Insights from a joint pediatric dermatology-gynecology vulvar clinic: A retrospective study.

BACKGROUND/OBJECTIVES: Pediatric vulvar disease has not been widely explored in the medical literature. Few studies focus on vulvar disease in skin of color. The vulvar disease can be distressing for young patients given the sensitive location, and providers may lack experience in diagnosing and managing vulvar dermatoses. We sought to characterize the conditions seen, diagnostic challenges encountered, and the racial and ethnic factors associated with vulvar diseases in our multidisciplinary pediatric dermatology-gynecology vulvar clinic at Children's National. METHODS: Medical records of 220 patients who presented to our multidisciplinary pediatric dermatology-gynecology clinic were reviewed retrospectively. RESULTS: Lichen sclerosus (LS) (36%, n = 80), inflammatory vulvitis (11%, n = 23), and vitiligo (9%, n = 19) were the three most frequent conditions observed. These conditions were often misdiagnosed as one another. There was a mean delay in diagnosis after symptom onset in LS patients of 16.43 months. CONCLUSIONS: LS, inflammatory vulvitis, and vitiligo are common vulvar diseases among pediatric patients. Accurate diagnosis is important because LS must be treated aggressively to prevent sequelae. Further studies are warranted to help differentiate LS and vitiligo with consideration of skin tone.

Topical Corticosteroid Phobia Among Women Affected With Vulvar Lichen Sclerosus: Results From a Cross-sectional Survey.

OBJECTIVE: Topical corticosteroid (TC) phobia (TCP) is common in subjects affected with chronic inflammatory skin diseases who need prolonged corticosteroid treatments. The aim of this study was to assess TCP in women affected with vulvar lichen sclerosus (VLS). MATERIALS AND METHODS: This observational, cross-sectional study included adult patients with VLS who either started or were undergoing a TC treatment at our vulva unit between May 2022 and May 2023. All patients completed the self-administered TOPICOP questionnaire, which is validated for measuring concerns, worries, and beliefs about TC use. The scores obtained were analyzed in relation to demographic, history, and clinical data. RESULTS: The majority of the 165 (92.1%, 66.5 ± 11.9 years) included patients who had previously undergone TC treatments, mostly for VLS; 81.8% of them had received information about TCs, mainly from dermatologists (86.7%). The median global TOPICOP score was 16.7% (interquartile range. 8.3-30.6), corresponding to a raw median value of 6.0 (interquartile range, 3.0-11.0). The median subscores for the 2 TOPICOP domains, namely, mistaken beliefs and worries about TCs, were equal to each other. At multivariate analysis, none of the collected variables showed a significant association with the degree of TCP. CONCLUSIONS: In our VLS patients, TCP resulted rather low, probably because of the small skin area being treated and the high percentage of women who had already used TCs and who had received information about them from a dermatologist. This latter point suggests that adequate counseling could be a strong basis for greater awareness and serenity in the long-term use of TCs.

[Clinical and pathological analysis of 345 cases of vulvar lichen sclerosus and a preliminary study on the frequency of maintenance treatment].

