Search

A healthy, 49-year-old healthy woman presented at a maxillofacial surgery department with pain and a burning sensations in the mouth. She had experienced the discomfort for a year and it had made eating difficult. Clinical examination revealed white, plaque-like lesions on the buccal mucosa and tongue that could not be scraped off. A biopsy was taken by another health professional and oral lichen planus was diagnosed; treatment with triamcinolone dental paste and later clobetasol mouthwash followed. Neither treatment was sufficiently effective. A new biopsy confirmed a diagnosis of oral lichen sclerosus. Lichen sclerosus is a mucocutaneous condition commonly affecting the anogenital region, while the oral variant presents as white plaque. The differential diagnoses consisted of oral lichen planus, oral manifestations of systemic sclerosus (scleroderma) and leukoplakia. When other kinds of corticosteroid therapy are insufficiently effective, an intralesional injection of triamcinolone acetonide might be considered, which proved to be effective in this case.

Lichen sclerosus (LS) was first described in women by a researcher in 1887. 1 It was recognized in men by another investigator in 1928. 2 Lichen sclerosus is a chronic, inflammatory lymphocytic dermatosis that occurs in anywhere from 1:30 to 1:1,000 adults. 3,4 There is a slight predominance of women, with a bimodal age distribution in pre-pubertal individuals and again in life's sixth-seventh decades. Studies have established that the majority with pre-pubertal onset continue to have adulthood disease. 5 Psychosocial implications of this disease, specifically self-image, anxiety, and sexual function, can be debilitating for patients. 6-9 As no cure has been described for lichen sclerosus. Treatment is aimed at symptomatic relief and preventing additional effacement. Unfortunately, the scarring that occurs is usually permanent. 10,11 As it is unclear whether treatment alters the theoretic risk of malignant degeneration, estimated at 4%-5%, 12,13 frequent clinical examinations are indicated. 14-17 .

Abstract: Differentiated vulvar intraepithelial neoplasia (d-VIN) is an HPV-independent precursor to vulvar squamous cell carcinoma. The histology of d-VIN lesions is difficult to differentiate from that of non-neoplastic epithelial disorders, especially lichen sclerosus (LS). The authors present a case of LS, where relying on histopathology alone could have led to misdiagnosis. The patient was a 17-year-old female patient with clinical features of vulvar dermatitis and LS for 2 years. She was counseled to apply clobetasol 0.05% to the affected area daily but reported no improvement after 6 months. A biopsy of the right labia majora revealed histologic findings typical of d-VIN and near-contiguous p53 expression. These features are characteristic of d-VIN. However, d-VIN is exceedingly rare in young patients. The case was reviewed by 6 dermatopathologists and gynecologic pathologists, who observed that the degree of inflammation would be unusual postclobetasol therapy and could be due to noncompliance. A review of the patient's chart revealed that she "does not always remember to apply" clobetasol. The patient's clinician confirmed that there were compliance issues, and the follow-up biopsy was negative for d-VIN. The case was signed out as LS, with a note describing the above, and to rebiopsy if concern persisted. The authors conjecture that inflammatory infiltrates in the biopsied area caused reactive atypia due to lack of adherence to treatment. Although the patient's age helped rule out d-VIN, similar cases in elderly patients may be occurring. Pathologists must be aware that reactive forms of untreated LS can mimic d-VIN, to avoid misdiagnosis., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: Conflicts of Interest The authors have no conflicts of interest to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Background/objectives: Lichen sclerosus (LS) is a chronic condition that warrants close follow-up due to the risk of scarring. The optimal long-term management of pediatric vulvar and perianal lichen sclerosus (PVPLS) is unknown. This study aimed to identify diagnostic, treatment, and maintenance regimens among pediatric dermatologists and pediatric/adolescent gynecologists, as well as assess provider confidence and desire for guidance on long-term PVPLS management., Methods: A cross-sectional 35-question survey was administered through the Pediatric Dermatology Research Alliance (PeDRA) and the North American Society for Pediatric and Adolescent Gynecology (NASPAG) between 7/13/2021 and 8/30/2021 to ascertain PVPLS diagnostic and management regimens., Results: Most responders were attending-level pediatric/adolescent gynecologists (46%) and pediatric dermatologists (41%). Although 85% of participants felt completely or very confident in diagnosing PVPLS, the majority (86%) desired further management guidelines. While the initial treatment was similar among providers, maintenance regimens and follow-up varied considerably, with only 42% recommending lifelong monitoring despite potential persistence into adulthood., Conclusions: While initial treatment was similar among practitioners, there was variation by specialty in subsequent management and a lack of uniformity in long-term follow-up. Additional studies are needed to clarify the optimal management of PVPLS and to provide evidence-based guidelines regarding long-term follow-up.  J Drugs Dermatol. 2024;23(6):450-455.     doi:10.36849/JDD.8084.

