Materiały źródłowe

• #1 Lichen Sclerosus | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/24256

  LS is relatively rare. Although reports indicate that it affects between 1 in 1000 and 1 in 300 individuals in the general population, the exact prevalence is unknown. […] It still seems unknown and underestimated because many patients are asymptomatic, and it is frequently misdiagnosed. Both sexes are affected, but it is more common in women than men with a female to male ratio varies from 1:1 to 10:1. There is no racial predilection. It occurs at all ages. Moreover, its incidence in women has two peaks, the first one occurs between eight and thirteen-year-old girls and the second one is during the fifth and the sixth decades. The mean age at diagnosis ranges between 52 and 60 years. LS occurs in the genital skin in 85 to 98 %, and on the extragenital skin in only 15 to 20 %. […] Extragenital lichen sclerosus is uncommon during childhood. LS can involve the oral mucosa, known as oral lichen sclerosus, and has been rarely reported.

• #2 Lichen Sclerosus: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1123316-overview

  Lichen sclerosus (LS) is a chronic inflammatory dermatosis of unknown cause that most commonly affects the genitalia (vulvar and penile lichen sclerosus) but can occur at any skin site (extragenital lichen sclerosus). […] Although LS can occur in males or females of any age, it more commonly affects prepubertal or perimenopausal females or males between puberty and age 60 years. […] LS is a relatively common dermatosis, though its true prevalence has not been established and is probably underestimated. This may be because patients are distributed among different specialties (eg, gynecology, dermatology, family practice, and urology), but it may also be because one third of cases can be asymptomatic. […] The prevalence of genital LS in prepubertal girls has been reported to be 0.1% (1 in 900).

• #3 Lichen Sclerosus—Incidence and Comorbidity: A Nationwide Swedish Register Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11122656/

  Data on the incidence and comorbidity of Lichen sclerosus (LS), based on validated nationwide population-based registries, remains scarce. […] The incidence of LS in Sweden was 80.9 per 100,000 person per year, with higher incidence in females (114.4) than in males (47.2). […] This study underscores the significantly increased association of various cancers and premalignant conditions in LS patients, highlighting the critical need for efficacious treatment and diligent follow-up. […] The correlation between LS and vulvar SCC is well-established, but research into the association of LS with other cancers remains limited. […] The prevalence of comorbid conditions in Swedish LS patients has not been thoroughly examined. […] Current awareness of LS among medical professionals is inadequate, often leading to misdiagnosis, delayed treatment, and insufficient follow-up.

• #4 Lichen Sclerosus—Incidence and Comorbidity: A Nationwide Swedish Register Study

  https://www.mdpi.com/2077-0383/13/10/2761

  Lichen sclerosus (LS) represents a chronic, lymphocyte-mediated inflammatory condition that primarily affects mucocutaneous regions, with a notable predilection for anogenital sites. […] The primary objective of this study was to investigate the incidence of LS in the Swedish population and to assess the correlation of comorbid conditions among patients diagnosed with LS compared to a control group without LS, with a special emphasis on its association with various types of cancer. […] The incidence of LS in Sweden was 80.9 per 100,000 person per year, with higher incidence in females (114.4) than in males (47.2). […] The mean annual incidence peaked for male and female patients in the 70–79 age group with 62.4/100,000 for males, and with 224.8/100,000. […] Our data revealed that individuals with LS exhibited a significant increased association for various malignant and premalignant conditions studied. […] This study corroborates the known risk of vulvar and penile cancers associated with LS and introduces pivotal insights into the links of LS with prostate, breast, testicular, and bladder cancers, among other conditions.

