Treatment algorithm for the comprehensive management of severe lichen sclerosus in boys based on the pathophysiology of the disease.

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.

Lichen Sclerosus and Sexual Dysfunction: A Systematic Review and Meta-Analysis.

BACKGROUND: Lichen sclerosus (LS) is a common autoimmune dermatological condition that is often under-diagnosed in women and has been documented to affect quality of life and sexual function. AIM: To determine the prevalence of sexual dysfunction among women with vulvar lichen sclerosus. METHODS: The authors conducted a systematic review and meta-analysis of the existing research on LS and sexual function in database including PubMed using search terms: lichen sclerosus OR vulvar lichen sclerosus OR vulvar lichen sclerosus et atrophicus OR kraurosis vulvae) AND (sexual function OR sexual functions OR sexual disorder OR sexual disorders OR sexual activity OR sexual activities OR sexual dysfunction OR sexual dysfunctions OR dyspareunia OR vaginismus). OUTCOMES: Nearly 60% of women with lichen sclerosus suffer from sexual dysfunction. RESULTS: Two hundred and ten studies were initially identified. Twenty-six articles met inclusion criteria and 3 were excluded as they did not relate to sexual function, were regarding a surgical or medical intervention and sexual dysfunction and one was a review article. Therefore, 23 studies were included in the final analysis resulting in a cumulative 486 participants with LS with 208 patients experiencing any kind of sexual dysfunction. Meta-analysis presented prevalence of sexual dysfunction among LS patients as 59% (95% CI: 48 - 70%). Dyspareunia or generalized pain with intercourse was the most commonly reported type of dysfunction. CLINICAL IMPLICATIONS: Discussing sexual concerns with women with LS could empower them to seek treatment. STRENGTHS AND LIMITATIONS: Few articles met criteria for inclusion. CONCLUSION: A large proportion of women with LS experience sexual dysfunction. More research is needed, especially that which includes biopsy-proven LS and validated tools on sexual function. Pope R, Lee MH, Myers A, et al. Lichen Sclerosus and Sexual Dysfunction: A Systematic Review and Meta-Analysis. J Sex Med 2022;19:1616-1624.

Autologous lipoaspirate as a new treatment of vulvar lichen sclerosus: A review on literature.

Lichen sclerosus (LS) is a chronic inflammatory dermatosis that mostly affects the genital and anal skin areas. Symptoms may vary from pruritis and pain to sexual dysfunction; however, LS can also be asymptomatic. LS occurs at all ages and in both sexes. Approximately 5% of all women affected by vulvar LS will develop vulvar squamous cell carcinoma. Topical treatment is safe but less effective resulting in chronic course in most patients, who suffer from persistent itching and pain. In severe cases of therapy-resistant LS, there is no adequate treatment. Fat grafting is a novel regenerative therapy to reduce dermal fibrosis. The therapeutic effect of adipose tissue grafts for LS is already investigated in various pioneering studies. This review provides an overview of these studies and the putative mechanisms-of-action of fat grafting to treat LS.

The management of penile intraepithelial neoplasia (PeIN): clinical and histological features and treatment of 345 patients and a review of the literature.

INTRODUCTION: Penile intraepithelial neoplasia (PeIN) is a histological term for precancerous penile lesions. PeIN is important due to the high morbidity and mortality associated with progression to penile squamous cell carcinoma (PSSC). But PeIN is rare, contributing to a limited evidence-base for the relative efficacy of available treatment options. OBJECTIVES & METHODS: To consolidate and expand knowledge about PeIN and its treatment, we describe the clinical and histological characteristics, treatments and outcomes of 345 patients with PeIN, managed by our multidisciplinary team. Our results are compared and contrasted with those in the literature, following comprehensive review. RESULTS: 8.7% of patients had concomitant, invasive PSCC, whilst 91.3% demonstrated PeIN alone. 84% had undifferentiated PeIN, and 10.7% differentiated PeIN (5.2%, not specified). Clinical or histological evidence of HPV alone was present in 58%; features of lichen sclerosus alone in 12%; features of both in 29.4%. Only 14.4% of patients could be treated solely with topical agents or cryotherapy, whereas the remaining 85.6% underwent some form of surgical intervention, circumcision being the mainstay. Just 2.6% progressed to PSCC. CONCLUSIONS: Clinical management of PeIN can be rationally optimized with excellent outcomes. Circumcision is important. Topical treatments alone are disappointing.