Objective: To analyze and summarize the clinical and pathological characteristics, management, and efficacy of patients with vulvar lichen sclerosus (VLS) through a single center large sample study, and preliminarily to explore the frequency of maintenance treatment medication for VLS. Methods: The clinical data of VLS patients in Obstetrics and Gynecology Hospital of Fudan University from 2018 to 2021 were retrospectively collected. The clinicopathological characteristics (patients' age, course of disease, complicated disease history, family history, symptoms, signs and pathology), treatment and effects were retrospectively analyzed. The patients in the maintenance treatment stage were followed up regularly to explore the minimum frequency of individual medication to maintain the stability of the disease. Results: (1) General situation: a total of 345 patients with VLS were included in this study. The average age was (50.4±14.7) years (ranged from 8 to 84 years old), prevalence was highest in the 50-59 years group (30.1%, 104/345). Immune diseases occurred in 18.6% (33/177) of patients, 24.3% (43/177) of patients had allergic skin diseases, and 5.6% (10/177) of the patients' immediate family members had chronic vulvar pruritus or vulvar hypopigmentation. (2) Clinical features: the most common symptom was vulvar pruritus (96.1%, 196/204) among 204 patients with recorded symptoms. The most common sign was hypopigmentation of the vulva (96.3%, 206/214). The most common involved sites were labia minora (70.3%, 142/202), labia majora (67.8%, 137/202), and labial sulcus (59.4%, 120/202). The cumulative number of sites involved in 62 vulvar atrophy patients (2.7±1.1) was significantly higher than that in 152 non-atrophy patients (2.2±1.0; t=3.48, P=0.001). The course of vulvar atrophy was (9.3±8.5) years, which was significantly longer than that of non-atrophy patients [(6.6±5.6) years; t=2.04, P=0.046]. (3) Pathological features: among the 286 patients with electronic pathological sections, the most common pathological feature in the epidermis was epithelial nail process passivation (71.3%, 204/286). The common pathological features in the dermis were interstitial collagenization (84.6%, 242/286), and inflammatory cell infiltration (73.8%, 211/286). (4) Treatment: 177 patients received standardized treatment after diagnosis and were followed up regularly in our hospital. In the initial treatment stage, 26.0% (46/177) of the patients were treated with 0.05% clobetasol propionate cream, and 74.0% (131/177) of the patients were treated with 0.1% mometasone furoate ointment. The complete remission rates of the two methods were respectively 80.4% (37/46) and 74.0% (97/131), and there was no statistically significant difference (χ²=0.76, P=0.385). During maintenance treatment, 27.1% (48/177) of the patients took the medication twice a week, 35.0% (62/177) took the medication once a week, and 37.9% (67/177) took the medication once every 10 days. During follow-up after 6 months of maintenance treatment, there were no patients with recurrence of pruritus or progression of vulvar signs. Conclusions: The majority of VLS patients have itching, hypopigmentation, involvement of labia minora and labia majora, progressive atrophy, and inflammatory infiltration of dermis. Local treatments of mometasone furoate and clobetasol propionate have good initial therapeutic effects. The frequency exploration of individualized maintenance treatment could minimize the occurrence of adverse reactions when ensuring the stability of the patients' condition.

Comparison of the efficacy of focused ultrasound at different focal depths in treating vulvar lichen sclerosus.

OBJECTIVE: This study aimed to compare the efficacy and safety of focused ultrasound (FU) at different focal depths in treating vulvar lichen sclerosus (VLS). METHODS: A retrospective study was conducted on 84 patients with VLS. Among them, 43 cases were treated with FU at a focal depth of 2.5 mm and 41 cases at a focal depth of 4.0 mm. Therapeutic time, treatment energy, postoperative efficacy, complications and recurrence rates were compared. RESULTS: No statistically substantially differences in age, disease course, history of immune system diseases, lesion size and severity of symptoms were found between the two groups. All patients successfully received FU therapy. No significant difference in curative rate was observed between the two groups at 3, 6 and 12 months after FU therapy. At 12 months after FU therapy, the recurrence rate of the experimental group (FU treatment at 2.5 mm focal depth) was lower than the control group (FU treatment at 4.0 mm focal depth) (7.0% vs 24.4%, p = 0.027). The experimental group was treated for a shorter period of time [22.69 ± 0.64 (min) vs 24.93 ± 0.72(min), p = 0.022] and at a lower dose[5,026.05 ± 148.00(J) vs 5,484.26 ± 160.60(J) p = 0.039]. CONCLUSION: Compared with that at the routine focal depth (4.0 mm), FU therapy at a low treatment depth (2.5 mm) can achieve a similar therapeutic effect but lower recurrence rate, therapeutic time and treatment energy. This work provides insight into the optimization of clinical protocols.

Quality of Life and Treatment Adherence in Patients with Vulvar Lichen Sclerosus.

