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Frontiers in Medicine 2023Lichen sclerosus (LS) is an underdiagnosed inflammatory mucocutaneous condition affecting the anogenital areas. Postmenopausal women are predominantly affected and, to a... (Review)
Review
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. The etiology of LS is still unknown. Hormonal status, frequent trauma and autoimmune diseases are well-known associations for LS, yet infections do not seem to be clear risk factors. LS pathogenesis involves factors such as a genetic predisposition and an immune-mediated Th1-specific IFNγ-induced phenotype. Furthermore, there is a distinct expression of tissue remodeling associated genes as well as microRNAs. Oxidative stress with lipid and DNA peroxidation provides an enabling microenvironment to autoimmunity and carcinogenesis. Circulating IgG autoantibodies against the extracellular matrix protein 1 and hemidesmosome may contribute to the progression of LS or simply represent an epiphenomenon. The typical clinical picture includes chronic whitish atrophic patches along with itching and soreness in the vulvar, perianal and penile regions. In addition to genital scarring, and sexual and urinary dysfunction, LS may also lead to squamous cell carcinoma. Disseminated extragenital LS and oral LS are also reported. The diagnosis is usually clinical; however, a skin biopsy should be performed in case of an unclear clinical picture, treatment failure or suspicion of a neoplasm. The gold-standard therapy is the long-term application of ultrapotent or potent topical corticosteroids and, alternatively, topical calcineurin inhibitors such as pimecrolimus or tacrolimus. Collectively, LS is a common dermatological disease with a so far incompletely understood pathogenesis and only limited treatment options. To foster translational research in LS, we provide here an update on its clinical features, pathogenesis, diagnosis and (emerging) treatment options.
PubMed: 36873861
DOI: 10.3389/fmed.2023.1106318 -
Journal of Paediatrics and Child Health Dec 2018Circumcision is one of the most commonly performed surgical procedures in the world. Despite this, the practice of paediatric circumcision remains highly controversial,... (Review)
Review
Circumcision is one of the most commonly performed surgical procedures in the world. Despite this, the practice of paediatric circumcision remains highly controversial, and continues to generate ongoing debate. This debate has become more relevant recently with the provisional guidelines from the Centers for Disease Control and Prevention recommending a change of practice. In this review article, we provide an overview of the history and incidence of circumcision, normal preputial development, types of phimosis, the absolute and relative indications for circumcision as well as the evidence base for its use as a preventative measure. Our aim is to provide paediatricians with a greater understanding of this common surgical procedure and the conditions it treats, to guide their clinical practice and parent counselling.
Topics: Child; Circumcision, Male; History, Ancient; Humans; Male; Pediatrics; Preventive Medicine
PubMed: 30246352
DOI: 10.1111/jpc.14206 -
American Journal of Clinical Dermatology Feb 2013Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and... (Review)
Review
Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.
Topics: Adult; Balanitis Xerotica Obliterans; Borrelia burgdorferi; Calcineurin Inhibitors; Child; Female; Glucocorticoids; Humans; Lichen Sclerosus et Atrophicus; Male; Phototherapy; Retinoids; Vulvar Lichen Sclerosus
PubMed: 23329078
DOI: 10.1007/s40257-012-0006-4 -
Urology Aug 2010Relatively little evidence is available in the published studies on the prevention of penile cancer and premalignant conditions of the penis. The present review examined... (Review)
Review
OBJECTIVES
Relatively little evidence is available in the published studies on the prevention of penile cancer and premalignant conditions of the penis. The present review examined the current evidence available in preventing penile cancer and pathologic subtypes of premalignant conditions and their treatment. The recommendations made in the present review formulate the basis of the recent 2009 International Consultation on Urologic Disease Consensus Publishing Group.
METHODS
The association of human papillomavirus subtypes and penile cancer is well-established, although the etiology, natural history, and treatment of premalignant lesions have mainly been reported in retrospective case series with short-term follow-up. The exact pathologic role of chronic inflammatory conditions, such as balanitis xerotica obliterans in the etiology of penile cancer remains largely unknown.
RESULTS
Some of the potential strategies for the prevention of penile cancer could include circumcision, reducing the risk of transmission of penile human papillomavirus infection with male vaccination, early treatment of phimosis, smoking cessation, and hygienic measures. Implementing some of these measures would require extensive cost/benefit analysis, with significant changes in the global health policy.
CONCLUSIONS
Owing to the current levels of evidence from published studies, firm guidelines cannot be formulated for the treatment of premalignant conditions, although preventative measures, such as reducing human papillomavirus transmission, could become strategic health targets.
