Elsevier

Urology

Volume 135, January 2020, Pages 11-19
Urology

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

https://doi.org/10.1016/j.urology.2019.09.034Get rights and content

Abstract

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.

Section snippets

EPIDEMIOLOGY

The exact prevalence of LS is unknown and generally considered underreported because of provider lack of familiarity and asymptomatic presentations or discomfiture.13 Early estimations based on referrals to dermatology practices suggest the prevalence is between 1:300 and 1:1000.14 The disease affects both women and men, though the ratio of women to men based on current estimates is approximately 3:1 to 10:1.15 A report from a general gynecology practice estimated the prevalence to be 1.7%

METHODS

The following search query was entered into Medline/PubMed and Embase: [“physiopathology” OR “etiology” OR “pathophysiology” OR “pathogenesis” OR “etiology” OR “physiopathology” OR “aetiology” OR “gene”] AND [“lichen sclerosus et atrophicus” OR “vulvar lichen sclerosus” OR “balanitis xerotica obliterans”]. This query yielded 1143 articles. We excluded non-English articles and nonhuman studies. We included only original research and articles published in 1970 or later. Two independent reviewers

LS in Men

Meatal stenosis and urethral stricture are also seen in men with LS and can result in significant morbidity for those affected with recurrence rates between 20% and 50%.38,39 If untreated, an inflexible phimotic foreskin may constrict the glans, which can manifest as a scrotalized appearance of penile shaft skin. Involvement of the meatus (Supplemental Fig. 2) and urethra in men can lead to urinary morbidity including changes in urinary stream and dysuria, or in severe cases urinary retention

Clinical Risk Factors

LS may be associated with environmental factors and nonautoimmune comorbidities. The most commonly cited risk in men is lack of circumcision.52,53 Mallon et al noted an age-adjusted odds ratio of 53.55 (95% confidence interval [CI]: 7.24-395.88) in association to uncircumcised male patients.53 Injury to genital skin can increase the risk of LS as can friction, genital piercings for jewelry, and surgery.54,55 An investigation into LS and medical comorbidities demonstrated an association between

CONCLUSION

Although the etiology of LS remains unknown, infectious, anti-immune, or chronic irritation may all play a role in its presentation. Future research should explore the intersection of infections, skin microtrauma, and immune dysregulation leading to this disease state. Clinically, LS has the potential to progress to malignancy or cause debilitating changes to normal tissues. Topical corticosteroids are the current mainstay of medical treatment. Surgical therapy should avoid use of genital skin

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    Declaration of Interest: None.

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