• #5 Lichen sclerosus: Causes, Diagnosis, and Images – DermNet

  https://dermnetnz.org/topics/lichen-sclerosus

  Lichen sclerosus can start at any age, although it is most often diagnosed in women over 50. Pre-pubertal children can also be affected. […] Lichen sclerosus is ten times more common in women than in men. […] 15% of patients know of a family member with lichen sclerosus. […] People with lichen sclerosus often have a personal or family history of another autoimmune disease such as thyroid disease (about 20% of patients), pernicious anaemia, or alopecia areata. […] Lichen sclerosus of anogenital sites is associated with an increased risk of vulval, penile or anal cancer (squamous cell carcinoma, SCC). […] Cancer is estimated to affect up to 5% of patients with vulval lichen sclerosus. […] Long-term follow-up is recommended to monitor the disease, optimise treatment and ensure early diagnosis of cancer.

• #6 Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

  https://www.ijbs.com/v15p1429.htm

  Most recently, a report on the prevalence of LS in Botswana amassed to 0.2%, falling within this range. […] In contrast, however, the European Dermatology Forum classified LS as a common disease and the prevalence of LS was observed to be 1.67% in a general gynecology clinic in the United States. […] Extrapolated data from the Oxford clinic approximates 150 to 200 women per million population seek medical treatment for LS each year and a more recent report suggests the incidence of LS is rising, from 7.4 to 14.6 women per 100,000 between 1991 and 2011 in the Netherlands. […] Taken together from the literature, LS is reported to be more prevalent in women (3%) than men (0.07%), and commonly affects more adults (1.5%) than children (0.3%). […] The sex ratio among women and men vary widely in the literature, from 10:1, 6:1, to 3:1.

• #6 Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

  https://www.ijbs.com/v15p1429.htm

  Lichen sclerosus (LS) exhibits a bimodal distribution in incidence, with the first peak occurring before puberty in pre-adolescent teens and the second peak arising in middle to late adulthood, specifically after menopause for women and between 30-50 years of age in men. […] In the literature, however, there is a lack of large-scale epidemiological studies that consider both the sex and age of onset. Therefore, the true incidence of LS is unknown. The estimated prevalence is most likely underestimated, as studies are limited to the confines of case-control studies, which mainly focuses on one demographic (i.e. men, women, boys, or girls) few studies include a sample of all four age and sex groups. […] Nevertheless, the classically cited prevalence approximates 0.1%-0.3% in a general hospital setting, and LS is therefore recognized as a rare condition by the Genetic and Rare Diseases Information Center (GARD) of the National Institutes of Health (NIH) and the National Organization for Rare Disorders (NORD).

• #7 Pediatric Vulvar Lichen Sclerosus—A Review of the Literature

  https://www.mdpi.com/1660-4601/18/13/7153

  VLS can occur at any age or in any sex, although the highest values can be observed in women aged 40–60 years old, and in pre-pubertal girls. There is a clear peak of incidence in girls aged four to six years old, which represents 7–15% of all vulvar lichen sclerosus cases. […] It is estimated that VLS can be observed in 1:900 of premenarchal girls. The first symptoms are usually very non-specific and misdiagnosed by non-gynecologist and non-dermatologist doctors. Some of the symptoms can spontaneously recede after the menarche, and the course of the disease can be latent. This is why the epidemiology of VLS is underestimated.

• #8 Lichen sclerosus – Wikipedia

  https://en.wikipedia.org/wiki/Lichen_sclerosus

  There is a bimodal age distribution in the incidence of LS in women. It occurs in females with an average age of diagnosis of 7.6 years in girls and 60 years old in women. The average age of diagnosis in boys is from 9 to 11 years old.[69] […] In men, the most common age of incidence is 21 to 30.[70]

• #9

  https://link.springer.com/article/10.1007/s40257-012-0006-4

  LS is a chronic, inflammatory skin disease with a distinct predilection for the anogenital region. Only 6 % of LS are isolated extragenital lesions. The exact prevalence of LS is difficult to ascertain and probably underestimated, since patients with LS may present to various clinical specialities, physicians do not always recognize LS, and patients may not report symptoms because of embarrassment or because they are asymptomatic. However, in 1971, Wallace calculated a prevalence of 0.10.3 % of all patients referred to a community-based dermatology department. Both female and male patients are affected and it occurs in children, and in adults. The disease may occur for the first time at any age. There is a typical bimodal onset in prepubertal children and in postmenopausal women, respectively, and in men in their fourth decade. Affected female patients clearly outnumber male patients.

