Vulvar Lichen Sclerosus

Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which may present in a prepubertal or adolescent patient. The most popular theories are its autoimmune and genetic conditioning, although theories concerning hormonal and infectious etiology have also been raised. The most common presenting symptoms of VLS is vulva pruritus, discomfort, dysuria and constipation. In physical examination, a classic "Figure 8" pattern is described, involving the labia minora, clitoral hood, and perianal region. The lesions initially are white, flat-topped papules, thin plaques, or commonly atrophic patches. Purpura is a hallmark feature of VLS. The treatment includes topical anti-inflammatory agents and long-term follow-up, as there is a high risk of recurrence and an increased risk of vulvar cancer in adult women with a history of lichen sclerosus. This article reviews vulvar lichen sclerosus in children and provides evidence-based medicine principles for treatment in the pediatric population. A systematic search of the literature shows recurrence of VLS in children. Maintenance regimens deserve further consideration.

PurposeVulvar lichen sclerosus (LS) is a chronic, progressive, autoimmune dermatologic condition that causes cutaneous changes accompanied by pruritus and pain. There remains a small population with vulvar LS refractory to topical corticosteroids. Injection of platelet-rich plasma (PRP) has been reported to have positive effects on tissue repair. The aim of this pilot study was to evaluate changes in symptom scores during and after PRP vulvar infiltration.MethodsThree PRP infiltrations were administered to 28 female postmenopausal patients with biopsy-proved LS with unsatisfactory response to steroid therapy. Change in score according to the Clinical Scoring System for Vulvar Lichen Sclerosus (CSS) was measured on six occasions over the course of a year. We used growth curve modeling to measure change over the period of the study.ResultsWomen in our study experienced a statistically significant improvement in auto-assessed symptoms of vulvar lichen sclerosus, and this improvement appears to be maintained throughout the monitoring year.ConclusionPlatelet-rich plasma may have a role in symptom relief in certain cases of patients with LS that do not respond to first-line therapy.

PurposeLichen 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.MethodsThis 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).ResultsIn 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.ConclusionsIn 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.

Vulvar Lichen Sclerosus (VLS) is a chronic inflammatory dermatosis of unknown etiology affecting the external genitalia. In pediatric patients, it can lead to significant discomfort and progressive structural changes in tissues. In recent years, there has been increasing interest in the participation of immune checkpoints and inflammatory mediators in the pathogenesis of chronic diseases, including VLS. Immune checkpoints, such as PD-1, PD-L1, CTLA-4, CD200, and CD200R, play a crucial role in modulating the immune response and may serve as potential diagnostic markers and therapeutic targets. This study aimed to evaluate the expression of PD-1, PD-L1, CTLA-4, CD200, and CD200R molecules on CD4+ T cells, CD8+ T cells, and CD19+ B lymphocytes in prepubertal girls diagnosed with VLS. Additionally, the concentrations of their soluble forms and the levels of proinflammatory cytokines, including IL-2, IL-6, and TNF-α, in serum were determined to assess their potential as diagnostic biomarkers. The study included patients with VLS (study group) and healthy children (control group). The expression of checkpoints was analyzed using flow cytometry, while the concentrations of soluble forms and cytokines were determined using the enzyme-linked immunosorbent assay (ELISA) technique. Statistical significance tests and Spearman's rank correlation analysis were used. Patients with VLS showed markedly higher serum C-reactive protein (CRP) levels compared with healthy controls (49.99 ± 6.09 mg/L vs. 2.70 ± 0.96 mg/L, p < 0.001). A significant increase in checkpoint expression was observed on lymphocyte subsets, including CD4+ T cells expressing PD-1 (4.90 ± 1.67% vs. 0.85 ± 0.56%, p < 0.001) and CTLA-4 (11.58 ± 4.70% vs. 0.96 ± 0.48%, p < 0.001), as well as CD8+ T cells expressing PD-1 (17.31 ± 4.81% vs. 0.76 ± 0.67%, p < 0.001) and CD19+ B cells expressing PD-L1 (16.10 ± 9.50% vs. 1.41 ± 0.45%, p < 0.001). Soluble checkpoint molecules were consistently elevated, for example, sPD-1 (26.69 ± 3.88 pg/mL vs. 4.40 ± 0.75 pg/mL, p < 0.001) and sCTLA-4 (43.96 ± 3.77 pg/mL vs. 5.09 ± 1.09 pg/mL, p < 0.001). Similarly, cytokine levels were significantly increased in VLS patients, including interleukin-2 (28.80 ± 6.36 pg/mL vs. 4.32 ± 1.33 pg/mL, p < 0.001), interleukin-6 (25.35 ± 7.52 pg/mL vs. 2.35 ± 0.78 pg/mL, p < 0.001), and tumor necrosis factor alpha (31.26 ± 3.10 pg/mL vs. 12.80 ± 1.30 pg/mL, p < 0.001). Correlation analyses confirmed significant positive associations between cytokine concentrations and checkpoint expression, highlighting their interdependence in VLS immunopathogenesis. The obtained results confirm an increased immunoactivation profile in children with VLS, characterized by elevated checkpoint expression and increased levels of proinflammatory cytokines. The studied parameters show potential as diagnostic and prognostic biomarkers, which may constitute the basis for the development of new diagnostic tools and targeted therapeutic strategies in VLS in pediatric patients.