Biopsy-verified vulvar lichen sclerosus and the risk of non-vulvar cancer: A nationwide cohort study.

Vulvar lichen sclerosus (VLS) is a chronic inflammatory mucocutaneous disease known to be associated with human papillomavirus-independent vulvar squamous cell carcinoma. Evidence on the association with other types of cancer, however, is sparce. We conducted a large nationwide cohort study examining the incidence of non-vulvar cancers among women with biopsy-verified VLS compared with the general female population. By using the nationwide Pathology Registry, we identified all women in Denmark with a biopsy-verified VLS diagnosis during 1978-2019 (n = 16,921). The cohort was followed up in the Danish Cancer Registry until 2022 for a subsequent non-vulvar cancer diagnosis. Standardized incidence ratios (SIRs) were computed with 95% confidence intervals (CIs) as relative risk estimates of all specific non-vulvar cancer sites. Compared with general female population rates, women with biopsy-verified VLS had decreased rates of several non-vulvar cancers, including HPV-related cancers (combined estimate: SIR = 0.5; 95% CI: 0.3-0.7), and lung (SIR = 0.6; 95% CI: 0.5-0.7), liver (SIR = 0.5; 95% CI: 0.2-0.9), and thyroid cancer (SIR = 0.5; 95% CI: 0.3-0.9). The decreased SIRs tended to sustain throughout the follow-up period following the VLS diagnosis. This large nationwide cohort study shows that women with biopsy-verified VLS may have a long-term reduced risk of developing HPV-related (cervical, vaginal, oropharyngeal, and anal) and smoking-associated cancers (lung, liver, and cervical) as well as thyroid cancer. Future studies focusing on the mechanisms behind the decreased cancer risk are needed.

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

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

Evaluation of the therapeutic effects of Photodynamic Therapy in vulvar lichen sclerosus and impact on patient quality of life and sexual funtion.

BACKGROUND: Vulvar lichen sclerosus (VLS) is often associated with irritable symptoms of itching, burning pain and can lead to scarring, architectural changes and sexual dysfunction as well as a decline in quality of life.The etiology of the disease is unknown. This study sought to assess the therapeutic effects of Photodynamic Therapy (PDT) in VLS, and improvment of patient quality of life and sexual funtion. METHODS: From January 2022 to April 2023, a total of 65 patients with vulvar sclerosi (VLS) were treated with PDT in our hospital. All 65 patients were divided into two groups: early-stage group and late-stage group. The Cattaneo scoring method, the Dermatology Life Quality Index (DLQI) and the Female Sexual Function Index (FSFI) scores were used to evaluate the clinical effectiveness of the treatment on patients' symptoms and clincal signs, quality of life as well as sexual function before and at 6-month after treatment. RESULTS: The total effective rate of early-stage patients was significantly greater than that of late-stage patients at 6-month after PDT treatment (90.91% [40/44] vs 76.19% [16/21], p <0.05). At 6-month follow-up, the symptoms and clinical signs of patients in early-stage group were significantly improved compared with baseline, the average scores of itching, skin elasticity, whitening and lesion range were significantly lower than the scores before treatment (p <0.05). In late-stage group, The decrease in scores of itching, whitening and lesion range at the 6-months follow-up is significant(p <0.05), but skin elasticity (p=0.0625). On post-treatment follow-up examination, FSFI score was seen to have significantly improved in early-stage patients(from a median score of 17.45 to 21.1, p<0.05); DLQI also significantly improved after treatment (from a median score of 7 to 4, p<0.05). In late stage patients, The DLQI score improved significantly after treatment (from a median score of 18 to 15, p<0.05). However, the improvement in sexual function is not statistically significant (pre-treatment: median=10.55, post-treatment: median=10, p=0.1865). CONCLUSION: Photodynamic therapy can effectively improve most symptoms and clinical signs, as well as quality of life of patients with VLS, especially for earlly stage patients. Moreover, improvement in sexual function is observed in early stage patients after PDT treatment. This study suggests that early and timely PDT treatment are recommended to achieve better results.