• #10 Lichen Sclerosus: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1123316-overview

  The prevalence of vulvar LS in elderly nursing home women in one study was found to be 3% (1 in 30). […] A Danish study covering the period from 1997 to 2022 found that the incidence of biopsy-confirmed vulvar LS rose from 5.0 per 100,000 person-years at the beginning of the period to 35.7 per 100,000 person-years at the end. […] The prevalence of male LS is thought to be influenced by the circumcision rate. […] The prevalence of extragenital LS is unclear. A study of foreskins submitted after therapeutic circumcision for phimosis revealed many cases of unrecognized LS. Extragenital LS is much less common than genital LS and is rare in children. […] Vulvar LS can occur at any age, and incidence increases with age. In females, the two peaks of onset are during the prepubertal period and during the perimenopausal/postmenopausal period. […] The female-to-male ratio has ranged from 3:1 to 10:1. […] Neither genital nor extragenital LS has been shown to have any racial predilection.

• #11 Anogenital Lichen Sclerosus: Clinical Considerations and Management – Journal of the Turkish Academy of Dermatology

  https://www.jtad.org/articles/anogenital-lichen-sclerosus-clinical-considerations-and-management/doi/jtad.galenos.2022.64936

  Lichen sclerosus (LS) is a chronic inflammatory disease, which commonly involves anogenital region. […] In general gynecology practice, vulvar LS prevalence of 1.7% was reported. […] Examination of 96 elderly women who were nursing home residents revealed that 3% of them had genital LS. […] An increasing incidence of premenarchal genital LS was observed, with an estimated prevalence of 1 in 900. […] Male genital LS is probably an under-recognized and under-reported condition. […] A United States based electronic medical record revealed an incidence of 1.4 male genital LS cases per 100,000 visits. […] Epidemiology of the disease may vary between countries as the condition has a propensity to occur in uncircumcised men. […] Males with a history of neonatal circumcision are unequivocally spared from the disease.

• #12 Association of Autoimmune Diseases with Lichen Sclerosus in 532 Male and Female Patients | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-1512

  Lichen sclerosus is a relatively common chronic inflammatory skin disease that predominantly affects the anogenital area. […] Accumulating evidence indicates that lichen sclerosus in women may be associated with other autoimmune disease, whereas this association seems to lack in male patients. […] Of the whole LS cohort, 82 (15.4%) had at least one autoimmune disease. Female patients had significantly more often at least one autoimmune disease compared with male patients (18.9% vs. 5.1%, odds ratio (OR) 4.3, 95% confidence interval (CI) 1.99.6, p 0.0001). […] Moreover, autoimmune thyroid diseases were significantly more frequent in women compared with men (15.2% vs. 3.8%, OR 4.7, CI 1.811.9, p = 0.0002). […] This study has clearly shown that female and male patients differ significantly in their percentages of autoimmune diseases, especially autoimmune thyroid disease, as well as autoantibody levels.

• #13 Lichen sclerosus – almostadoctor

  https://almostadoctor.co.uk/encyclopedia/lichen-sclerosus

  Lichen sclerosus (LS) is a relatively common chronic, autoimmune skin disorder involving the anogenital region, particularly in women. […] It can occur at any age, but typically affects women over the age of 50, and to a lesser extent, children of both genders pre-puberty. […] About 10x more common in women […] Affects about 3% of women and about 0.05% of men […] Usually aged 50 […] Probably related to post-menopausal reduction in oestrogen. […] Sometimes seen in pre-pubescent children. […] Associated with other autoimmune skin conditions, e.g. psoriasis, lichen planus, vitiligo. […] 20% of patients have another autoimmune disease.

