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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 -
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 -
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 -
F1000Research 2020Lichen sclerosus (LS), or balanitis xerotica obliterans as it was previously known, is a chronic inflammatory lymphocyte-mediated scarring dermatosis that often affects... (Review)
Review
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.
Topics: Balanitis Xerotica Obliterans; Circumcision, Male; Humans; Lichen Sclerosus et Atrophicus; Male; Penile Neoplasms; Penis
PubMed: 32518626
DOI: 10.12688/f1000research.21529.1 -
European Journal of Pediatrics Jan 2020Lichen sclerosus (LS) is a severe, chronic, dermatosis characterised by inflammatory, sclerotic, pruritic lesions that causes significant morbidity in patients of all... (Review)
Review
Lichen sclerosus (LS) is a severe, chronic, dermatosis characterised by inflammatory, sclerotic, pruritic lesions that causes significant morbidity in patients of all genders and ages. In boys, the lesions typically affect the foreskin and glans (termed balanitis xerotica obliterans (BXO)), leading to phimosis and potentially meatal stenosis. The incidence of the disease is not well reported but the average age of affected boys is 8 years (range 1-16). Diagnosis can often be made clinically, although histological study remains important to rule out important differential diagnoses. Complications include genital scarring, urinary and sexual dysfunction as well as the development of carcinomas in adult life. Circumcision has been regarded as definitive management of BXO in boys, but this may be supplemented with medical therapies such as topical steroids, immune modulators, intralesional triamcinolone and ozonated olive oil. Supportive measures including emollients, avoidance of irritants, surveillance of complications and recurrence as well as education and counselling remain important.Conclusion: BXO remains an important cause of phimosis in boys. The frequency of this condition appears unclear but seems likely to be less than 1% of males. Treatment generally involves circumcision, with some evidence that topical steroids or immunomodulators may decrease the incidence of recurrent meatal stenosis.What is Known:• Surgical circumcision is considered the definitive management of BXO• Many aspects of BXO are still in contention or require further study including the epidemiology and aetiology.What is New:• There is increasing awareness of non-surgical modalities that may be used in adjunct to surgery including topical corticosteroids, immune modulators, intralesional triamcinolone and ozonated olive oil• Awareness of meatal stenosis-related BXO has led to the development of surgical techniques such as preputioplasty as well as buccal mucosal inlay grafts.
Topics: Adolescent; Anti-Inflammatory Agents; Balanitis Xerotica Obliterans; Child; Child, Preschool; Circumcision, Male; Europe; Humans; Infant; Male; Prognosis; United States
PubMed: 31760506
DOI: 10.1007/s00431-019-03516-3 -
Indian Journal of Sexually Transmitted... 2021Role of male circumcision (MC) as a tool to prevent sexually transmitted infections (STIs)/human immunodeficiency virus (HIV) was assessed. An attempt was made to search... (Review)
Review
Role of male circumcision (MC) as a tool to prevent sexually transmitted infections (STIs)/human immunodeficiency virus (HIV) was assessed. An attempt was made to search articles related to association between MC and STIs/HIV. A thorough search was carried out to find out quality articles published in indexed specialty journals. Centers for Disease Control and Prevention and World Health Organization (WHO) sites were also referred. Warm and moist environment of area under foreskin facilitates some pathogens to persist and replicate. Further, the thinness of foreskin predisposes it to minor trauma and abrasions that facilitate the entry of pathogens. MC reduces HIV infection risk by 50%-60% over time and reduces the risk of men acquiring herpes simplex virus-2 and human papillomavirus (HPV) that can cause penile and other anogenital cancers, by 30%. There is no significant reduction in risk of acquiring syphilis, but reduced risk of acquisition of is reported. MC is reported to be beneficial in conditions such as traumatic injury, Balanitis Xerotica Obliterans, refractory balanoposthitis, and chronic, recurrent urinary tract infections. MC also reduces the chances of penile carcinoma by facilitating improved penile hygiene, lowering HPV/HIV transmission rates, and reducing chronic inflammatory conditions such as phimosis and balanitis. MC has been recommended by the WHO and UNAIDS in 2007 as an additional HIV prevention intervention in settings of high HIV prevalence. MC is an important adjunct to safe sex education, condom use, and vaccination (HPV) in reducing the global burden of HIV/STIs-related morbidity and mortality.