Patient satisfaction and sexual issues in vulvar lichen sclerosus treatment: a monocentric certified dysplasia unit survey analysis.

OBJECTIVE: Vulvar lichen sclerosus (VLS) is an underestimated chronic disease. It can cause significant symptom burden and sexual dysfunction. This study aimed to evaluate patient satisfaction and current challenges in the management of VLS in a certified dysplasia unit, particularly during the COVID-19 pandemic. METHODS: This survey analyzed patients who had been diagnosed with VLS and treated at our DKG-certified dysplasia unit. The study was conducted during the COVID-19 pandemic in the Department of Gynecology and Obstetrics at the University of Aachen. The questionnaire contained 43 questions on general treatment, diagnostic delays, disease education, psychologic and sexual issues, and specific questions regarding the COVID-19 pandemic. The questionnaires were distributed between January 2021 and September 2023. RESULTS: This study included 103 patients diagnosed with VLS, who were treated at our certified dysplasia unit. Overall, 48% of the patients were satisfied with the success of the therapy. Most participants reported psychologic problems (36.8%), fear of cancer (53.3%), or sexual restrictions (53.3%). Among the patients, 38% were bothered by the regular application of topical cortisone. However, 72% were willing to undergo treatment for more than 24 months. The COVID-19 outbreak in March 2020 had a significant negative impact on general VLS care from the patient's perspective (3.83/5 before vs. 3.67/5 after; p = 0.046). There was a general request for booklets to inform and educate the patients about their disease. Furthermore, the respondents demanded a telephone hotline to answer the questions and wished for follow-up visits via e-mail to cope better with their current situation. CONCLUSION: This study highlights the need for more effective treatments for VLS and an increased awareness of psychologic and sexual distress. To ensure patient well-being and satisfaction, it is imperative to offer individualized care with adequate disease education in a team of specialists from various disciplines.

Is there a role for platelet rich plasma injection in vulvar lichen sclerosus? A self-controlled pilot study.

BACKGROUND: Owing to the evidence that as many as 30-40% of patients with vulvar lichen sclerosus (VLS) fail to report a remission of symptoms with first-line corticosteroid treatment (TCS), especially as what regards dyspareunia, we aimed to analyze patients' satisfaction following vulvar injection of autologous platelet-rich plasma (PRP). This is intended as an adjunctive treatment, to be used following TCS, and appears to promote tissue repair. It may also possibly have immunomodulatory proprieties. MATERIALS AND METHODS: Patients with VLS were considered eligible for this pilot study if, despite having been treated with a 3-month TCS regimen, they reported a persistence of symptoms. PRP was produced in a referral center using a manual method and a standardized protocol. Each patient received three treatments 4 to 6 weeks apart. RESULTS: A total of 50 patients with a median age of 53 years [IQR 38-59 years] were included in the study. 6 months after the last injection of PRP all patients were either satisfied or very satisfied with the treatment (100%; 95% CI 93-100%). Median NRS scores for itching, burning, dyspareunia and dysuria were significantly reduced (p < 0.05) and FSFI, HADS and SF-12 questionnaires revealed a significant improvement in sexual function, psychological wellbeing and quality of life (p < 0.05). The number of patients reporting the need for maintenance TCS treatment was reduced by 42% (p < 0.001) and an improvement in vulvar elasticity and color was reported in all patients. CONCLUSION: Following standard medical therapy, PRP may be effective not only in improving symptoms, but also in restoring function.

The Need of Differential Diagnosis Between Vulvar Lichen Sclerosus and Autoimmune Dermatoses in Adolescent Girls.

