retrieved from the archives of a pathology department. Alpha HPVs were genotyped with the INNO-LiPA HPV Genotyping Extra II kit. Beta and gamma HPVs were searched by multiplex Polymerase Chain Reaction. Immunostaining for p16 INK4a was performed on high-risk HPV-positive samples., Results: Patients had a median age of 61 years, were mostly women ( n = 73, 55.3%), and with an early disease stage ( n = 79, 59.8%). Alpha HPVs were detected in 12/132 cases (9.1%). Among the 5 high-risk HPV-positive cases, only 2 displayed a strong and diffuse p16 INK4a staining. Beta genus was the most prevalent (35/132, 26.5%) and HPV5 was the most frequent beta genotype (25/132, 18.9%). There were 3 gamma HPV-positive cases among those with a valid result (3/131, 2.3%). Multiple infections with genotypes belonging to different genera were infrequent (3/131, 2.3%). No significant differences in the prevalence of the individual genera were observed according to sex and disease stage., Conclusions: Of the 3 HPV genera, beta genus showed the highest prevalence. Further research is needed to clarify whether the presence of beta HPVs in genital LS has a clinical significance., Competing Interests: Conflict of Interest: M.B. has received nonfinancial support from Roche Diagnostics and Hologic S.r.L. and grants and nonfinancial support from Arrow Diagnostics and Becton and Dickinson for work performed outside the range of the current study. The other authors have declared they have no conflicts of interest., (Copyright © 2023, ASCCP.)

Competing Interests: Conflicts of interest Dr LaChance is a Principal Investigator for a research grant from Pfizer looking to explore the role of the JAK/STAT pathway in connective tissue disease. The other authors have no conflicts of interest to declare.

Purpose: Lichen sclerosus (LS) is a benign, cutaneous, chronic inflammatory (autoimmunological) disease. The differentiated vulvar intraepithelial neoplasia (dVIN) accounts for a precursor lesion of vulvar squamous cell carcinoma and is often associated with lichen sclerosus. Although the association between lichen sclerosus and vulvar carcinoma has long been recognized, there is a lack of evidence in literature., Methods: This retrospective study examined pseudonymized data of 499 women diagnosed with vulvar pathology between 2008 and 2020 at the Department of Gynaecology and Obstetrics of Hannover Medical School (MHH). Data were further stratified for the time of onset, location of disease, accompanying disease, HPV status and progression of disease into vulvar squamous cell carcinoma (VSCC)., Results: In total, 56 patients were diagnosed with vulvar lichen sclerosus. The mean onset of disease was at 60.3 years of age. After subdividing cases of diagnosed LS into those who did not develop vulvar carcinoma in their course and those who did, the ages at onset are 52.66 ± 17.35 and 68.41 ± 10.87, respectively. The incidence of vulvar cancer in women diagnosed with lichen sclerosus was 48.2%. Twenty-five patients reported a diagnosis of VIN in their self-reported history., Conclusions: In our retrospective study, we showed a trend between vulvar lichen sclerosus and VSCC. The difference between the two age groups of patients diagnosed with lichen sclerosus who developed vulvar carcinoma and those who did not is statistically significant. Our results highlight the importance to diagnose lichen sclerosus early to ensure adequate follow-up and prevent progression to VSCC., (© 2022. The Author(s).)

Competing Interests: Conflicts of interest Dr Cohen serves on a data and safety monitoring board (DSMB) for Advarra. The remaining authors have no conflicts of interest to declare.