• #14 Patient education: Vulvar lichen sclerosus (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/vulvar-lichen-sclerosus-beyond-the-basics

  Lichen sclerosus (LS) is a skin disorder that causes the skin to become thin, whitened, and wrinkled and can cause itching or pain. […] Estimates for vulvar LS vary from 1 in 30 older adult females seen in general gynecology offices to 1 in 300 to 1000 patients referred to dermatologists. […] Adults with vulvar lichen sclerosus (LS) are at a slightly increased risk for developing squamous cell skin cancer of the vulva. […] Diagnosing vulvar LS early, treating it effectively, and biopsying any abnormal areas may help to reduce the risk of developing or missing a diagnosis of skin cancer. A once-yearly examination of the skin of the vulva is recommended, and women should examine themselves regularly (for example, monthly) for lumps or sores that do not heal. […] There is evidence that suggests that good control of vulvar LS may reduce the risk of vulval cancer.

• #15 Vulvar Lichen Sclerosus and Vulvar Cancer: What You Need to Know – LSSN

  https://lssupportnetwork.org/vulvar-lichen-sclerosus-and-vulvar-cancer-what-you-need-to-know/

  Vulvar lichen sclerosus is associated with an increased risk of developing vulvar cancer. Importantly, the risk is low, only slightly higher than population risk. The general population risk is approximately 0.5% (Viera-Baptista et al., 2022). In the medical literature, you may see anything from 2-6%, give or take (Lee and Fischer, 2018; Viera-Baptista et al., 2022). […] The review showed people with vulvar lichen sclerosus have a higher chance of developing vulvar cancer, especially with certain conditions like dVIN and as they get older. In people developing vulvar cancer for the first time, the risk was 2.2%; the risk for people who previously had or currently have vulvar cancer is 4% (ibid). […] Therefore, they note, it’s important for people with VLS to receive treatment and ongoing check-ups throughout their lives to monitor and manage any potential risks of developing vulvar cancer.

• #16 Lichen sclerosus (syn. lichen sclerosus et atrophicus)

  https://www.pcds.org.uk/clinical-guidance/lichen-sclerosis

  Lichen sclerosus (LS) is an uncommon condition characterised by white sclerotic macules and patches, commonly affecting the genital and perianal skin, although it can affect non-genital skin separately or together. LS can cause permanent scarring of the genitalia, and in approximately 5% of cases is associated with squamous cell carcinoma, mainly of the female genitalia. […] The suspected prevalence varies between 0.1% and 3% for children and post-menopausal women respectively, and 0.07% for males. […] LS is six times more common in women than in men. […] Although LS is most often seen in women around and after the time of the menopause, it can affect any age group – up to 15% of cases are in children with the majority affecting the vulva. […] LS is associated with pre-malignant change and squamous cell carcinoma (SCC), which arises in 5% of patients. SCC presents mainly as a nodule, tending to arise on sclerotic tissue, especially the anterior vulva.

• #17

  https://aacr.figshare.com/collections/Data_from_Lichen_Sclerosus_Incidence_and_Risk_of_Vulvar_Squamous_Cell_Carcinoma/6516094

  Between 1991 and 2011, the incidence rate of lichen sclerosus increased from 7.4 to 14.6 per 100,000 woman-years. […] The cumulative VSCC incidence was 6.7%. […] This historical cohort showed a nearly 100% increase in incidence of lichen sclerosus between 1991 and 2011. […] The incidence of lichen sclerosus is rising and special attention is needed in particular in women with concurrent VIN because of their high risk of cancer.

• #18 Lichen sclerosus: Review of a vulvar dystrophy with malignant potential

  https://www.contemporaryobgyn.net/view/lichen-sclerosus-review-of-a-vulvar-dystrophy-with-malignant-potential

  This acquired chronic dermatosis has a 6- to 10-fold predilection for women, so it is especially relevant to a gynecologists practice. […] Lichen sclerosus is significantly debilitating, with vulvar pain and pruritus compounded by scarring, stenosis, and phimosis, which contribute to dyspareunia and other functional impairments. The potential for malignant transformation in the chronically inflamed tissues is an additional sequala, which may benefit from therapy and requires long-term informed clinical follow-up. […] The precise incidence of lichen sclerosus is uncertain, as many cases are asymptomatic or remain undiagnosed. The US gynecologic population has a 1.7% to 3% prevalence of biopsy proven lichen sclerosus. […] Lichen sclerosus has been associated with squamous cell carcinoma of the vulva at a cumulative rate of 6.7%.