Topics: Carcinoma in Situ; Circumcision, Male; Humans; Male; Papillomavirus Infections; Penile Neoplasms; Precancerous Conditions; Risk Factors
PubMed: 20691883
DOI: 10.1016/j.urology.2010.04.007 -
Urology Jan 2020To elucidate current understanding on the pathophysiological mechanism of genital lichen sclerosus (LS), urologic manifestations, and treatment options.
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.
Topics: Administration, Topical; Balanitis Xerotica Obliterans; Biopsy; Circumcision, Male; Diagnosis, Differential; Disease Progression; Female; Glucocorticoids; Humans; Lichen Sclerosus et Atrophicus; Male; Penis; Prevalence; Treatment Outcome; Vulva; Vulvar Lichen Sclerosus
PubMed: 31605681
DOI: 10.1016/j.urology.2019.09.034 -
World Journal of Pediatrics : WJP Nov 2015Abnormalities involving the skin coverage of the penis are difficult to define, but they can significantly alter penile appearance, and be a cause of parental concern. (Review)
Review
BACKGROUND
Abnormalities involving the skin coverage of the penis are difficult to define, but they can significantly alter penile appearance, and be a cause of parental concern.
DATA SOURCES
The present review was based on a nonsystematic search of the English language medical literature using a combination of key words including "penile skin anomalies" and the specific names of the different conditions.
RESULTS
Conditions were addressed in the following order, those mainly affecting the prepuce (phimosis, balanitis xerotica obliterans, balanitis, paraphimosis), those which alter penile configuration (inconspicuous penis and penile torsion), and lastly focal lesions (cysts, nevi and vascular lesions). Most of these anomalies are congenital, have no or minimal influence on urinary function, and can be detected on clinical examination. Spontaneous improvement is possible. In the majority of cases undergoing surgery, the potential psychological implications of genital malformation on patient development are the main reason for treatment, and the age generally recommended for surgery is after 12 months of age.
CONCLUSION
This review provides the pediatrician with a handy tool to identify the most common penile skin anomalies, counsel parents adequately, make sensible and evidence based choices for management, and recognize complications or untoward outcomes in patients undergoing surgery.
Topics: Humans; Infant; Infant, Newborn; Male; Penile Diseases; Penis
PubMed: 25754752
DOI: 10.1007/s12519-015-0015-5 -
BJU International Nov 2011What's known on the subject? and What does the study add? The clinical presentation and complications of lichen sclerosus are well known. What is less well known is the... (Review)
Review
UNLABELLED
What's known on the subject? and What does the study add? The clinical presentation and complications of lichen sclerosus are well known. What is less well known is the true incidence of the condition. The published figures are all based on attendance at general medical clinics or specialist clinics, but it is likely that the true incidence is much higher than this reported incidence as many men will not present to the doctor for treatment. The other uncertainty is the relationship of lichen sclerosus to the subsequent development of cancer of the penis. As pointed out in the paper, it is likely that between 4% and 8% of men with this condition will develop squamous cell cancer of the penis. However, it is unclear if lichen sclerosus itself causes the development of squamous cell cancer or if it is due to coexistent infection with human papillomavirus. This review provides a concise summary of the clinical and pathological features of the disease and describes its current medical and surgical treatment. It brings together a number of papers which have addressed the association of lichen sclerosus with squamous cell carcinoma of the penis and shows that the likely incidence of carcinoma is approximately 4-8% in men with this condition.
SUMMARY
Penile lichen sclerosus, also known as balanitis xerotica obliterans, is a chronic inflammatory condition of the penis which can occur at all ages. The inflammation leads to the formation of white plaques most commonly on the foreskin or penis, and can lead to inability to retract the foreskin or blockage to the flow of urine. Cancer may occur rarely. Penile lichen sclerosus is a progressive, sclerosing, inflammatory dermatosis of the glans penis and foreskin which is of uncertain aetiology. Recent studies have shown a link between lichen sclerosus and squamous cell carcinoma of the penis. In this review, we discuss the clinical presentation, pathology and current approach to treatment of this condition.
Topics: Balanitis Xerotica Obliterans; Circumcision, Male; Humans; Male; Penile Neoplasms; Penis; Precancerous Conditions; Secondary Prevention; Urethral Diseases
PubMed: 22085120
DOI: 10.1111/j.1464-410X.2011.10699.x -
Frontiers in Immunology 2023Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected... (Review)
Review
Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.
Topics: Scleroderma, Systemic; Humans; Diagnosis, Differential
PubMed: 37600771
DOI: 10.3389/fimmu.2023.1180221