Female genital lichen sclerosus is an underdiagnosed, distressing, chronic dermatosis affecting the well-being of women. The aim of this retrospective case-control study was to assess whether the disease is connected with work productivity and activity impairment, depression and decreased sexual quality of life. Fifty-one female patients with genital lichen sclerosus and forty-five healthy women were enrolled to the study and filled out an online survey including: Work Productivity and Activity Impairment: General Health (WPAI:GH), Patient Health Questionnaire-9 (PHQ-9) and The Sexual Quality of Life-Female (SQOL-F) questionnaires. The results showed that women with genital lichen sclerosus are at risk of having a diminished work productivity, are more often screened for depression and have a decreased sexual quality of life. The study highlights the importance of a multidisciplinary approach to treating female genital lichen sclerosus., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2023 Jabłonowska et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Lichen sclerosus (LS) is a chronic inflammatory skin disease commonly affecting the anogenital area with less frequent extragenital occurrence. Extragenital LS cutaneous manifestations vary and precipitating factors are not well described. Recent evidence for etiology and clinical associations of extragenital LS provide insight into disease recognition and pathogenesis. Novel diagnostic techniques as well as treatment standardization have the potential to improve management of this rare condition. This review details both past and new insights into the pathogenesis, clinical manifestations, and treatment options of extragenital LS., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Lichen sclerosus (LS) is a cutaneous disease of unknown etiology that often involves the vulva or foreskin but also can affect extragenital sites. Regardless of the anatomic site, the histomorphology and presumably pathogenesis are similar. Perhaps a clue to the pathophysiology of LS lies in its frequent association with morphea, specifically, when occurring in an extragenital context. In our experience a striking feature evident in established lichen sclerosis (LS) is one of superficial vascular drop out whereby residual vessels exhibited endothelial cell necrosis and microvascular basement membrane zone thickening, the latter reflective of antecedent episodes of microvascular injury. We sought to understand the pathophysiology that underlies the distinct vascular changes and in doing so, shed light on the pathogenesis of LS. We examined 44 cases of LS over a period of 2019 to 2021. We were able to obtain past medical histories in 34 of the 44 cases. Regarding pathological assessment, the predominant focus was on microvascular changes. We assessed the role of C5b-9 mediated vascular injury in the pathogenesis of the vasculopathy and enhanced type I interferon signaling in vessels given the morphologic semblance to the select interferonopathy syndromes, namely fibrosing dermatomyositis and Kohlmeier Degos disease. We examined the expression of CMV DNA and protein based on prior observations in an earlier study that isolated early protein expression in the microvasculature in the setting of LS and scleroderma. From a clinical perspective, the most striking association was an older age at the time of diagnosis (mean age of 62 years and median age of 61.5 years) and the presence of vascular comorbidities of diabetes, hypertension, and hyperlipidemia in almost 80% of cases. All cases showed significant microvascular changes in the superficial corium with the most frequent findings being those of significant basement membrane zone reduplication and vascular drop out. A number of cases showed prominent microvascular deposits of C5b-9 in the zone of hyalinizing fibrosis or subjacent to the discernible table of fibroplasia in the absence of enhanced type I interferon signaling. In no case were there viral cytopathic changes associated with CMV affecting the endothelium. The studies that encode CMV DNA or protein did not show a significant role for CMV reactivation in endothelium in the majority of the studied cases. It is concluded that the pathophysiology of LS includes a microvascular injury syndrome within the papillary dermis. The mechanism of endothelial cell injury is complement mediated at least in part and could reflect an adaptive immune response targeting endothelium indicative of classic complement pathway activation when coexisting with morphea or occurring in younger individuals. A non-immune based endothelial dysfunction and complement mediated injury unrelated to antibody driven classic complement pathway activation are more likely pathogenetically in the setting of certain diseases like diabetes mellitus and hypertension. Vascular drop out can be explained by the diminished endothelial progenitor pool needed to repopulate the damaged microvessels in certain settings like hypertension and diabetes., Competing Interests: Declaration of competing interest None., (Copyright © 2022. Published by Elsevier Inc.)