The Diagnosis and Treatment of Lichen Sclerosus and Bulbous Urethral Squamous Cell Carcinoma: A Case Report.

Lichen sclerosus (LS) is a rare disease with malignant potential and has been identified by the European Society of Urology Guidelines as a risk factor for penile squamous cell carcinoma (SCC) cancer. LS combined with urethral squamous cell carcinoma (USCC) is extremely rare. There has been only one case report of this combination in China over the last decade. The prevalence of this combination in East Asian population is unclear. In this report, a 49-year-old patient with the presence of LS, perineal masses, and abscesses was hospitalized. He underwent anti-infectious therapy, meatotomy, perineal abscess incision, and drainage and debridement of the perineal wound. USCC was diagnosed following a series of biopsies and treated with radiation therapy. The patient remains well and alive with no recurrence of USCC 13 months after radiation treatment.

Common Benign Chronic Vulvar Disorders.

Common benign chronic vulvar conditions include genitourinary syndrome of menopause (formerly called vulvovaginal atrophy), lichen sclerosus, lichen planus, lichen simplex chronicus, and vulvodynia. Genitourinary syndrome of menopause results from the hypoestrogenic state that leads to atrophy of normal vulvar and vaginal tissues. It is typically treated with lubricants, moisturizers, and intravaginal estrogen. Lichen sclerosus is an inflammatory condition characterized by intense vulvar itching. It is treated with topical steroids or, in some cases, topical calcineurin inhibitors. Patients with lichen sclerosus are at risk of vulvar squamous cell carcinoma and should be monitored closely for malignancy. Lichen planus is an inflammatory autoimmune disorder that can affect the vulva and vagina in addition to other skin and mucosal surfaces. The first-line treatment is topical steroids, and significant scarring can occur if left untreated. Lichen simplex chronicus manifests as persistent itching and scratching of the vulvar skin that leads to thickened epithelium. Breaking the itch-scratch cycle, often with topical steroids, is the key to treatment. Vulvodynia is a common vulvar pain disorder and is a diagnosis of exclusion. A multimodal treatment approach typically includes vulvar hygiene, physical therapy, psychosocial interventions, and antineuropathy medications.

Recent advances in understanding and managing Lichen Sclerosus.

Lichen sclerosus (LS), or balanitis xerotica obliterans as it was previously known, is a chronic inflammatory lymphocyte-mediated scarring dermatosis that often affects the preputial skin and glans, leading to phimosis and urethral strictures if left untreated. We present a narrative review of the literature assessing its aetiology and pathogenesis and discuss its links to penile cancer and its medical and surgical management. Possible hypotheses for the development of LS include chronic exposure to trapped urine, leading to changes in the epithelial structure. This is supported by the fact that circumcision is often curative in the early stages of the disease. Although circumcision can be curative, the use of topical steroids is typically the first-line treatment and may preserve the foreskin and forgo the need for circumcision altogether. Patients should be made aware of a possible association with penile cancer. Although the majority of cases can be treated by medical therapy and circumcision, a significant number of patients may also require penile reconstructive procedures.

Gender differences in genital lichen sclerosus: data from a multicenter Italian study on 729 consecutive cases.

BACKGROUND: Studies specifically conducted to assess gender differences in genital lichen sclerosus (GLS) are not available. This multicenter study aimed to identify possible gender-related differences on GLS clinical features, history and course, through collecting data from a large mixed-sex sample of patients. METHODS: This was a cross-sectional study on 729 subjects (53.8% females, 46.2% males) affected with GLS, consecutively observed within a network of 15 Italian dermatology units. The following information was specifically collected: clinical features and severity of symptoms related to GLS, extragenital involvement, previous therapies, diagnostic suspicion at referral, type of referring physicians, development of genital squamous-cell carcinoma (SCC). RESULTS: Females complained of symptoms more frequent and severe than men; pallor and scarring-sclerosis-atrophy were the most frequent features without gender differences; itching-related signs were more frequent in females than in males as well as extragenital involvement; prior to receiving a definitive diagnosis, females received treatment more frequently than males; 40% of patients were referred with a misdiagnosis; the highest rate of correct suspected diagnosis at referral came from dermatologists than from other physicians; duration of the disease was found to predispose to SCC development. CONCLUSIONS: Our findings highlighted several gender differences on clinical presentation and symptom profile of GLS. In spite of some characteristic features, misdiagnosis at referrals was frequent.