INTRODUCTION: Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which can manifest in prepubertal or adolescent patients. The prevailing theories point to autoimmune and genetic factors. The primary symptoms of VLS typically include vulvar itching, discomfort, dysuria, and constipation. Physical examination often reveals a characteristic figure 8 pattern, involving the labia minora, clitoral hood, and perianal region. However, these symptoms and the age of onset are nonspecific and require differentiation from autoimmune dermatoses such as bullous diseases, pemphigus diseases, epidermolysis bullosa acquisita, and dermatitis herpetiformis. We performed this study to distinguish VLS from autoimmune dermatoses, and in doing so, uncover the underlying causes of chronic vulvar changes. This knowledge will enable healthcare providers to offer appropriate medical care to affected patients. METHODS: The study was conducted between July 2020 and February 2021, with a sample of 55 girls aged 2-18 years who did not have any systemic diseases. The study group was composed of 20 girls previously diagnosed with vulvar lichen sclerosus, while the control group included 35 girls without VLS. Questionnaires regarding the medical history of the children were completed by their legal guardians. Blood samples were collected and analyzed biochemically to assess human immunoglobulin A (IgA), IgG, and IgM antibodies against various substrates, including the desmosome of stratum spinosum, basement membrane zone, desmoglein 1 (DSG1), desmoglein 3 (DSG3), BP180-NC16A-4X, BP230gC, pemphigoid antigen, collagen type VII NC1, transitional epithelium, gliadin (GAF-3X), endomysium (EMA), and cellular nucleus (ANA). RESULTS: The analysis of the study group revealed that the most commonly observed signs and symptoms included: itching, soreness, burning sensations, and excoriation, as well as erythema or/and pallor of the skin and perineal mucosa. Among the assessed antibodies, only anti-GAF3x antibodies and ANA antibodies were detected. However, the results did not reach statistical significance (p > 0.5).

Clinical efficacy analysis of 5-aminolevulinic acid photodynamic therapy for vulvar lichen sclerosus.

OBJECTIVE: The purpose of this study is to analyze the efficacy of photodynamic therapy in the treatment of vulvar lichen sclerosus who do not respond to topical glucocorticoid therapy, analyze whether there are factors that affect the efficacy, and identify adverse reactions to the treatment. METHOD: This retrospective study included 42 patients with vulval lichen sclerosis treated with ALA-PDT. Basic data of all patients were collected, and the clinical symptoms and signs of the patients before treatment were evaluated. After one year of treatment, the clinical efficacy was evaluated and analyzed whether there were any factors that affected the treatment effect. RESULT: One year after the ALA-PDT treatment, the clinical effective rate was 64.29 % (27/42), the general effective rate was 19.05 % (8/42), the ineffective rate was 4.76 % (2/42), and the recurrence rate was 11.90 % (5/42). There was no correlation between menopause, number of births given, body mass index, duration of disease, treatment times and treatment effect. For patients with severe itching and atrophy, PDT was less effective. Adverse effects were minimal and no structural complications were reported. CONCLUSION: ALA-PDT can obviously alleviate itching in VLS patients, improve skin elasticity, skin color and reduce lesion area. ALA-PDT for VLS has a low recurrence rate and few side effects.

Advances in the pathogenesis of vulvar lichen sclerosus.

Vulvar lichen sclerosus (VLS) is a chronic non-neoplastic skin lesion characterized by vulvar itching, pain, atrophy, whitening of the skin and mucous membranes, and gradual atrophy and disappearance of the labia minora, which can eventually lead to vulvar scarring, causing functional impairment and seriously affecting the patient's physical and mental health. VLS can occur at any age, however, its pathogenesis and etiology are not fully understood. Considerable progress has been made in related research on genetic susceptibility factors, autoimmune disorders, collagen metabolism abnormalities, and their triggering factors in disease formation and progression. This article reviews the etiology of vulvar lichen sclerosus.

Assessment of the Cutaneous Hormone Landscapes and Microbiomes in Vulvar Lichen Sclerosus.