Objective: Lichen sclerosus (LS) is a chronic inflammatory disease with a significant impact on quality of life. The aim of this cross-sectional case-control study was to characterize concomitant urogynecological and gastrointestinal disorders in female patients with LS., Methods: A medical records search between 2004 and 2012 yielded 455 women and girls (mean age 64 years) with LS. The study cohort was compared with a 10-fold age- and sex-matched control cohort. Gynecological cancers and their precursors; gynecological, urinary, and gastrointestinal disorders; and pain syndromes were evaluated., Results: The well-known association between LS and increased risk of vulvar cancer and its precursors was also found in our study (relative risk [RR] = 100.0; p < .001 and high-grade squamous intraepithelial lesions RR = 110.0; p < .001, respectively), but we also found an increased risk for cervical cancer (RR = 6.0; p = .005) and endometrial cancer (RR = 2.9; p < .001). Gynecological pain syndromes such as dyspareunia (RR = 20.0; p < .001) and interstitial cystitis (RR = 5.0; p < .001) and urinary incontinence (RR = 4.8; p < .001) were also increased. Among gastrointestinal disorders, we found increased risk for celiac disease (RR = 6.8; p < .001), diverticular intestine diseases (RR = 1.9; p < .001), functional intestinal disorders (RR = 2.3; p = .003), and anal and rectal fissures (RR = 2.4; p = .046)., Conclusions: We found that female patients with LS have an increased risk for gynecological cancers as well as for several urogynecological and gastrointestinal disorders. Increased awareness is required to identify and treat these concomitant disorders., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.)

Background: Women with lichen sclerosus (LS) may suffer sexually because of dyspareunia, fissures, and introital narrowing. However, the literature remains limited on the biopsychosocial aspects of LS and its impact on sexual health., Aim: To examine the biopsychosocial aspects and impact of LS on the sexual health of Danish women with vulvar LS., Methods: The study was conducted with a mixed methods approach, including women with LS from a Danish patient association. The quantitative sample consisted of 172 women who completed a cross-sectional online survey that included 2 validated questionnaires: the Female Sexual Function Index (FSFI) and the Female Sexual Distress Scale (FSDS). The qualitative sample consisted of 5 women with LS who volunteered for audiotaped, individual, semistructured interviews., Outcomes: This mixed methods study combined data from 2 quantitative questionnaires (FSFI and FSDS) with qualitative interviews to achieve a comprehensive insight into the biopsychosocial aspects of sexual health in women living with LS., Results: The sexual function of women with LS was considerably affected, with FSFI scores below the cutoff value of 26.55, indicating a risk of sexual dysfunction. On average, 75% of the women were sexually distressed, with a total FSDS score of 25.47. Furthermore, 68% of the sexually active women were considerably affected in terms of sexual function and sexual distress, thus meeting international criteria for sexual dysfunction. However, a negative impact on sexual function was not always related to sexual distress and vice versa. The qualitative analysis identified 4 overarching themes: (1) decrease in or loss of sexual activity, (2) interference with relationship dynamics, (3) importance of sex and intimacy - loss and restoration, and (4) worries about sexual insufficiency., Clinical Implications: Insight into the influence of LS on sexual health is important for health care professionals, including doctors, nurses, sex therapists, and physiotherapists, to provide the best guidance, support, and management for women with LS., Strengths and Limitations: The strengths of the study are its use of a mixed methods design and the inclusion of sexual function and sexual distress. A limitation is related to the properties of the FSFI regarding women with no sexual activity., Conclusions: LS has a considerable influence on women's sexual health in terms of sexual function and sexual distress, as supported by quantitative and qualitative measures. Our understanding of the complex interactions among sexual activity, intimate relations, and causes of psychological distress has been enriched., (© The Author(s) 2023. Published by Oxford University Press on behalf of The International Society of Sexual Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Introduction and Hypothesis: Lichen sclerosus is a chronic inflammatory dermatitis, with a predilection for the anogenital area. In later stages, lichen sclerosus may develop into widespread scarring, and occasionally leading to severe introital stenosis and urinary retention. Our video is aimed at presenting a case of surgical management of lichen sclerosus-related introital stenosis determining urinary retention., Methods: An 82-year-old woman was evaluated for almost complete urinary retention, with concomitant continuous enuretic urinary leakage all day and night and recurrent urinary tract infection symptoms. The gynecological evaluation demonstrated a complete introital obliteration, without obvious communications for urine passing. After proper informed consent, the patient was admitted for vulvo-perineoplasty., Results: The featured procedure was completed in 25 min and blood loss was negligible. No surgical complications were observed. On postoperative day 1, the patient was successfully discharged home with topical steroid treatment. Histological examination confirmed typical features of lichen sclerosus pathology. At follow-up visits the patient was asymptomatic and examination confirmed persistence of introital patency., Conclusions: The featured video shows a vulvo-perineoplasty performed in a patient with lichen sclerosus-related complete introital obliteration and urinary retention. The procedure was successful in obtaining anatomical repair and relieving urinary symptoms., (© 2022. The International Urogynecological Association.)