Pathophysiology, Clinical Manifestations, and Treatment of Lichen Sclerosus: A Systematic Review.

OBJECTIVE: To elucidate current understanding on the pathophysiological mechanism of genital lichen sclerosus (LS), urologic manifestations, and treatment options. MATERIALS AND METHODS: The Medline/PubMed and Embase databases were systematically reviewed for publications pertaining to LS. After applying inclusion and exclusion criteria, references were assessed for relevance to the pathophysiology, presentation, and treatment of LS by title and abstract review by 2 independent reviewers, yielding 186 articles for assessment. RESULTS: The contemporary understanding of the epidemiology and histology of LS is reviewed herein. Additionally, we explore in detail the 3 hypotheses regarding the pathophysiological mechanism contributing to disease presentation: infectious etiology, primary immune dysregulation, and the isotraumatopic response. We summarize the available biological evidence supporting each hypothesis. This discussion provides context for understanding LS morbidity and may spur new avenues of research. For the clinician, we review the clinical presentation of disease, including the risk of progression to squamous cell carcinoma. The current medical and surgical treatment options are also detailed. CONCLUSION: LS remains a potentially insidious disease which may lead to debilitating urinary and sexual dysfunction. Cross disciplinary research should aim for earlier detection, as well as more effective and durable treatment. The exact cause of LS remains unknown.

Oral and Vulvar Lichen Sclerosus.

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin (1). Simultaneous involvement of the oral mucosa is extremely rare, but it may be the only affected area (2). A 55-year-old woman was referred to the Department of Oral Medicine, School of Dental Medicine University of Zagreb due to whitish lesions on the right ventrolateral part of the tongue and buccal mucosa with desquamative gingivitis (Figure 1, a-c). The lesions were asymptomatic but indurated on palpation. Histology was conclusive for oral lichen sclerosus (OLS). The lesions on gingiva were successfully treated with betamethasone ointment, three times a day for two weeks. One year earlier, she had been referred to the Department of Dermatology and Venereology with progressive pruritus and dyspareunia, white patches, obliteration of the labia minora, and stenosis of the introitus (Figure 2). Histology was conclusive for vulvar LS (Figure 3, a and b). She was successfully treated for 5 months with clobetasol propionate 0.05% ointment. The patient was taking levothyroxine to treat hypothyroidism associated with Hashimoto's thyroiditis and was otherwise healthy. Oral LS is clinically characterized by the appearance of white macules, papules, or plaques mostly appearing on labial mucosa but also on buccal, palate mucosa and on the lower lip (2,3). On the genitals, it typically manifests as atrophic white plaques, which may be accompanied by purpura or fissuring (1). While vulvar LS is often associated with pruritus, dyspareunia, and dysuria, OLS is often asymptomatic, although pain, soreness, pruritus, and tightness when opening the mouth can be present (1,2). Oral manifestations of LS, as well as association of anogenital and oral LS, are rarely reported in the literature (4-6). Tomo et al. searched the Medline database for papers reporting oral LS cases with histological diagnosis confirmation from 1957 to 2016 and found only 34 cases of oral LS with histopathologic confirmation of the diagnosis (4). Kakko et al. reported 39 histologically proven cases of OLS (2). Attilli et al. (5) reviewed the clinical and histologic features of 72 cases of LS with oral/genital involvement. They reported that LS was diagnosed with exclusive genital lesions in 45, exclusive lip involvement in 20, and orogenital involvement in only 7 cases (5). Some believe that many cases of clinically diagnosed lichen planus may actually be LS and that isolated oral mucosal LS may not be as rare as is generally thought (2). While vulvar LS can occur at any age with increasing incidence with age, the median age of patients with OLS was 34 years and most of the patients were female (1,2,5). Due to the small number of patients in the literature, treatment recommendations for OLS are not available. In case of symptomatic oral lesions, topical or intralesional corticosteroids are considered to be the first-line treatment (2). First-line treatment for anogenital LS is a potent to very potent topical corticosteroid ointment, and second-line therapies include topical calcineurin inhibitors 1% pimecrolimus and 0.1% and 0.03% tacrolimus (1). For treatment-resistant genital LS, oral retinoids, methotrexate, and possibly local steroid injections for single lesions are mainly applicable for women (1). There is limited evidence for systemic treatments for both conditions. If it is not treated, genital LS is associated with a greater degree of scarring and an elevated risk of progression to squamous cell cancer; however, malignant transformation of OLS has not been reported (1-6). Due to the very rare presentation in the oral cavity, it is important to notice these lesions during a dental exam.