BACKGROUND: Vulvar lichen sclerosus (VLS) is a chronic, relapsing, inflammatory dermatosis that has significant impact on patients' quality of life (QoL). While disease severity and associated QoL impact have been studied, factors associated with treatment adherence and their relation to QoL in VLS remain unexplored. OBJECTIVE: The objectives of this study were to describe demographics, clinical characteristics, and skin-related QoL in VLS patients and to assess the relationship between QoL and treatment adherence. METHODS: This was a cross-sectional, single institution, electronic survey study. The relationship between adherence, measured using the validated Domains of Subjective Extent of Nonadherence (DOSE-Nonadherence) scale, and skin-related QoL, using the Dermatology Life Quality Index (DLQI) score, was assessed using Spearman correlation. RESULTS: Of 28 survey respondents, 26 provided complete responses. Among 9 patients classified as adherent and 16 classified as nonadherent, mean DLQI total score was 1.8 and 5.4, respectively. Spearman correlation between summary nonadherence score and DLQI total was 0.31 (95% CI: -0.09-0.63) overall and 0.54 (95% CI: 0.15-0.79) when patients who reported missing doses due to asymptomatic disease were excluded. Most frequently reported factors preventing treatment adherence included application/treatment time (43.8%) and asymptomatic or well-controlled disease (25%). CONCLUSIONS: Though Qol impairment was relatively small in both our adherent and nonadherent groups, we identified important factors preventing treatment adherence, with the most common being application/treatment time. These findings may help dermatologists and other providers generate hypotheses as to how to facilitate better treatment adherence among their patients with VLS, with the goal of optimizing QoL.

Long-term consequences of juvenile vulvar lichen sclerosus: A cohort study of adults with a histologically confirmed diagnosis in childhood or adolescence.

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.

A prospective pilot study to assess for histologic changes on vulvar biopsies in postmenopausal women with lichen sclerosus treated with fractionated CO2 laser therapy.

OBJECTIVES: To investigate the histologic characteristics of vulvar tissues before and after completion of fractionated carbon dioxide (CO2 ) laser therapy (FxCO2) for vulvar lichen sclerosus (LS). The secondary objective was to assess subjective improvement in symptoms via the Skindex-16 questionnaire. METHODS: This prospective single-arm study was conducted from April 2021 to August 2022 at one academic medical center. Ten postmenopausal women with biopsy-proven LS planning FxCO2 laser treatment were enrolled. Exclusion criteria included prior transvaginal mesh for prolapse, topical corticosteroid use within 8 weeks, prior pelvic radiation, malignancy, active genital infection, or pregnancy. The vulvovaginal SmartXide2-V2-LR laser system fractionated CO2 laser (DEKA) was utilized to treat visually affected areas of vulvar and perianal LS with a single pass. Subjects underwent three treatments 4-6 weeks apart. Subjects completed the Skindex-16 questionnaire and had vulvar biopsy at baseline and at 4 weeks after completion of fractionated CO2 laser therapy. Blinded histologic slides were scored by one dermatopathologist (Michael A. Cardis) rating from 1 to 5 the degree of dermal sclerosis, inflammation, and epidermal atrophy. Change scores were calculated as the difference between pre- and post-treatment scores for each subject. RESULTS: The 10 subjects enrolled had a mean age of 61 and most were white, privately insured, and had a college/graduate-level education. Post-fractionated CO2 laser treatment vulvar biopsies showed significant improvement in sclerosis and epidermal atrophy compared with pretreatment baseline biopsy specimens (p < 0.05) with no statistically significant change found in inflammation score. Skindex-16 and FSFI scores showed a trend towards improvement (p > 0.05 for both). A statistically significant correlation was found between change in sclerosis and Skindex-16 symptoms scores with an average change of 21.4 units in Skindex-16 symptoms score for every one-point change in histologic sclerosis score (p = 0.03). CONCLUSIONS: In postmenopausal women with vulvar LS undergoing fractionated CO2 laser, symptomatic improvements correlated with histologic change in degree of sclerosis on vulvar biopsy. These results demonstrate FxCO2 laser therapy as a promising option for the treatment of LS and suggest that further studies should assess degree of sclerosis on histopathology.