Vulvar lichen sclerosus (VLS) is a progressive skin disease of unknown etiology. In this longitudinal case-control exploratory study, we evaluated the hormonal and microbial landscapes in 18 postmenopausal females (mean [SD] age: 64.4 [8.4] years) with VLS and controls. We reevaluated the patients with VLS after 10-14 weeks of daily topical class I steroid. We found that groin cutaneous estrone was lower in VLS than in controls (-22.33, 95% confidence interval [CI] = -36.96 to -7.70; P = .006); cutaneous progesterone was higher (5.73, 95% CI = 3.74-7.73; P < .0001). Forehead 11-deoxycortisol (-0.24, 95% CI = -0.42 to -0.06; P = .01) and testosterone (-7.22, 95% CI = -12.83 to -1.62; P = .02) were lower in disease. With treatment, cutaneous estrone (-7.88, 95% CI = -44.07 to 28.31; P = .62), progesterone (2.02, 95% CI = -2.08 to 6.11; P = .29), and 11-deoxycortisol (-0.13, 95% CI = -0.32 to 0.05; P = .15) normalized; testosterone remained suppressed (-7.41, 95% CI = -13.38 to -1.43; P = .02). 16S ribosomal RNA V1-V3 and ITS1 amplicon sequencing revealed bacterial and fungal microbiome alterations in disease. Findings suggest that cutaneous sex hormone and bacterial microbiome alterations may be associated with VLS in postmenopausal females.

The Potential Coexistence of Autoimmune Thyroid Diseases and Pediatric Vulvar Lichen sclerosus.

INTRODUCTION: Vulvar lichen sclerosus (VLS), a chronic inflammatory skin disorder, often coexists with autoimmune thyroid disease (AITD). VLS presents with subtle symptoms including vulvar itching and discomfort. Clinically, a "Figure 8" pattern involving the labia minora, clitoral hood, and perianal region is often observed. It is prevalent both in pre-pubertal girls and women aged 40-60, and the link between VLS and AITD remains unclear, with proposed causes including autoimmune, hormonal or genetic factors, and environmental triggers. This study addresses the lack of research on the association in children, aiming to investigate the largest group of underage girls to date. AIM: This study aimed to investigate the coexistence of thyroid autoimmune diseases in girls diagnosed with vulvar lichen sclerosus (VLS) and to assess the presence of antibodies for specific thyroid autoimmune diseases. MATERIALS AND METHODS: Our study was conducted from July 2020 to February 2021, involving a sample of 55 girls aged 2-18 years old, all free from systemic diseases. The study group comprised 20 girls previously diagnosed with vulvar lichen sclerosus (VLS), while the control group included 35 girls without VLS. Legal guardians completed questionnaires detailing the medical history of their children. Blood samples were collected from all participants and subjected to biochemical analysis. The presence of human IgG antibodies against thyroid peroxidase and IgG antibodies against thyroglobulin was assessed using the immunoenzymatic method with commercially available ELISA kits. RESULTS: In the study group, common symptoms included itching, soreness, burning sensation, excoriation, and erythema or pallor of the skin and perineal mucosa. An evaluation of anti-thyroglobulin and anti-thyroid peroxidase antibodies revealed no statistical significance between the study and control groups (anti-TG p = 0.379, anti-TPO p = 0.96). Family history of autoimmune diseases showed no significant correlation with anti-thyroid antibodies in girls. Although no significant relation between VLS occurrence and antibody levels was found, it emphasizes the need for multidisciplinary medical care. Further research with larger patient groups is necessary.

Peri-clitoral Epidermal Inclusion Cyst as Initial Presentation of Lichen Sclerosus in a Pediatric Patient.

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.

The efficacy of 5-aminolevulinic acid photodynamic therapy for pediatric vulvar lichen sclerosus.

BACKGROUND: Prepubertal girls are one of the vulnerable populations of vulvar lichen sclerosus (VLS), which results in a decreased quality of life and increases risk of vulvar cancer. But the therapeutic effects of traditional topical remedies are unsatisfactory in some pediatric patients. 5-Aminolevulinic acid photodynamic therapy (ALA-PDT) is an effective treatment for refractory VLS patients, but no study has been conducted in child patients. METHODS: The patients included in this study underwent three sessions of ALA-PDT at 2-week intervals. All patients were evaluated for objective clinical appearances and subjective symptoms quantitatively. Statistical analysis comparing parameters at baseline and after three-time ALA-PDT was performed. RESULTS: A total of seven VLS girl patients were included in this study. Both primary objective appearances (lesion size and depigmentation) and subjective symptoms (itching and burning pain) were improved remarkably after the third treatment. Besides, adverse effects, mainly as pain and post-treatment edema, were mild and could be tolerated. CONCLUSIONS: ALA-PDT is an effective and safe therapeutic option for VLS girl patients. Compared with adult patients, the symptoms resolved more quickly in child patients.