The color of skin: white diseases of the skin, nails, and mucosa.

White diseases are a heterogenous group characterized by hypopigmentation or depigmentation. Skin and eye color are determined by the number and size of melanosomes present. Melanin is produced by melanosomes in the melanocytes present within the epidermis of the skin, uvea, and retinal pigmented epithelium (RPE). Conditions altering the number of melanocytes or concentration of melanin result in a lack of pigmentation, appearing as "white diseases" ranging from the well-known albinism and vitiligo to more esoteric white hand syndrome and Degos disease.

Coexistence of genital lichen sclerosus and genital warts.

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of the genital and extragenital skin. Anogenital warts are benign proliferative lesions caused by human papillomavirus (HPV), which is found in > 95% of lesions. We present two cases of the coexistence of LS and genital warts: one patient with and one without a previous history of genital warts. According to our knowledge and a literature search, only a few cases of the coexistence of LS and genital warts have been reported.

[Morphea or localized scleroderma and extragenital lichen sclerosus]. / Morphea/lokalisierte Sklerodermie und extragenitaler Lichen sclerosus.

Localized scleroderma (LoS) is a very heterogeneous connective tissue disease characterized by progressive sclerosis of the skin with possible involvement of extracutaneous structures. Both children and adults can be affected but show different frequencies for the individual subtypes of the disease. The clinical heterogeneity has already caused several modifications of existing classification criteria. Patients suffering from LoS can essentially be subdivided into five different subsets, which are defined by the horizontal and vertical extent of the tissue involvement. The quality of life of these patients is significantly impaired depending on the extent of the cutaneous and subcutaneous involvement. A causal treatment does not yet exist; however, patients should be treated with the currently available medications for progressive subtypes during the early phase of inflammation to reduce or avoid severe, cosmetic and functional impairments. Lichen sclerosus (LS) usually affects the genital as well as extragenital skin and both children and adults can be affected. This article focuses on the extragenital LS, which occurs more frequently in adults. The cause of the disease as well as causal treatment strategies are still lacking. Currently, treatment is adapted to the therapeutic strategies for LoS.

Lichen Sclerosus Accompanied by Urethral Squamous Cell Carcinoma: A Retrospective Study From a Urethral Referral Center.

To evaluate the characteristics of lichen sclerosus (LS) accompanied by urethral squamous cell carcinoma (USCC) and to raise urologists' awareness about the early management of LS, a retrospective analysis was performed on the clinical features, diagnosis, treatment, and prognosis of 18 male genital LS accompanied by USCC patients who were referred to Shanghai Sixth People's Hospital between June 2000 and August 2014. All of the patients had a long-term history of LS, urethral strictures, and urethral dilatation. Seven patients are with distal (glanular or penile) USCC, 10 patients with proximal USCC, and one with entire USCC. The most common presentation, except for LS and urethral strictures, was periurethral abscess, followed by extraurethral mass, pelvic pain, urethrocutaneous fistula, hematuria, and bloody urethral discharge. All had primary surgical excision that was adapted to tumor location and extension. All of the USCC were positive for P53 and Ki-67. P16 was positive in four cases of human papillomavirus (HPV)-associated USCC and negative in 14 cases of HPV-independent USCC. Patients with distal USCC had a significant longer survival time than proximal USCC ( p < .05). LS should be treated early to prevent the disease progression. LS probably has some associations with USCC. Distal USCC has a relatively better prognosis than proximal USCC.

[Lichen sclerosus : Symptoms, diagnosis, therapeutic procedures]. / Lichen sclerosus : Symptomatik, Diagnostik, therapeutisches Procedere.

Lichen sclerosus is a chronic, inflammatory dermatosis that usually affects the anogenital area. Early diagnosis and subsequent long-term anti-inflammatory treatment may reduce symptoms and signs and the risk of a mutilating course and the development of carcinomas.

Diagnostic criteria, severity classification and guidelines of lichen sclerosus et atrophicus.