Characterization of patients with vulvar lichen sclerosus and association to vulvar carcinoma: a retrospective single center analysis.

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.

Incidence of Vulvar Lichen Sclerosus and of Cancer Among Vulvar Lichen Sclerosus Patients: Does Socioeconomic Status Have a Role?

OBJECTIVE: Socioeconomic status (SES) impacts on the incidence of many inflammatory diseases and cancers, but there is no evidence on its implication in vulvar lichen sclerosus (VLS). The authors aimed to assess possible associations between SES and both occurrence of VLS and cancer occurrence among VLS patients. MATERIALS AND METHODS: A retrospective cohort of women resident in the province of Ferrara, Italy, affected with VLS diagnosed between 2001 and 2020, was investigated for assessing any association of SES with VLS and cancer incidence. The SES was expressed through an ecological-based deprivation index identifying 5 subgroups. RESULT: Four-hundred women were diagnosed with VLS during the study period, with double the number of cases in the second decade (2011-2020) compared with the first (2001-2010). More VLS patients belonged to the high rather than the low SES groups (p = .032). From VLS diagnosis to 2018 (1,958.4 total person*years at risk), 22 patients received their first diagnosis of cancer, mainly the skin, breast, and vulva. No significant differences in cancer incidence were found between high/medium-high and low/medium-low SES subjects. CONCLUSIONS: The fact that more VLS patients belonged to the highest socioeconomic classes may be due to a more frequent diagnosis in those with greater health seeking behavior and resources. An involvement of SES-related factors in VLS pathophysiological background can also be taken into consideration. Both the lack of marked social and economic differences in the study area and the availability of free visits and screening may account for the lack of association between SES and cancer development.

Fat Grafting in Vulvar Lichen Sclerosus: Long Term Follow-Up.

OBJECTIVE: The rationale for the use of autologous fat grafting in the treatment of vulvar lichen sclerosus (VLS) consists in reduction of inflammation, regeneration of tissues, volume increase, and pain fiber control. The main outcome of this study was the evaluation of patients' satisfaction after treatment. Secondary outcomes included modifications in symptoms, psychosexual wellbeing, vulvar hydration, and histology after surgery. METHODS: Eligible for this study were women aged 18-85 years with a histological diagnosis of VLS who underwent at least one autologous vulvar fat grafting at the authors' center, between 2010 and 2019. In 2021, all women underwent a clinical reevaluation, comprehensive of vulvoscopy, vulvar biopsy, and handing out of validated questionnaires. RESULTS: Overall, 88.7% of patients declared themselves very satisfied/satisfied with the procedure. All symptoms were improved postsurgery; in particular, the difference was statistically significant for pruritus, burning, and dyspareunia ( p < .05). Sexual function was also improved at time of reevaluation, as were depressive and anxiety symptoms ( p < .05). No cases of vulvar intraepithelial neoplasia or cancer occurred during follow-up and vulvar architecture remained stable, although patients reported a significantly reduced need for topical steroids ( p < .0001). Lastly, in postoperative biopsies, inflammatory infiltrate was stable or reduced, and the distribution of elastic fibers was comparable or restored in most patients. CONCLUSIONS: Patient satisfaction with fat grafting is detectable up to 11 years after surgery, and as such, it may represent a valid therapeutic option in selected cases of VLS.

Steroid Phobia in Patients With Vulvar Lichen Sclerosus.