Vulvar Lichen Sclerosus: Navigating Sex Hormone Dynamics and Pioneering Personalized Treatment Paradigm.

Vulvar lichen sclerosus (VLS) is a frequently overlooked inflammatory disorder affecting the skin and mucous membranes of the vulva. With a propensity for atrophy, severe scarring, functional impairment, and malignant evolution, VLS is a disease that recurs frequently; early diagnosis, rapid treatment, and ongoing patient follow-up are essential. Potent topical corticosteroids (TCSs) are now widely recognized as the most effective treatment for achieving remission in VLS, but considering the potential complications of long-term treatment with potent TCSs, understanding the evolution of VLS during puberty becomes particularly crucial in determining the necessity for aggressive or more conservative therapeutic interventions. Emerging treatments, including PRP (platelet-rich plasma), stem cell therapy, and energy-based lasers like fractional CO2 and Nd-YAG, are being investigated to identify more effective VLS treatments than ultrapotent topical corticosteroids. However, more research is needed to assess the efficacy and safety of these new medicines. Topical clobetasol 0.05% ointment daily for 4-12 weeks is the gold standard for treating VLS. This article is a narrative review of the English-language medical literature from 2017 to November 2023, following three main sections concerning VLS: studies of the evolution amid pubertal hormonal changes; studies of the outcomes of personalized conventional therapies; and studies addressing the spectrum of innovative modalities for VLS.

Monitoring adherence to vulvar lichen sclerosus treatment - a prospective study.

Background: Vulvar lichen sclerosus treatment consists of topical corticosteroids followed by maintenance therapy. Self-reported adherence to topical corticosteroids in vulvar lichen sclerosus is approximately 66-70.4% and adherence to chronic topical medications is poor.Objective: To measure treatment adherence for vulvar lichen sclerosus.Methods: Adults with vulvar lichen sclerosus who were receiving or who were candidates to receive treatment with topical clobetasol propionate 0.05% ointment twice daily received medication tubes equipped with adherence monitors capturing the time and amount of dose dispensed. After 2 months, monitors were returned, and patients were surveyed regarding their adherence.Results: Ten patients participated for a median (range) of 8.5 (7-11) weeks. Eight (80%) and 7 (70%) caps captured medication timing and dosing events, respectively. Median (interquartile range) adherence was 65% (42-77) and median (interquartile range) medication dispensed per use was 0.15 (0.14 - 0.5) grams. Of the 8 patients using active adherence monitors, 2 did not clinically improve; adherence rates and mean quantity dispensed for these two patients were 31% and 0.13 grams, and 9% and 0.74 grams, respectively.Conclusion: Poor adherence to both twice daily application and prescribed medication quantity occurred frequently. Factors related to self-reported non-adherence included perceived greater efficacy, inconvenience, and time-constraints. Patient adherence to recommended treatment and clinical outcomes are areas for improvement in patients with vulvar lichen sclerosus.

WNL we never looked: vulvar carcinoma incidence after screening cutoff.

The incidence of vulvar carcinoma increases with age, though elderly women receive less aggressive cancer therapies and fewer strategies aimed at cancer prevention. Furthermore, elderly women dual enrolled in Medicaid-Medicare experience poor survival rates for vulvar carcinoma. Herein, we provide recommendations for the prevention of and guidelines for the multidisciplinary care of vulvar carcinoma. Prevention of vulvar carcinoma can be categorized into primary, secondary, and tertiary prevention. Primary prevention consists of vaccination, secondary prevention consists of screening, and tertiary prevention is aimed at the management of premalignant and early-stage lesions.

Searching for a "Window of Opportunity" in the Treatment of Vulvar Lichen Sclerosus: Evidence for Therapeutic Benefits of an Early Corticosteroid Treatment.