• #19 Lichen Sclerosus in Men — DermNet

  https://dermnetnz.org/topics/lichen-sclerosus-in-men

  Male genital lichen sclerosus seems poorly recognised; reported figures likely underestimate the prevalence of this disease. […] Anogenital lichen sclerosus is approximately 10 times more common in women than men. […] One-third to one-half of all penile cancer is associated with lichen sclerosus with an incidence rate ranging from 0-12.5%; this range probably reflects the timeliness of diagnosis and the effectiveness of treatment. […] The diagnosis of lichen sclerosus in men is usually made clinically by eliciting cardinal symptoms (eg, dyspareunia and urinary dribbling) and signs; dermoscopy can provide further clues. […] About 60-70% of men with genital lichen sclerosus can be cured long-term by medical treatment.

• #20 Pulsenotes | Lichen sclerosus

  https://app.pulsenotes.com/specialities/gynaecology/notes/lichen-sclerosus

  Lichen sclerosus is much more common in woman. […] Lichen sclerosus is up to ten times more common in women and has two peaks of onset in prepubertal girls and peri- or post-menopausal woman. It may co-exist with other skin conditions and is thought to occur in 1 in 30 older woman. […] Women with lichen sclerosus should have the area of skin examined at least annually and non-resolving lesions biopsied, particularly hyperkeratotic areas. […] In men, there is an increased risk of penile squamous cell carcinoma, but this is rare (~1%). Extragenital sites of lichen sclerosus do not seem to predispose to cancer.

• #21 Vulvar Lichen Sclerosus and Vulvar Cancer: What You Need to Know – LSSN

  https://lssupportnetwork.org/vulvar-lichen-sclerosus-and-vulvar-cancer-what-you-need-to-know/

  This evidence suggests steroids may reduce the risk of vulvar cancer. To date, only steroids as a treatment option seem to support this. […] Follow-up appointments are important to track your VLS’s progress and address any concerns you may have. They should also include a clinical examination, where your healthcare provider examines the skin of your vulva. During this exam, they will also check for any potential signs of cancer. If they suspect anything at all, they will do a biopsy. […] In sum, we reviewed what vulvar cancer is and the association between vulvar lichen sclerosus and vulvar cancer. We noted the scientific literature holds a higher than average risk for developing vulvar cancer with VLS and that the risk can be reduced by proper and consistent application of topical corticosteroids. Finally, it is important to attend follow-up appointments and do a monthly vulva check to be proactive in your health and wellness.

• #22 Lichen Sclerosus – Vulvovaginal Disorders

  https://vulvovaginaldisorders.org/atlas_topic/lichen-sclerosus/

  A retrospective chart review of 129 adult patients with lichen sclerosus who had been under surveillance for a minimum of three years was performed. Squamous cell carcinoma was seen only in non-compliant patients, as suggested in two previous studies. A 2015 study of 976 women histologically or clinically designated as having LS who had a one month minimum follow-up were selected. The median age at diagnosis was 60 (8-91) years. The median follow-up was 211-339 months. 34 patients developed neoplasms (incidence risk: 3.5%). The probability of neoplasia occurrence at 24, 60, 120, 180, 240, and 300 months was 1.2%, 3.0%, 7.1%, 11.0%, 21.6% and 36.8% respectively. The authors concluded that the higher probability of malignant transformation in the longer periods suggests careful and lifelong follow-up in all LS patients.