OBJECTIVE: Steroid phobia in people with dermatologic conditions is associated with noncompliance with topical corticosteroids (TCS). Although it has not been studied in those with vulvar lichen sclerosus (vLS), first-line therapy is lifelong maintenance TCS, and noncompliance is associated with impaired quality of life, progression of architecture changes, and vulvar skin cancer. The authors aimed to measure steroid phobia in patients with vLS and determine their most valued sources of information to direct future interventions to address this phenomenon. METHODS: The authors adapted a preexisting, validated scale for steroid phobia (TOPICOP), which is a 12-item questionnaire that produces a score of 0 = no phobia and 100 = maximum phobia. The anonymous survey was distributed across social media platforms with an in-person component at the authors' institution. Eligible participants included those with clinical or biopsy-proven LS. Participants were excluded if they did not consent or did not communicate in English. RESULTS: The authors obtained 865 online responses over a 1-week period. The in-person pilot obtained 31 responses, with a response rate of 79.5%. Mean global steroid phobia score was 43.02 (21.9)% and in-person responses were not significantly different (40.94 [16.03]%, p = .59). Approximately 40% endorsed waiting as long as they can before using TCS and stop as soon as possible. The most influential sources to improve patient comfort with TCS were physician and pharmacist reassurance over online resources. CONCLUSIONS: Steroid phobia is common in patients with vLS. Focused efforts to address steroid phobia among health care providers is the next best step toward improving patient comfort with TCS.

Differential proteomic expression in indolent vulvar lichen sclerosus, transforming vulvar lichen sclerosus and normal vulvar tissue.

Vulvar lichen sclerosus (VLS) confers approximately 3% risk of malignant transformation to vulvar squamous cell carcinoma (VSCC). We used unbiased proteomic methods to identify differentially expressed proteins in tissue of patients with VLS who developed VSCC compared to those who did not. We used laser capture microdissection- and nanoLC-tandem mass spectrometry to assess protein expression in individuals in normal vulvar tissue (NVT, n = 4), indolent VLS (no VSCC after at least 5 years follow-up, n = 5) or transforming VSCC (preceding VSCC, n = 5). Interferon-γ and antigen-presenting pathways are overexpressed in indolent and transforming VLS compared to NVT. There was differential expression of malignancy-related proteins in transforming VLS compared to indolent VLS (CAV1 overexpression, AKAP12 underexpression), particularly in the EIF2 translation pathway, which has been previously implicated in carcinogenesis. Results of this study provide additional molecular evidence supporting the concept that VLS is a risk factor for VSCC and highlights possible future biomarkers and/or therapeutic targets.

Lichen Sclerosus and Sexual Dysfunction: A Systematic Review and Meta-Analysis.

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.

Acantholytic Squamous Cell Carcinoma Arising From Lichen Sclerosus: A Rare Case Affecting Vulvar Skin.

We present the case of an 82-year-old female with acantholytic squamous cell carcinoma affecting vulvar skin. The patient had a history of perineal lichen sclerosus for 5 years before presentation. She was referred to a dermatologist for intractable severe pain associated with the lesions. Biopsies showed an infiltrative squamous cell carcinoma with histology consistent with the acantholytic subtype. Acantholytic squamous cell carcinoma is a rare histologic variant characterized by dyscohesive keratinocytes with pseudoglandular formation and dyskeratosis. It is associated with sun-damaged skin and most commonly occurs in the head and neck of elderly men. Few cases have been reported at nondermal sites and non-sun-exposed dermis. The patient underwent a radical vulvectomy and bilateral inguinal node dissection. The 1.6 cm tumor was diffusely acantholytic and pseudoglands were present. The tumor cells were diffusely positive for p63 immunohistochemical stain. As expected at this site, there was no solar elastosis identified histologically. However, vulvar intraepithelial neoplasia and chronic lichen sclerosus were apparent. This case represents a rare histologic subtype of squamous cell carcinoma in an unusual site associated with lichen sclerosus instead of solar elastosis.

High-intensity focused ultrasound therapy for pediatric and adolescent vulvar lichen sclerosus.