INTRODUCTION: Vulvar lichen sclerosus (VLS) is characterized by progressive anatomical changes which become increasingly severe and irreversible. The objective of this study was to investigate if a "window of opportunity" exists in VLS, i.e., to assess if an early treatment may prevent disease progression and facilitate clearance of symptoms and/or signs. METHODS: This retrospective, cohort study included VLS patients treated for the first time with a topical corticosteroid, namely with mometasone furoate 0.1% ointment, for 12 weeks (2016-2021). Scoring of subjective symptoms (global subjective score, GSS, and dyspareunia) and clinical features (global objective score [GOS] and sclerosis-scarring-atrophy) was performed at baseline (T0) and at the control visit (T1). We assessed if the achievement of clearance in GSS, GOS, sclerosis-scarring-atrophy, or dyspareunia depended on the time elapsed between VLS onset and treatment initiation. RESULTS: Among the 168 patients (59.2 ± 13.2 years) included, the median time between VLS onset and first treatment was 14.0 months. At T1, 48.8% of patients achieved clearance of GSS, 28% of GOS and 11.9% of both GSS and GOS, 57.9% of dyspareunia, and 19.2% of sclerosis-scarring-atrophy. The logistic regression model showed that each 10-month increase in treatment initiation adversely affected the clearance of GSS while starting treatment within 6 months of disease onset was significantly associated with clearance of GOS and sclerosis-scarring-atrophy. CONCLUSION: Early treatment is crucial in determining a complete healing of VLS-related symptoms and signs, especially of tissue sclerosis-scarring-atrophy, which appear poorly responsive, or even unresponsive, after the earliest stages of the disease. Thus our findings provide evidence for a "window of opportunity" in VLS treatment.

Combination of high-frequency electrocautery therapy and ALA-PDT in hyperkeratotic vulvar lichen sclerosus: Series of seven cases.

SIGNIFICANCE: ALA-PDT effectively treats Vulvar lichen sclerosus et atrophicus (VLSA), but it requires multiple repetitions for satisfactory results. To enhance efficacy, we employed a combination of high-frequency electrocautery therapy and ALA-PDT in treating seven VLSA patients. APPROACH: Lesions and leukoplakia in the seven women with VLSA were removed using a high-frequency generator. PDT was administered after wound healing, and it was repeated six times. Follow-up assessments were carried out at 1, 3, and 6 months to evaluate the severity of pruritus and investigate lesion repigmentation. RESULTS: Following the combined therapy, the disappearance of pruritus was observed in all patients, and normal color and thickness were restored to their skin. Two patients reported mild pruritus with a score of 2 one month after treatment, which persisted until the 6-month follow-up, while the remaining patients remained free from pruritus. No recurrence of skin lesions was observed in any of the patients. CONCLUSIONS: The combined therapy for the treatment of VLSA is found to be convenient, effective, and easily promotable.

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.

Quality of life and burden of disease of vulvar lichen sclerosus: A single-center retrospective study in China.

OBJECTIVE: This study examined the quality of life and burden of disease of vulvar lichen sclerosus (VLS) patients in three states of menstruation to better understand VLS. METHODS: A total of 607 VLS patients were enrolled into this retrospective study. According to the ages of onset, menarche and menopause, the patients were divided into three groups: prepubertal group (n = 96), reproductive group (n = 400) and postmenopausal group (n = 111). Data were collected by direct interview and clinical examination. RESULTS: A total of 93% of patients had itching, with a median numerical rating scale score of five. In the prepubertal group, the median score was three. Nocturnal itching occurred in 49.6% patients. Nearly half of the patients (45.9%) thought the itching affected their sleep. However, this ratio was very different in the prepubertal group (20.7%). Some patients (12.8%) cleaned their vulva more than seven times per week. Only 17.2% of patients experienced no effect on their sex life. The median dermatology life quality index score in all patients was six, but it was only three in the prepubertal group. The median number of hospital visits was two times, and the number of clinic visits was three times. Previous expenses, in median, were 2000 RMB. For 84.3% patients, the gynecologic clinic was their first choice. CONCLUSION: VLS places great physical, mental and economic burdens on patients. Patients in the prepubertal group had milder symptoms and dermatology life quality index score. VLS should arouse the attention of patients and specialists.