• #22 Lichen Sclerosus – Vulvovaginal Disorders

  https://vulvovaginaldisorders.org/atlas_topic/lichen-sclerosus/

  Lichen sclerosus has been identified in patients of all ages including those in the first years of life. It is more common in women than in men. LS has two peak ages of presentation. The first occurs in prepubertal girls, and the other in postmenopausal women. Lichen sclerosus may improve with puberty but this is not always the case, as shown in at least one study. It has been pointed out that milder, more subtle symptoms in pre-menopausal women may distort the assumption of a bimodal presentation. In one study of patients with biopsy-proven LS, the mean age at diagnosis was 32 with onset of symptoms at age 27. Long-term follow up is important regardless of age. […] The prevalence is difficult to determine since patients with this disease may present to pediatricians, dermatologists, gynecologists, and urologists, and many cases go unreported. Estimates range from 1 in 30 elderly women, to 1 in 59 women in a general gynecology practice, to 1 in 300 to 1000 patients referred to dermatologists.

• #23 Lichen sclerosus: An overview – Medical Independent

  https://www.medicalindependent.ie/clinical-news/lichen-sclerosus-an-overview/

  Lichen sclerosus can affect individuals of all ages, with increasing incidence with age. In females, two peaks of onset are during prepubertal and perimenopausal/postmenopausal ages. The exact prevalence in the general population is unknown, but estimates suggest a range between one-in-300 to one-in-1,000 individuals. Prevalence may be underestimated because many patients are asymptomatic, and it is frequently misdiagnosed. It is more common in women than men, with a female-to-male ratio varying from 1:1 to 10:1. […] A genetic predisposition, based on family clustering, is apparent. Of patients with vulvar lichen sclerosus, 8-to-39 per cent report a family history of the condition, while only 1 per cent of male genital lichen sclerosus patients have a family history. […] Given the potential for malignant transformation to SCC, patients with anogenital lichen sclerosus should receive long-term follow-up. […] Although the risk is relatively low, individuals with lichen sclerosus have a slightly increased risk of developing SCC in the affected skin. Regular monitoring and follow-up are essential to detect any signs of malignancy promptly.

• #24 Clinical Profile of Vulvar Lichen Sclerosuslogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na52513/2020/11/13/clinical-profile-vulvar-lichen-sclerosus

  Lichen sclerosus (LS) is a chronic, inflammatory dermatologic condition most often affecting the anogenital region, although it can occur on any skin surface. While both men and women can be affected, the disorder is much more common in women, accounting for up to 10% of presenting complaints at gynecology offices. The prevalence of lichen sclerosus is unclear, as the condition is frequently underdiagnosed and underreported (the latter because of asymptomatic presentations). […] Depending on symptom severity, patients return for evaluation in 1, 3, or 6 months and should then be followed for long-term surveillance every 6 to 12 months, particularly given the increased risk for squamous cell carcinoma of the vulva (2%–5%) in patients with LS. […] It is important to emphasize to patients that vulvar LS is treatable but not curable; once symptoms are controlled, maintenance and surveillance is important to prevent flares and progression of disease and to reduce cancer risk.

• #25 Lichen sclerosus: Review of a vulvar dystrophy with malignant potential

  https://www.contemporaryobgyn.net/view/lichen-sclerosus-review-of-a-vulvar-dystrophy-with-malignant-potential

  The risk increases with age and duration of disease; therefore, lichen sclerosus warrants lifelong surveillance. […] Lichen sclerosus is a debilitating and premalignant vulvar condition that benefits from long-term follow up and maintenance steroid therapy. Long-term annual or semi-annual evaluation even with patients treated to remission is prudent due to the risk of recurrence and the risk of malignancy. […] Biopsy should be promptly considered for patients unresponsive to corticosteroid therapy, suspected vulvar intraepithelial neoplasia, or atypical clinical presentations.