We established diagnostic criteria and severity classification of lichen sclerosus et atrophicus, because there is no established diagnostic criteria or widely accepted severity classification of the disease. Also, there is no clinical guideline for lichen sclerosus et atrophicus in Japan, so we proposed its clinical guideline. The clinical guidelines were formulated by clinical questions and recommendations on the basis of evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guidelines easy to use and reliable including the newest evidence, and to present guidance for various clinical problems in treatment of lichen sclerosus et atrophicus.

Liquen escleroso genital y extragenital en la infancia: a propósito de un caso clínico / Lichen sclerosus in childhood

El liquen escleroso es una dermatosis inflamatoria crónica de piel y semimucosas que afecta princi-palmente vulva y región perianal, la localización extragenital vista sólo en 15 a 20% de los casos es rara en la infancia. La presentación más frecuente es la anogenital con pápulo-placas blanco marfil, atrofia, erosiones, púrpura y esclerosis. En mujeres afecta simétricamente zona interna de vulva, pe-rianal y periné adquiriendo forma típica en ocho, sin afectar vagina o himen. El liquen escleroso ex-tragenital se localiza principalmente en el dorso, cuello, periumbilical, muñecas y axilas con lesiones similares a las descriptas. Presentamos el caso clínico de una escolar de 11 años, sin patologías previas, que consulta por der-matosis situada en piel y mucosas topografiada en tronco y genitales. En piel, zona escapular bilate-ral, se observan placas blanco-nacaradas de cen-tro atrófico, sin esclerosis palpable. En mucosas, afectaba labios mayores, menores y zona perianal en forma de ocho, con atrofia y coloración blanco-brillosa. Refiere un año de evolución, con prurito vulvar y constipación. Se realiza diagnóstico de li-quen escleroso genital y extragenital y tratamiento con buena evolución.La importancia de conocer su diagnóstico radica en que es una patología que afecta la calidad de vida de los pacientes, por su sintomatología, su po-sible repercusión sexual y urinaria, y su potencial riesgo de carcinoma espinocelular. Es importante tener un alto índice de sospecha, ya que, debido a su baja frecuencia en la edad pediátrica, muchas veces el diagnóstico es tardío.

Lichen sclerosus is a chronic inflammatory der-matosis of skin and semi-mucosae, important for its sexual and urinary repercussion, and its poten-tial risk of squamous cell carcinoma.Mainly affects vulva and perianal region, extrage-nital localization seen only in 15 to 20% of cases is rare in childhood. The most frequent presenta-tion is anogenital with white ivory papule-plates, atrophy, erosions, purpura and sclerosis. In women it affects the inner area of the vulva, peria-nal and perineum, acquiring a typical shape in eight, without affecting the vagina or hymen. The extragenital sclerosus lichen is located mainly on the back, neck, periumbilical, wrists and armpits with lesions similar to those described.We present the case of a schoolgirl of 11 years, without previous pathologies, who consulted for dermatosis located on the skin and mucous mem-branes on the trunk and genitals. In skin, bilateral scapular area, white-nacreous plates of atrophic center are observed, without palpable sclerosis. In mucous membranes, it affected the labia mi-nora, majora and perianal zone in eight form, with atrophy and white-shiny coloration. She referred a year of evolution, with vulvar pruritus and constipation. Genital and extragenital lichen sclerosus diagnosis was made and had a good evolution with treatment.

Oral lichen sclerosus-a review of clinical presentation, treatment, and clinical outcomes.

OBJECTIVE: The aim of this study was to evaluate the disease involvement, treatment and response, and malignant potential of oral lichen sclerosus (LS). STUDY DESIGN: We conducted a review of the literature of 37 cases of oral LS. We looked specifically for the following data: patient demographic characteristics, disease involvement, treatment, response to treatment, duration of follow-up, symptoms, risk factors for oral malignancy, and malignant transformation. RESULTS: The most common area of oral involvement included the labial mucosa (67.6%). Of patients with oral LS, 38.5% reported symptoms, and 35.1% exhibited extraoral manifestations. Patients were less likely to receive treatment when asymptomatic (62.5%) than when symptomatic (80%). Topical steroids were more efficacious (66.7%) compared with other treatments. Risk factors for malignancy were found in 25% of patients. No patient had malignant transformation of oral lesions at follow-up (mean follow-up 22.1 months; median 12 months). CONCLUSIONS: More data and longer follow-up are required to determine the long-term clinical outcomes of oral LS.