OBJECTIVE: The study's objective was to retrospectively evaluate the efficacy and safety of high-intensity focused ultrasound (HIFU) for vulvar lichen sclerosus (VLS) in pediatric and adolescent patients. METHODS: Pediatric and adolescent patients presenting to our hospital from June 2007 to July 2021, with VLS were retrospectively evaluated. The participants' information, including age, symptoms and vulvar examination, were documented, and they were treated with HIFU. The effectiveness of HIFU and its complications were analyzed. RESULTS: A total of 36 patients with VLS undergoing HIFU for whom complete follow-up data were available participated in the study. The mean age of the patients at diagnosis was 13.3 ± 4.1 years. All patients successfully underwent HIFU therapy. The mean sonication time was 20.3 ± 8.6 min, and the median treatment energy was 3579.0 J. A few blisters developed in 8 (22.2%) patients and 2 (5.6%) had ulcers. The skin burns were treated medically without scar formation. On average, patients were followed up for 52.0 months after the procedure (a range of 6-175 months). At 6 months after therapy, the total response rate was 91.6%, and 86.6% at 12 months post HIFU. Overall, 16 patients were followed up for more than 5 years. The total response rate was 75%, and the recurrence rate was 12.5%. CONCLUSIONS: Based on our results, HIFU was demonstrated to be effective and relatively safe for the treatment of VLS in pediatric and adolescent patients, but it is necessary to pay attention to the possible skin burns.

Presenting Symptoms and Diagnosis of Vulvar Lichen Sclerosus in Premenopausal Women: A Cross-Sectional Study.

OBJECTIVES/PURPOSE: Presenting symptoms of vulvar lichen sclerosus (LS) specific to premenopausal women are not well reported in the literature and may differ from those in postmenopausal women. This study aimed to characterize the presentation of vulvar LS among premenopausal women. MATERIALS AND METHODS: An observational web-based study was conducted in premenopausal women with biopsy-confirmed vulvar LS between the ages of 18-50 years. Participants completed a 28-question survey evaluating characteristics of symptoms, timing of diagnosis, alternate diagnoses, and presence of concomitant autoimmune conditions. RESULTS: Of the 956 responses received, 503 met inclusion criteria of biopsy-confirmed LS and premenopausal status. Average age of symptom onset was 27 years, and average age of diagnosis was 32 years, with a 4-year delay in diagnosis. Symptoms most present were dyspareunia (68%) and tearing with intercourse or vaginal insertion (63%). Symptoms that affect the individual most were also dyspareunia (44%) and tearing with intercourse or vaginal insertion (39%). Symptoms that most frequently prompted patients to seek medical attention were dyspareunia (35%), pruritus (31%) and tearing with intercourse or vaginal insertion (26%). Most common skin changes included hypopigmentation (81%), vulvar fissures (72%), and labial resorption (60%), with fissures affecting the individual the most (48%). Sixty-six percent of the respondents initially received an alternative diagnosis, most commonly vulvovaginal yeast infection (49%). Hypothyroidism was the most common concurrent autoimmune condition (10%). CONCLUSIONS: Vulvar LS affects premenopausal women, commonly presenting with dyspareunia and tearing with intercourse. This condition should be considered and evaluated in premenopausal women presenting with vulvar symptoms and sexual pain.

Comorbid Vulvar Lichen Sclerosus and High-Grade Squamous Intraepithelial Lesions: A Management Conundrum.

OBJECTIVE: This study aimed to determine if treating lichen sclerosus (LS) with high-potency topical corticosteroids (TCS) increases the risk of high-grade squamous intraepithelial lesion (HSIL) recurrence in patients with comorbid vulvar LS and HSIL. METHODS: This is a retrospective study of patients with comorbid vulvar LS and HSIL treated with TCS between 2015 and 2020. Patients with clinically diagnosed or biopsy-proven LS and biopsy-proven HSIL of the vulva were included. Clinical data included demographics, tobacco use, immune-modifying conditions, specimen pathology, treatment types, and HSIL recurrence. Bivariate analysis was performed to compare demographic and clinical characteristics between patients with and without HSIL recurrence. RESULTS: Twenty-six patients with comorbid LS and HSIL were identified. The median age was 66.0 years and median time in treatment for LS was 5.5 years. Thirteen (50%) had recurrence of HSIL and 13 (50%) did not have recurrence. Exposure to high-potency TCS was present in 20 (77%) patients, with 17 (65%) having use of more than 1-year duration and 9 (35%) having use at the time of HSIL diagnosis. When comparing the groups with and without HSIL recurrence, there was no significant difference in high-potency TCS exposure, duration of use, or use at time of HSIL diagnosis. CONCLUSIONS: High-potency TCS use for the treatment of LS did not seem to increase the risk of HSIL recurrence in patients with comorbid vulvar LS and HSIL. This suggests that high-potency TCS can be appropriately used for the treatment of LS even when HPV-associated disease is present.