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory disorder, most often characterized by atrophic skin plaques located on female genitalia. Infrequently, LSA may present extragenitally; however, much is unknown about the temporal relationship between genital and extragenital LSA. Morphea, also known as localized scleroderma, is a rare inflammatory skin condition characterized by sclerotic plaques. Investigators debate whether LSA and morphea exist on the same spectrum of disease, with LSA representing a superficial variant of morphea involving genitalia, or if they are distinct but coincidental entities. Although researchers have described LSA and morphea occurring in different locations on the same patient, few reports describe LSA and morphea occurring in the same lesion and in the inguinal folds. Herein, we report a case of a 62-year-old woman with extragenital LSA-morphea overlap in the inguinal folds, who three months later developed genital LSA. Extragenital LSA-morphea in the same plaque, with no signs of genital lesions on initial exam, with later development of genital LSA, is especially uncommon. The temporal progression of extragenital LSA-morphea overlap to genital LSA over a three-month period is an important contribution to the literature, as the temporal relationship between extragenital and genital LSA is not previously discussed.

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., Competing Interests: O.H. serves occasionally on scientific advisory boards organized by Bayer AG and Gedeon-Richter, and has lectured at educational events organized by these companies. The other authors have declared they have no conflicts of interest., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.)

Introduction: It is estimated that approximately one out of 200 boys has the diagnosis of lichen sclerosus (LS), previously referred to as BXO (balanitis xerotica obliterans). Severe progressive disease is rare however, mismanagement of urethral tissues may contribute to progression of LS., Study Design: The current literature regarding the management of severe lichen sclerosus was reviewed alongside our management of seven patients with ages ranging from six to ten years of age with severe lichen sclerosus who required surgical intervention. These patients were identified out of a busy pediatric practice that saw 5507 patients during the four-year span. Based on the pathophysiology of lichen sclerosus, urethral anatomy, and our management an algorithm was developed for medical and surgical management., Results: All patients received initial medical treatment with topical steroids. Three patients underwent urethral mobilization and serial biopsy. One of these patients with severe disease required a second distal urethral mobilization. Three patients were treated with circumcision, and one is responding well to topical steroids after complex reconstruction. None developed postoperative urethral disease., Discussion: Lichen sclerosus affects squamous epithelium but can extend to unaffected tissue if traumatized via the Koebner phenomenon. There are no randomized control trials for the management of the disease. Thus, appropriate early management with avoidance of urethral dilation or incision may prevent extension down the urethra that can lead to severe stricture disease. Several authors identified this as one of the worst forms of stricture diseases to manage. Based on the pathophysiology of the disease and our 4-year experience treating patients, we propose an algorithm for management of severe lichen sclerosus in boys. The diagnosis of lichen sclerosus in boys requires a high level of suspicion, and early biopsies should be obtained if suspected. If identified before circumcision or meatotomy, initial treatment should be medical. If the patient fails topical steroid therapy, circumcision and biopsy are the initial recommended surgical approach. Optimally, a biopsy with the first meatotomy establishes the diagnosis. If the disease persists, urethral mobilization may represent a curative treatment as it advances healthy urethra and allows complete removal of distal squamous epithelium instead of traumatic repeated dilations or incisions., Conclusion: This paper summarizes the available literature on the management of severe LS and provides a flow diagram based on the pathophysiology of the disease and our experience sever cases., Competing Interests: Conflict of interest None to disclose., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

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., (© 2023 Wiley Periodicals LLC.)

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., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.)

Competing Interests: Conflicts of interest None disclosed.

Competing Interests: Conflicts of interest The authors declare no conflicts of interest.

Competing Interests: Conflicts of interest Dr LaChance is a Principal Investigator for a research grant from Pfizer looking to explore the role of the JAK/STAT pathway in connective tissue disease. The other authors have no conflicts of interest to declare.

