Risk Factors for Lichen Sclerosus: A Case-Control Study of 43,000 Finnish Women.

OBJECTIVES: Lichen sclerosus (LS) is an inflammatory skin disease probably arising from an interplay of genetics, local irritation, and autoimmune processes. We identified potential risk factors for the disease using data from nationwide Finnish registries. METHODS: We identified all women diagnosed with LS within specialized health care during 1998-2016 (n = 10,692) and selected 3 age-matched population control women for each case. We calculated odds ratios (ORs) for possible risk factors using conditional logistic regression. RESULTS: Dermatological autoimmune conditions were strongly associated with LS (OR = 15.1, 95% confidence interval [CI] = 13.6-16.7 for morphea; OR = 10.3, 95% CI = 5.02-19.0 for lichen planus; OR = 6.86, 95% CI = 5.65-8.33 for alopecia; OR = 2.20, 95% CI = 1.88-2.56 for vitiligo). A diagnosis of Crohn or celiac disease increased the odds of LS (OR = 1.80, 95% CI = 1.71-1.89; OR = 1.49, 95% CI = 1.28-1.73, respectively) as did urge and stress incontinence (OR = 1.79, 95% CI = 1.71-1.87; OR = 1.28, 95% CI = 1.22-1.35, respectively).The odds of LS were lower in women after a diagnosis of type 1 diabetes (OR = 0.43, 95% CI = 0.41-0.45), coronary artery disease (OR = 0.41, 95% CI = 0.38-0.43), and rheumatoid arthritis (OR = 0.38, 95% CI = 0.36-0.41).Parous women had higher odds of LS (OR = 1.11, 95% CI = 1.04-1.17) than nulliparous ones, but increasing number of births decreased the risk. Lichen sclerosus was not associated with socioeconomic status nor the urbanicity level of the place of residence. CONCLUSIONS: Certain autoimmune diseases and urinary incontinence were associated with LS.

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.

Photodynamic therapy for male genital lichen sclerosus with urethral stricture-Case report.

Male genital lichen sclerosus (MGLSc) typically impacts the external genitalia, resulting in balanitis, erectile pain, urination symptoms, and/or urinary retention. Urethral stricture develops in up to 20 % of these patients, which is usually found in the distal part of the urethra but can, in severe instances, impact the entire urethra and cause structural changes. Patients with skin lesions limited to the foreskin and partially extending to the glans can typically be cured by circumcision, but the recurrence rate of stricture is high when the glans or urethra is extensively involved. In the following case report, we describe a 45-year-old man with a history of MGLSc for 3 years and urethral stricture for 2 years, and these conditions remained untreated after circumcision. We emphasize that treatment with 5-aminolevulinic acid-induced photodynamic therapy (ALA-PDT) may further improve outcomes in such severe cases.

The impact of limited access of photodynamic therapy during COVID-19 pandemic on patients with vulvar lichen sclerosus.

OBJECTIVES: Stressful situations have an impact on progression of lichen sclerosus. The aim of the study was to investigate fears and complaints of patients with vulvar lichen sclerosus and progression of disease at the beginning of the COVID-19 pandemic. MATERIAL AND METHODS: The analysis was based on 103 women with mean age was 64.81 ± 11.36 years divided into two groups. The first one comprised of patients with stabilization of disease during the pandemic with mean age 66.02 ± 10.01 (32-87), while the second one with progression of vulvar symptoms with mean age 63.49 ± 12.66 (25-87). RESULTS: Delay of diagnosis was reported to be a problem for respectively 25.93% of women from both groups. Fear about COVID-19 was described respectively by 57.4% and 55.1%. Stabilization of disease was more frequent in patients after photodynamic therapy before pandemic. Progression of vulvar symptoms and features were observed more in patients who did not conduct PDT previously. All patients from the second group who underwent photodynamic therapy reported disappointment because of no access for continuation of treatment. On the other hand, 81.4% (43 women) regret that have no chance for trying photodynamic therapy. CONCLUSIONS: Photodynamic therapy seems to be a method of treatment with longer survival without progression of lichen sclerosus in times of pandemics. There has been no investigation until now about concerns of patients with vulvar lichen sclerosus. Better understanding of problems connected with the pandemic can help medical personnel in taking care of patients with vulvar lichen sclerosus.

Nonsclerotic Lichen Sclerosus: Definition of a Concept and Pathologic Description.

OBJECTIVE: Nonsclerotic lichen sclerosus (NSLS) refers to the clinicopathologic situation of examination findings consistent with lichen sclerosus (LS) but without dermal sclerosis on microscopy. This review aims to describe the features of NSLS and provide a classification framework. METHODS: The International Society of the Study of Vulvovaginal Diseases tasked the Difficult Pathologic Diagnoses Committee with development of consensus documents for conditions with problematic histopathology. The Difficult Pathologic Diagnoses Committee reviewed the literature on NSLS and formulated descriptions and diagnostic criteria, then approved by the International Society of the Study of Vulvovaginal Diseases membership. RESULTS: Nonsclerotic LS may be categorized into 4 histopathologic subtypes: lichenoid dermatitis, hypertrophic lichenoid dermatitis, dermal fibrosis without acanthosis, and dermal fibrosis with acanthosis. Each has a pathologic differential diagnosis of 1 or more entities, so clinical correlation is required for final diagnosis of LS. There is no evidence to support a reliable association between absent sclerosis and clinical appearance, duration, or oncogenic potential of LS. CONCLUSIONS: Pathologists and clinicians should be familiar with the concept of NSLS and its implications for patient management. Use of the term "early LS" to indicate a lack of sclerosis in presumed LS should be abandoned. Clinical correlation is required to confirm LS from among the differential diagnoses.