• #26 Comorbidity in patients with Lichen sclerosus: a retrospective cohort study | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-023-01335-9

  Lichen sclerosus (LS) is a chronic lymphocyte mediated inflammatory mucocutaneous disease of unknown aetiology with a predilection for the anogenital region, and affecting both sexes. […] The cumulative incidence of LS for the entire population over a 20-year period was 1.54% (15.4 per 1000 people). The cumulative incidences over a 20-year period for females and males were 2.13% and 0.97%, respectively. […] Due to the lack of large epidemiological studies, it is not possible to determine the true incidence of LS. […] LS has an estimated prevalence of 1:60 to 1:1,000 in adults and children in the United States but since LS can be asymptomatic and is an under-recognized disease, these prevalences are probably underestimated. […] A Dutch study found that the incidence of vulvar LS had increased from 7.4 per 100,000 woman-years in 1991 to 14.6 per 100,000 woman-years in 2011.

• #27 Frontiers | Lichen sclerosus: The 2023 update

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2023.1106318/full

  Lichen sclerosus (LS) is an underdiagnosed inflammatory mucocutaneous condition affecting the anogenital areas. Postmenopausal women are predominantly affected and, to a lesser extent, men, prepubertal children, and adolescents. […] A bimodal peak incidence of LS in premenarchal girls and in menopausal women has been outlined. However, premenopausal women could experience LS with mild symptoms, delaying the diagnosis for several years and underestimating the real incidence in this age group. […] The exact prevalence and incidence of LS are underestimated. On the one hand, one third of the cases are asymptomatic, while, on the other hand, LS is frequently misdiagnosed or unrecognized. […] The estimated incidence of LS in both sexes is 0.1 to 0.3%. […] The incidence of pediatric LS is estimated at 0.04 to 0.06%, with a female-to-male ratio of 1:1.7. […] Other forms of LS are rare. It is estimated that eLS comprises only 15–20% of LS patients.

• #28 Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

  https://www.ijbs.com/v15p1429.htm

  As such, the incidence is an underestimate given various additional compounding factors, such as under-reporting due to physician under-recognition and misdiagnosis, patient trepidation in seeking treatment (as LS may be easily mistaken for sexual abuse in premenarchal girls, and therefore may heighten hesitancy), LS management under different specialties, and asymptomatic cases that go unnoticed.

• #29 Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

  https://www.ijbs.com/v15p1429.htm

  In children, the sex ratio is reversed, as there is a prevalence of 0.5% in boys and 0.11% in girls. […] This reversal in childhood prevalence may be due to a detection bias, as boys are often admitted to a clinical setting for LS-associated phimosis, whereas girls may be asymptomatic in early LS and develop characteristic lesions later in adulthood. […] Additionally, the bimodal prevalence observed in females may also be skewed. […] Given that changing levels of estrogen is associated with changes in skin hydration, collagen content, and glycosaminoglycan concentrations, estrogen deficiency may therefore directly weaken the structural integrity of vulvar skin. […] Such changes may lead to worsening symptoms, causing pre-pubertal girls and menopausal women to more likely seek treatment than otherwise well estrogenized female age groups.

• #30 Epidemiologie van lichen sclerosus – Richtlijn – Richtlijnendatabase

  https://richtlijnendatabase.nl/richtlijn/lichen_sclerosus/epidemiologie_van_lichen_sclerosus.html

  Epidemiologie van lichen sclerosus […] De exacte incidentie i prevalentie van lichen sclerosus is onbekend. In een aantal studies lijkt het een veel voorkomende aandoening. Mede dankzij patintenverenigingen, informatie op internet en het aantal vulvapoliklinieken in Nederland, neemt de aandacht voor lichen sclerosus toe. Er is echter nog steeds sprake van diagnostic delay. […] De geschatte prevalentie loopt uiteen van 1:60 tot 1:300 – 1:1000 afhankelijk van de populatie. […] Veel gegevens zijn afkomstig van klinieken voor vulvapathologie. Vaak worden dan berekeningen gemaakt op basis van verwijzingen i adherentie. Dat is echter een zeer onbetrouwbare manier om de prevalentie in de algemene bevolking te schatten. Lichen sclerosus is de meest gestelde diagnose op een vulvapolikliniek: bij ongeveer een derde van de bezoekers blijkt sprake te zijn van lichen sclerosus. […] Soms wordt de diagnose ook niet gesteld door onbekendheid met het ziektebeeld. In verband met referral bias worden er verschillende man-vrouw ratios genoemd bij het voorkomen van lichen sclerosus. De grootste groep wordt waarschijnlijk gevormd door postmenopauzale vrouwen, echter betrouwbare prevalentiecijfers voor beide seksen ontbreken. […] In principe kan lichen sclerosus op elke leeftijd voorkomen, ook bij kinderen. Lichen sclerosus komt voor bij alle etnische groepen.