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., (© 2023 Wiley Periodicals LLC.)

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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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., (© 2022. The International Urogynecological Association.)

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.

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.

Background: Vulval lichen sclerosus (VLS) is a chronic inflammatory condition predominantly affecting the anogenital skin. Symptoms can be distressing and affect quality of life and everyday activities. Very little research has been undertaken to explore the experience of living with VLS from the perspective of people with the condition., Objectives: To understand individuals' experiences of VLS and its impact on their lives., Participants and Methods: Semi-structured remote (telephone or video) interviews were conducted with a purposive sample of 20 women living in the UK with VLS recruited via online support groups and social media. Data collection and analysis was informed by social constructionist grounded theory, using a constant comparison method., Results: We developed three themes to interpret the experience of living with VLS: missed opportunities (participants experienced delayed diagnosis, lack of information and disempowering encounters with healthcare professionals); learning to live with a long-term condition (the amount of work involved in learning how to self-manage the disease and the impact on everyday life); a secret life (experiences of the condition were often shrouded in secrecy, and there was stigma associated with a vulval skin condition resulting in them feeling isolated and lonely)., Conclusions: Patients attending healthcare appointments with vulval complaints should be examined and LS should be considered as a diagnosis. Healthcare professionals' awareness and knowledge of VLS needs to be improved and they should avoid language which is blaming or minimizing of patients' experiences. VLS is a chronic condition and patients need to be supported in self-management. Support groups may be a source of support and information but can also be challenging when hearing others' difficult experiences. Wider public health educational activities are needed to change societal attitudes towards female genitals and tackle the stigma around vulval conditions. What is already known about this topic? Vulval lichen sclerosus (VLS) can have a profound impact on quality of life and self-identity but is relatively underexplored from the perspective of those living with the condition. What does this study add? In-depth findings about the experiences of living with VLS including ongoing issues with timely diagnosis, learning to live with a long-term condition and the secrecy and stigma about the condition. The needs of women with symptoms of and diagnoses of VLS are not being met sufficiently by the healthcare system. What are the clinical implications of this work? Healthcare professionals should consider addressing knowledge gaps in vulval conditions including VLS to prevent delayed diagnosis and avoid the use of certain terminology which can minimize patients' experiences. Patients with vulval complaints should be examined and LS should be considered as a diagnosis. Regular follow-up would reflect its chronic nature and could provide patients with reassurance and confidence in self-management. Wider public health activities are needed to change societal attitudes and tackle stigma around vulval conditions., (© 2022 The Authors. British Journal of Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.)

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.

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.

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.

Background Early inflammatory lesions of lichen sclerosus are histopathologically difficult to diagnose until the hallmark of the disease i.e., papillary sclerosis becomes visible in histological sections. Pre-sclerotic and late or resolved phases of the disease have not been extensively studied. Methods We retrospectively reviewed all cases diagnosed as genital lichen sclerosus over a ten-year period from 2006 to 2016, correlating the clinical findings with the histological features. Results A total of 133 cases of genital lichen sclerosus (90 males and 43 females) were identified. Both genders demonstrated a similar histological spectrum. Fifty eight (44%) cases were identified as having pre-sclerotic lichen sclerosus, 64 (48%) as having progressive disease and 11 (8%) cases were classified as fully resolved with atrophy. Asymptomatic vitiligoid lesions were identified in 19 (14%) cases of which 12 were male. Low-grade squamous cell carcinoma was seen within the areas affected by long-standing lichen sclerosus, in four patients (3%, 2 male). Limitations We studied only haematoxylin and eosin stained sections. The presence of basement membrane thickening could have been better illustrated with the periodic acid-Schiff stain. Conclusion The pathogenesis of lichen sclerosus probably involves an immune reaction to the basement membrane at the epidermal interface and around the adnexa. The initial band of inflammation shifts gradually downwards from the epidermal interface into the dermis destroying the vascular channels and appendages, resulting in excessive deposition of altered extracellular matrix. Basilar infiltration of lymphocytes along with a grossly vacuolated or thickened basement membrane is proposed as the characteristic diagnostic feature of the pre-sclerotic stage. Greater awareness of the clinicopathological spectrum of lichen sclerosus should enable early diagnosis and treatment, thereby preventing structural damage and possible malignant transformation in chronic cases.