Risk of Development of Vulvar Cancer in Women With Lichen Sclerosus or Lichen Planus: A Systematic Review.

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

Quality of life of women with untreated vulval lichen sclerosus assessed with vulval quality of life index (VQLI).

BACKGROUND/OBJECTIVE: The diagnosis of vulval lichen sclerosus (VLS) is often delayed, and little is known about quality of life (QoL) of women with it prior to receiving diagnosis and treatment. This study aimed to investigate the impact on QoL on patients with previously untreated VLS using the Vulval Quality of Life Index (VQLI). METHODS: A prospective cross-sectional survey of patients attending a dermato-gynaecology practice in Sydney, Australia from March 2018 to November 2019. Patients with a new biopsy-proven diagnosis of VLS not previously commenced on topical corticosteroid treatment were invited to complete the VQLI. RESULTS: A total of 68 participants with median age of 58 (interquartile range, IQR 48-67) years. Median symptom duration was 24 (IQR 11-60) months. Scarring was present in 53% of participants. The median total score was 13.5 (IQR 7.5-22.0), global score 1.0 (IQR 1.0-2.0). Twelve per cent had nil to minimal effect on QoL, 38% had a mild effect on QoL, 28% had a moderate effect on QoL, 22% had a severe effect on QoL, and none had a very severe effect on QoL. The highest-scoring domains were symptoms, followed by sexuality, anxiety and activities of daily living. CONCLUSION: There was significant impairment in all QoL areas of women with untreated VLS. Most experienced moderate-to-severe impairment as a result of the disease and approximately half had already developed scarring at the time of diagnosis. The findings emphasise the importance of improved awareness, early diagnosis and early commencement of treatment in VLS.

Characteristics Among Premenarchal Girls With Lichen Sclerosus.

OBJECTIVES: Lichen sclerosus (LS), a chronic inflammatory dermatosis localized in the anogenital region, is known to be associated with lower urinary tract symptoms (LUTS) in postmenopausal women; however, there is a paucity of data on prepubertal girls. We sought to characterize the relationship between LS, LUTS, and constipation among premenarchal girls. MATERIALS AND METHODS: We conducted a retrospective chart review of premenarchal girls presenting at the pediatric adolescent gynecology and pediatric urology clinics at our institution diagnosed with vulvar LS during a 4-year period. Demographics, clinical presentation, time from onset of urinary and/or bowel symptoms to LS diagnosis, and treatment response were assessed. Analysis was completed using median, range, and nonparametric statistical analysis where appropriate. RESULTS: Nineteen premenarchal girls with LS met inclusion criteria. The median age at LS diagnosis was 6.2 years (range = 3.3-13.3 yrs). Most girls (12/19, 63.2%) presented with LUTS during LS evaluation, and 11 (57.9%) of 19 patients had constipation. Pruritus and hypopigmentation were the most frequently encountered LS symptom and sign, respectively. Fourteen (73.7%) of the 19 girls had resolution/improvement of LS symptoms after LS treatment. Of the 12 patients with LUTS, 7 (58.3%) had resolution/improvement of LUTS symptoms, 3 (25.0%) had unchanged voiding symptoms, and 2 (16.7%) had unknown changes. CONCLUSIONS: Although further studies are needed to better characterize the relationship between LUTS, constipation, and LS, our findings suggest that premenarchal subjects presenting with LUTS should be assessed for LS. Likewise, patients presenting with LS should be assessed for LUTS and constipation.