PubMed: 34765930
DOI: 10.4103/ijstd.ijstd_20_21 -
Journal of Pediatric Urology Apr 2021Circumcision is a common procedure. Recently, tissue-sparing approaches have become a matter of interest, and a nerve-sparing approach is described in adults. Although...
BACKGROUND
Circumcision is a common procedure. Recently, tissue-sparing approaches have become a matter of interest, and a nerve-sparing approach is described in adults. Although circumcision is common in the practice, the nerve-sparing approach has not been evaluated in the pediatric age group.
OBJECTIVE
To give a contemporary evaluation of the preputium histology, challenge the phenomenon of a genuine nerve-sparing approach, and report the results of a prospective cohort contrasting the tissue-sparing fine dissection technique to the regular sleeve circumcision in the pediatric age group.
STUDY DESIGN
A total of 20 healthy children between 7 and 12 years of age were enrolled in the study. All circumcisions were carried out for religious purposes, and children with any anatomical anomaly, skin lesions, or Balanitis Xerotica Obliterans were not included in the study. The first 10 children underwent regular sleeve circumcision, while the latter 10 children underwent tissue-sparing fine dissection modification of the sleeve technique. All materials obtained from the circumcision were examined by a single pathologist, and relevant tissue structures were counted and compared between the groups.
RESULTS
Both techniques were satisfactory in terms of final cosmetic results, without significant complications, such as bleeding, massive edema, iatrogenic chordee, or unacceptable cosmetics. None of the children required readmission or medical intervention other than analgesics and topical moisturizing creams. Preservation of all nervous system structures, including the receptors, appeared to be not possible with macroscopic dissection techniques due to micrometer scale depth of the touch receptors. Nerve trunks were also located in less than 1-mm depth. The tissue-sparing technique could preserve significantly more vascular structures, nerve trunks, and Pacinian Corpuscles, which can be a matter of further long-term research.
CONCLUSION
We propose the term "tissue-sparing" instead of "nerve-sparing" for the available techniques. The tissue-sparing technique did not affect the clinical outcomes and the postoperative course in our study. However, it showed to be superior in terms of preserving the vascular structures, nerve trunks, and Pacinian Corpuscles.
Topics: Adult; Balanitis Xerotica Obliterans; Child; Circumcision, Male; Foreskin; Humans; Lichen Sclerosus et Atrophicus; Male; Prospective Studies
PubMed: 33339734
DOI: 10.1016/j.jpurol.2020.11.040 -
International Journal of Dermatology Jul 2019Balanitis xerotica obliterans (BXO), or penile lichen sclerosus, is a progressive sclerosing inflammatory dermatosis of the glans penis and foreskin. It is associated... (Review)
Review
Balanitis xerotica obliterans (BXO), or penile lichen sclerosus, is a progressive sclerosing inflammatory dermatosis of the glans penis and foreskin. It is associated with significant morbidity and may result in impaired urinary and sexual function. It was initially described by Stuhmer in 1928, named after its pathological features, and is considered the male equivalent of vulvar lichen sclerosis (LS). The etiology of BXO is uncertain; however, autoimmune disease, local trauma, and genetic and infective causes have been proposed. BXO occurs most commonly on the prepuce and glans penis. It is considered to have premalignant potential to transform into squamous neoplasia. This postulation rests on retrospective studies and parallels drawn with vulvar LS and squamous cell carcinoma (SCC) development. Histologically, BXO and vulvar LS are considered the same disease. There is a paucity of evidence-based guidelines to assist with appropriate follow-up for patients with BXO.
Topics: Administration, Topical; Balanitis Xerotica Obliterans; Carcinoma, Squamous Cell; Circumcision, Male; Dermatology; Diagnosis, Differential; Glucocorticoids; Humans; Male; Penile Neoplasms; Penis; Phimosis; Practice Guidelines as Topic; Precancerous Conditions; Sexual Dysfunction, Physiological; Urination Disorders
PubMed: 30315576
DOI: 10.1111/ijd.14236