This review investigates women suffering from chronic vulval pruritus who may suffer from lichen sclerosus (LS). LS is a chronic inflammatory skin condition involving the vulva and anogenital regions. Treatment may be initiated upon suspicion, although the diagnosis is predominantly clinical and at times a skin biopsy is needed to confirm the diagnosis. LS is treated with potent to very potent topical steroids initially for three months. Prophylactic lifelong treatment is recommended to avoid long term scarring, sexual dysfunction, improve quality of life and to prevent cancer. Mild cases can be managed in general practice.

Background: The incidence of preputial lichen sclerosus (PLS) among children presenting with phimosis varies from 10 to 95%, depending on the age, the protocol for the treatment of pediatric phimosis, the method of diagnosis (clinical versus histological), and case mix (congenital versus acquired phimosis)., Objective: PLS may not be clinically obvious. Our aim is to show that a systematic histological examination of the prepuce may diagnose PLS in clinically unsuspected cases., Methods: Prospective observational study of the histology of all prepuces resected from boys undergoing circumcision for phimosis but not clinically suspected to have PLS., Results: PLS was diagnosed histologically in 22 boys (32%). Boys with PLS were significantly older (mean 8.4 versus 4.7 years old). Diagnosis of PLS was not related to the degree of phimosis (summary figure). In three patients (grade 4 phimosis) glans discoloration was observed during surgery, and all had PLS. Follow up for boys found to have PLS ranged from 1 to 10 years. One patient developed recurrent phimosis, attributed to inappropriate conservative resection, and required further surgery. There were no cases of meatal stenosis., Discussion: Subtle cases of PLS may be difficult to detect clinically. Children are frequently asymptomatic, except for being unable to retract the prepuce. Physical examination has a low negative predictive value for the diagnosis of PLS. Complete removal of the prepuce with permanent glans exposure is regarded as essential to cure PLS and to avoid recurrent phimosis, but our patients were treated with partial circumcisions for cultural reasons. Only one needed reoperation for recurrent phimosis., Conclusion: Histological PLS was present in approximately 1/3 of boys with phimosis, frequently without typical manifestations. Those patients may be cured with partial circumcisions., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Lichen sclerosus (LS) is a rare and chronic autoimmune disease, and extragenital LS is particularly challenging to diagnose in children. Histopathology is the gold standard for diagnosis. Dermoscopy is a useful tool when follicular plugs (FP) are observed, though FP are sometimes hard to recognize. The ink test may provide a useful technique to enhance FP visualization and support a diagnosis of extragenital LS, thereby avoiding diagnostic delays or biopsy., (© 2022 Wiley Periodicals LLC.)

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., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2022, ASCCP.)

Background: Studies concerning pediatric lichen sclerosus are limited, and, to date, there have been no studies comparing the course of lichen sclerosus in boys and girls. We sought to examine all publications on boys and girls with lichen sclerosus and assess and compare epidemiology, symptoms and signs, genetic background, risk factors, treatment, and prognosis., Methods: A systematic search was performed in the Embase, Medline, Cochrane, and Web of Science databases. Inclusion criteria were information on children ages 0-18 years and a clinical or histologic diagnosis of lichen sclerosus. Literature from 1985 to 2021 was reviewed., Results: A total of 1780 articles were retrieved from the search, of which 90 articles were eligible for inclusion. Boys and girls present similarly on many aspects; nonetheless, treatment and follow-up are approached differently., Conclusions: Though the clinical approach is often different, lichen sclerosus in boys and girls demonstrates many similarities. More research is needed, especially on follow-up, to gain a better understanding of the course of lichen sclerosus and establish an advanced management plan for children., (© 2022 The Authors. Pediatric Dermatology published by Wiley Periodicals LLC.)

Background: Lichen sclerosus is a chronic relapsing inflammatory dermatosis with a predilection for anogenital skin in 85%-98% of cases and is more prevalent in women (3%) than in men (> 0.07%)., Objectives: The purpose of this study was to investigate gender differences in clinical presentation and therapeutic response to treatment with platelet-rich plasma (PRP), an emerging innovative strategy for LS., Methods: Forty-three male and 51 female patients affected by LS were evaluated. Each patient was subjected to PRP treatment (1 infiltration every 15 days, for 3 times)., Results: The PRP procedure was well tolerated by all patients and an overall significant decrease in symptoms was reported 6 months after PRP infiltration. Reduction in pain and burning sensation was significant in both gender but more accentuated in women than in men, whereas reduction of itching was similar. On the contrary, dyspareunia evidenced sex-related difference since a significant diminution was observed only in male., Conclusions: This study demonstrates that PRP based therapy may exert a relevant role in LS patient management due to its effect on Quality of Life (QoL) and sexual function in both gender. In addition, the study underlined gender-related differences in severity of symptoms and disease age onset.