Purpose of Review: Our objective is to review the currently available literature on the use of platelet-rich plasma (PRP) in patients with pelvic floor disorders and to critically appraise the latest evidence on the safety and efficacy of the PRP application., Recent Findings: The evidence on the use of PRP for the treatment of stress urinary incontinence (SUI) appears promising, although limited to case series, case reports and animal studies. PRP has also been proposed to enhance the surgical outcomes of pelvic organ prolapse (POP) by native tissue repair with promising success rates. The application of PRP in other PFDs including vaginal fistulas, genitourinary syndrome of menopause (GSM), mesh exposure and lichen sclerosus has been also associated with beneficial outcomes and a favorable safety profile., Summary: The currently available literature indicates that PRP could have a beneficial effect as a single or adjuvant treatment in patients with SUI, POP, GSM, vaginal fistula and genital lichen sclerosus with minimal adverse events. However, valid evidence is still lacking and further well-designed studies are warranted in the field to confirm the validity of the so far reported outcomes., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

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.

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., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 by the International Society of Gynecological Pathologists.)

Background: Lichen sclerosus (LS) is a common autoimmune dermatological condition that is often under-diagnosed in women and has been documented to affect quality of life and sexual function., Aim: To determine the prevalence of sexual dysfunction among women with vulvar lichen sclerosus., Methods: The authors conducted a systematic review and meta-analysis of the existing research on LS and sexual function in database including PubMed using search terms: lichen sclerosus OR vulvar lichen sclerosus OR vulvar lichen sclerosus et atrophicus OR kraurosis vulvae) AND (sexual function OR sexual functions OR sexual disorder OR sexual disorders OR sexual activity OR sexual activities OR sexual dysfunction OR sexual dysfunctions OR dyspareunia OR vaginismus)., Outcomes: Nearly 60% of women with lichen sclerosus suffer from sexual dysfunction., Results: Two hundred and ten studies were initially identified. Twenty-six articles met inclusion criteria and 3 were excluded as they did not relate to sexual function, were regarding a surgical or medical intervention and sexual dysfunction and one was a review article. Therefore, 23 studies were included in the final analysis resulting in a cumulative 486 participants with LS with 208 patients experiencing any kind of sexual dysfunction. Meta-analysis presented prevalence of sexual dysfunction among LS patients as 59% (95% CI: 48 - 70%). Dyspareunia or generalized pain with intercourse was the most commonly reported type of dysfunction., Clinical Implications: Discussing sexual concerns with women with LS could empower them to seek treatment., Strengths and Limitations: Few articles met criteria for inclusion., Conclusion: A large proportion of women with LS experience sexual dysfunction. More research is needed, especially that which includes biopsy-proven LS and validated tools on sexual function. Pope R, Lee MH, Myers A, et al. Lichen Sclerosus and Sexual Dysfunction: A Systematic Review and Meta-Analysis. J Sex Med 2022;19:1616-1624., (Copyright © 2022 International Society for Sexual Medicine. Published by Elsevier Inc. All rights reserved.)

Background: 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., Objectives: To determine the clinical and histological characteristics of cases of PEKMB, and evaluate treatments used and clinical course., Methods: 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., Results: 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., Conclusions: 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., (© 2022 European Academy of Dermatology and Venereology.)

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.

Abstract: 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 8&18, 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., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)