[Possibilities of multiphoton microscopy for the diagnosis of the vulvar lichen sclerosus]. / Vozmozhnosti mul'tifotonnoi mikroskopii dlya diagnostiki skleroticheskogo likhena vul'vy.

BACKGROUND: Vulvar lichen sclerosus (VLS) is a chronic and recurrent dermatosis of an inflammatory nature with severe focal atrophy of the skin. Connective tissue changes are polymorphic and are still not taken into account in histological diagnostics due to the difficulty of interpreting routine histological methods. In this work, we use multiphoton microscopy (MPM) as a new imaging technique that provides detailed information about the organization of collagen fibers in the dermis based on a non-linear second harmonic generation (SHG) process. OBJECTIVE: To determine the degree of connective tissue damage in lichen sclerosus using standard histological techniques and to reveal the diagnostic capabilities of multiphoton microscopy. MATERIAL AND METHODS: We studied 42 biopsies with a histopathological diagnosis of VLS and 10 biopsies of normal vulvar skin. Histological, histochemical and immunohistochemical evaluation was used in comparison with MPM data. Quantitative analysis included the determination of the thickness, length of collagen fibers and the average intensity of the SHG signal. RESULTS: A comprehensive study of the skin showed 4 groups of changes that can be regarded as the degree of the dermis damage: initial, mild, moderate, severe. The affected area at the initial and mild degree has subtle changes, however, it is reliably identified by quantitative analysis of the SHG signal. So, the initial degree is characterized by thin (1.3-1.8 µm) long (56-69 µm) collagen fibers, with a moderate degree, the fibers are thickened (3.4-4.3 µm) and fragmented (22-37 µm). The affected area in moderate and severe cases undergoes homogenization, which is associated with the deposition of extremely thin (0.6-0.9 µm) short (16-28 µm) collagen fibers and the expression of type V collagen. CONCLUSION: Multiphoton microscopy in the second harmonic generation mode is a reliable method for identifying collagen fibers in tissues. The study made it possible to identify 4 degrees of the dermis damage in vulvar lichen sclerosus.

Pediatric vulvar lichen sclerosus: Does it resolve or does it persist after menarche?

BACKGROUND AND OBJECTIVES: Although data regarding the rates of remission and progression of the disease are still scarce, it is generally now acknowledged that pediatric vulvar lichen sclerosus (pVLS) can persist beyond puberty. Recent studies reveal that this condition may persist in as many as 75% of cases. The present study aims to answer the following query: how does pVLS evolve after menarche? METHODS: This observational retrospective study conducted on premenarchal girls diagnosed with pVLS in our institution between 1990 and 2011 describes 31 patients who returned for multidisciplinary clinical evaluation following menarche. RESULTS: The mean follow-up time was 14 years. At the post-menarche clinical examination, patients were classified as follows: 58% were still affected by VLS, 16% presented with a complete remission of disease, and 26% were completely asymptomatic although with persistent clinical signs of VLS. CONCLUSIONS: In our series, pVLS persists following menarche in the majority of patients. These findings suggest the importance of a long-term follow-up even among patients who report resolution of symptoms following menarche.

Total introital obliteration as a consequence of lichen sclerosus: a rare cause of urinary retention.

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.

Extragenital lichen sclerosus: a comprehensive review of clinical features and treatment.

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.

The Distribution of Innervation and Immune Cell Infiltration Is Different in Genital and Extragenital Variants of Lichen Sclerosus.

Lichen sclerosus (LS) is a progressive skin disease that is characterized by chronic inflammation of either genital or extragenital skin, and it disproportionately affects women. We analyzed the distribution of nerve fibers, vanilloid receptors, cell proliferation, mast cells and macrophages in genital and extragenital LS samples, as well as in healthy skin, by using immunohistochemistry. The total amount of intraepidermal nerve fibers was lower in LS samples compared to healthy controls, while the total amount of subepidermal nerve fibers and calcitonin gene-related peptide (CGRP) positive fibers was higher in genital LS samples compared to both extragenital LS and healthy controls. Cell proliferation, macrophage and mast cell density were increased in LS samples compared to healthy controls. Genital LS had a higher macrophage density compared to the extragenital variant. Mast cell distribution significantly differed between genital and extragenital LS samples, even though their total mast cell densities were similar. These findings could explain the differences between pruritic symptoms of genital and extragenital LS and provide targets for the research of novel therapeutic strategies for LS management.

The Importance of Immunological Disorders in the Pathogenesis of Lichen Sclerosus in Pediatric Patients: A Systematic Review.

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.

[Pediatric genital lichen sclerosus: a case series of 11 girls].

BACKGROUND: Genital lichen sclerosus decreases the quality of life of women; 10-15% of cases occur in prepubertal girls. METHODS: Retrospective and descriptive study on the characteristics of girls diagnosed with genital lichen sclerosus at the Hospital Universitario de Navarra (Pamplona, Spain) between 2019 and 2022. RESULTS: Eleven girls aged between 4 and 14 year-old were diagnosed. Frequently, diagnostic delays were up to two years after the appearance of the lesions; the girl with a four-year delay showed a significant vulvar architectural alteration. All cases showed the typical sclerotic lesions on the genital area, and two of them also on the back. While six patients were asymptomatic, the rest reported pruritus and/or pain. Treatment with high/very high potency topical cortico-steroids achieved a good partial response, without complete remission of the lesions. CONCLUSION: Early diagnosis of genital lichen sclerosus is key to start early treatment, avoiding ireversible genital structural alteration.