Vulvar dermatoses are often debilitating chronic skin conditions associated with pain and pruritus. In oncologic patients, cancer treatments can precipitate and exacerbate vulvar dermatoses. Cytotoxic chemotherapy, hormonal therapies, and local pelvic radiation therapy can lead to vulvar symptoms, and cancer treatment-induced vulvar conditions include graft-versus-host-disease and radiation dermatitis. There have also been reports of lichen sclerosus development or exacerbation secondary to hormonal therapy and immune checkpoint inhibitors, attributed to proposed hormonal and immunologic pathogenesis of lichen sclerosus. Early recognition and treatment of these conditions can significantly improve quality of life. In this review, we summarize the clinical features and management characteristics of six types of common vulvar dermatoses that may present in the oncologic patient., Competing Interests: Declaration of Competing Interest Sarah J. Noor funded in part by the NIH/NCI Cancer Center Support Grant P30 CA008748. SJN has a consultant role for Kyowa Kirin., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

This Clinical Opinion replaces the NASPAG Clinical Recommendation: Pediatric Lichen Sclerosus published in 2014. The objective of this document is to provide guidance in the diagnosis and management of vulvar lichen sclerosus (LS) in the pediatric and adolescent patient in order to treat patient symptoms and reduce long-term sequelae. LS is a chronic inflammatory condition affecting the anogenital region that may present in the prepubertal or adolescent patient. Clinical presentations include significant pruritus, loss of pigmentation and vulvar adhesions with loss of normal vulvar architecture. Management includes topical agents for induction and maintenance therapy, as well as long-term follow-up for identification and treatment of recurrence and sequelae. This document is intended for use by both primary and specialty pediatric and adolescent gynecology (PAG) providers, including specialists in pediatrics, gynecology, adolescent medicine, and dermatology., (Copyright © 2021. Published by Elsevier Inc.)

Lichen Sclerosus (LS) is a chronic inflammatory skin disease with unknown etiology. In pediatric age the main disease "lichenlinked" is the phimosis in male. This is a retrospective study that reports the experience of our clinic and review of the literature. We included all patients affected by pathological phimosis, treated by circumcision between January 2015 and May 2020, older than 6 years old and with an histopathological diagnosis of lichen sclerosus. The aim was to identify prognostic factors based on histological report to plan the clinical management of patients. Statistical analysis was done. We included 207 patients. The mean age of children was 9,78 years (5-18 years, DS±3.29). Based on the histological features we divided patients in 2 groups: early lesions (70/207, 34%) and advanced (137/207, 66%). In term of complications lichen linked we considered meatal stenosis that needed of urethral dilatations. We included 7 patients (7/207, 3,4%). We report P value Statistical Significance in many aspects. An early diagnosis of LS and surgical treatment of foreskin are essential to prevent early and late complications in children. The size of sample is a limit of the study but results encourage our management.

Lichen sclerosus (LS) is a disabling chronic inflammatory disease of skin and genital mucous membrane causing itch, pain, dysuria and restriction of micturition, and significant sexual dysfunction and dyspareunia both in women and men. If left untreated, LS is associated with a high degree of sclerosis and scarring, as well as with an elevated risk of cancer in the genital area. Although a central role of autoimmunity is suggested, the pathogenesis of LS is still not clearly understood and the disease remains difficult to treat. The goals of treatment of LS are to alleviate symptoms and discomfort, prevent anatomical changes and prevent malignant transformation. This guideline has been developed by an Italian group of experts. It summarizes evidence-based and expert- based recommendations. The highest level of evidence favors the use of topical high potency corticosteroids; second- and third-line treatments include topical calcineurin inhibitors and topical retinoids, respectively. Surgical treatment has become the treatment of choice in male genital LS with persistent phimosis not responsive to medical treatment. The aim of this paper is to offer evidence-based and easily applicable recommendations for the management of LS.