• #31 Lichen Sclerosus | 5-Minute Clinical Consult

  https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/816822/1.2/Lichen_Sclerosus

  LS is a rare entity. Occurs at all ages and in both genders, but a higher incidence is present in postmenopausal women (49% of cases are in women 50 years). Pediatric disease makes up 715% of all cases (1). The female to male ratio is between 3:1 and 10:1. It is more common in white patients than in those who are Hispanic or black. […] The exact prevalence is unknown but is estimated to be between 0.1% and 0.3% among patients referred to a community-based dermatology department and 1.7% of patients in a general gynecology practice (1).

• #32 393763 | Stanford Health Care

  https://stanfordhealthcare.org/v2/publications/393/393763.html

  Lichen sclerosus (LS) is a rare, chronic, inflammatory disease of the skin that primarily affects postmenopausal women but may occur in men and children as well. Approximately 7% to 15% of cases are believed to occur in children. […] The epidemiologic data for LS have been limited and treatment options are not well studied, particularly in children. We reviewed new developments available in the current literature on the epidemiology and management of LS for children.

• #33

  https://turkjpediatr.org/article/view/278

  Lichen sclerosus (LS) is a chronic disease of the skin, for which the pathogenesis is not known. […] In our study, the most common LS type was genital type (60%). There was extragenital involvement in 6 patients (40%). Extragenital involvement was the most common on the trunk. Diagnosis, treatment and follow-up during childhood is highly important to prevent any possible future anatomical or psychological damage and genital malignancies.

• #34 Comorbidity in patients with Lichen sclerosus: a retrospective cohort study | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-023-01335-9

  The association between LS and vulvar SCC has previously been studied and is well established; however, there are only a few studies investigating whether an association exists between LS and other types of cancer. […] The comorbidity in patients with LS has not been analyzed in a Swedish population. […] Todays knowledge about LS is limited among medical professionals, sometimes resulting in wrong or delayed diagnosis, and inadequate treatment and follow-up. […] The primary aim of this study is to investigate whether the selected diagnoses are overrepresented in patients with LS compared to controls without LS. […] The current study did not find a significant association between LS and malignant tumors of the testicle or urethra, nor for thyroiditis, myasthenia gravis, multiple sclerosis or GuillainBarr syndrome. […] The outcomes of this study not only validate the well-established risk of vulvar and penile cancer development but also uncover novel insights concerning LS.

• #35 Lichen sclerosus – VALINTERMED treatment in Valencia

  https://valintermed.com/en/medlibrary/sclerotic-leech/

  The epidemiology of lichen sclerosus remains the subject of active research, but there is already data on its prevalence. According to statistics, the incidence of this disease ranges from 0.1% to 0.6% among the population, and it is most often observed in women aged 40 to 60 years. […] The peak incidence is observed during menopause, which may indicate the role of hormonal changes in the pathogenesis of this disease. […] Monitoring the condition of patients with lichen sclerosus requires periodic control over the course of the disease, assessment of symptoms and analysis of possible complications. It is important to conduct regular examinations to promptly identify possible changes in the skin condition and introduce the necessary corrections to the therapy.

• #36 Lichen sclerosus (syn. lichen sclerosus et atrophicus)

  https://www.pcds.org.uk/clinical-guidance/lichen-sclerosis

  Long-term follow-up in Secondary Care is appropriate for patients with genital LS associated with troublesome symptoms, localised skin thickening (associated with a higher risk of malignant change), or VIN. […] Should be reviewed annually except in well-counselled patients who control their symptoms well.

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