Background: Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory cutaneous disease., Aim: To evaluate the characteristics of paediatric LSA patients in Turkey., Material and Methods: Data for patients aged <18 years who were diagnosed with LSA by a paediatric dermatologist were retrospectively reviewed. Patient demographic characteristics, clinical features, disease duration, the vitamin D level, accompanying diseases, presence of atopy, therapeutic approach and treatment response were recorded., Results: The study included 38 patients, of whom 35 (92.1%) were female and 3 (7.9%) were male. Mean age at onset of disease was 6.4 ± 3.3 years in females versus 3.1 ± 2.6 years in males. Mean diagnostic delay was 20.6 ± 28.9 months in females and 2 ± 1.7 months in males. In 28 (80%) patients the time from onset of lesions to diagnosis was ≥3 months. The majority (76.3%) of the patients were asymptomatic, whereas five had itching, two had itching and burning, and two had pain. Among the females, 12 (34.3%) and 23 (65.7%) presented with isolated extragenital and anogenital involvement + extragenital lesions, respectively. All three males had isolated extragenital involvement. The most commonly recommended treatments were topical calcineurin inhibitors and calcipotriol/betamethasone ointment. Accompanying diseases were as follows: alopecia areata (n = 2); atopic dermatitis (n = 2); vitiligo (n = 2); ulcerative colitis and juvenile idiopathic arthritis (n = 1); Hashimoto's thyroiditis (n = 1). Among the 28 patients whose vitamin D level was measured, 24 (85.7%) had vitamin D deficiency., Conclusion: LSA can be asymptomatic in the majority of affected children. Diagnostic delay was noted in 80% of the study's LSA patients, highlighting the lack of awareness of the condition among parents and clinicians., (© 2021 John Wiley & Sons Ltd.)

Lichen sclerosus of the vulva (LSV) is seen frequently enough to warrant knowing how to diagnose it and institute appropriate treatment strategies. LSV is a chronic skin disorder, very likely of autoimmune origin, which may affect various areas of the perineum, although some women may be affected in extragenital areas. The disease has significant adverse impact on quality of life and sexual activity and may undergo malignant transformation. History of symptomatology and clinical examination is sufficient to make the diagnosis. Skin biopsy is only necessary in specific scenarios. Topical corticosteroids are still the mainstay of therapy, initially to institute remission and then for maintenance. Long-term therapy and surveillance are invariably necessary. Surgery does have a role, but only in specific associated conditions. A number of alternative treatment options have been mooted, especially if the disease is resistant to topical corticosteroids, but these options are still being assessed and studied.

Vulvar vitiligo (VV) and vulvar lichen sclerosus (VLS), both feature skin and mucosal hypo-/depigmentation. The aim of this study was to describe the clinical and dermoscopic features of VV and VLS in the pediatric population, providing diagnostic clues, and to define their association. We performed a systematic literature review of the clinical and dermoscopic features of pediatric VV and VLS. An observational study was conducted on children affected by VLS associated with VV, referred to the Dermatology Unit of the Sant'Orsola Polyclinic in Bologna, Italy. Medical history, age at diagnosis, ethnicity, clinical and dermoscopic features, and symptoms were recorded for all patients. 124 cases of VLS and 10 cases of VV were reviewed. Clinical manifestations included hypo-/depigmented patches in both conditions, while ecchymosis/purpura and fissures/erosion were observed in VLS. Symptoms including pruritus, pain, or burning were reported only by VLS patients. In our study five patients with VLS associated with VV were retrieved. Clinical features included well-demarcated depigmented patches in VV and translucent areas, erythema, ecchymoses/purpura, and labial fusion in VLS. Dermoscopy showed white structureless areas with a whipped cream-like appearance, linear or dotted vessels, white chrysalis-like structures, erosion and red-purpuric blotches in VLS and reduced pigment network or pigment absence, intralesional spots of residual pigmentation and telangiectasias in VV. Symptoms were present in all patients. Both VV and VLS show hypo-/depigmented patches. In the presence of associated symptoms, possible VLS should be investigated with clinical and dermoscopic examination to achieve a prompt diagnosis., (© 2021 Wiley Periodicals LLC.)

Competing Interests: Competing interests: The BMJ has judged that there are no disqualifying financial ties to commercial companies. The authors declare the following other interests: none.