Lichen sclerosis (LS) is a chronic, relapsing disease with a variable presentation. In men, genitourinary LS may affect the penile foreskin, glans, meatus, and urethra. Treatment is multifaceted, ranging from pharmacotherapy to surgery. Urethral reconstruction due to stricture disease from LS is frequently plagued by a high recurrence rate. At the authors’ institution, the high recurrence rate has shifted their practice toward potent steroids and minimally invasive surgical techniques. Management of recurrence includes dilation, meatotomy/meatoplasty, 1-stage and 2-stage repairs. Recalcitrant cases may necessitate abandonment of most of the urethra resulting in a perineal urethrostomy.
Key points
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Lichen sclerosis is a chronic inflammatory skin disease with a variable presentation commonly affecting the anogenital area in both men and women.
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Management of urogenital lichen sclerosis is predicated on the extent of disease.
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Most patients can be treated with conservative therapies consisting of minimally invasive surgical techniques and potent topical steroids.
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Surgical intervention may be indicated when the disease process is extensive or recalcitrant to conservative therapy.
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Perineal urethrostomy is an attractive option with high patient satisfaction for extensive urethral lichen sclerosis in patients who are unwilling or unfit for 2-stage repair.
Introduction
Lichen sclerosis (LS), previously termed balanitis xerotica obliterans, is a chronic inflammatory skin disease commonly affecting the anogenital area in both men and women. In 1976, the International Society for the Study of Vulvovaginal Disease concluded that the terminology LS should be adopted for men and women. In men, it commonly manifests in excess of simple balanitis, hence the accepted change in terminology. The presentation of LS in both men and women is variable and ranges from a focal disease process to an extensive degree of involvement presenting in childhood or adulthood. It may extend beyond the glans penis and affect the penile shaft skin, urethral meatus, and urethra. Consequently, the diverse presentation means diverse treatment is necessary with variable success rates. A higher predilection of LS in women compared with men is well established. Urethral involvement of LS is largely seen in men, however. The extent of urethral disease may be limited to the urethral meatus or progress to panurethral involvement.
In this article, the authors describe the presentation, pathogenesis, epidemiology, and their current management algorithm for male patients with LS.
Introduction
Lichen sclerosis (LS), previously termed balanitis xerotica obliterans, is a chronic inflammatory skin disease commonly affecting the anogenital area in both men and women. In 1976, the International Society for the Study of Vulvovaginal Disease concluded that the terminology LS should be adopted for men and women. In men, it commonly manifests in excess of simple balanitis, hence the accepted change in terminology. The presentation of LS in both men and women is variable and ranges from a focal disease process to an extensive degree of involvement presenting in childhood or adulthood. It may extend beyond the glans penis and affect the penile shaft skin, urethral meatus, and urethra. Consequently, the diverse presentation means diverse treatment is necessary with variable success rates. A higher predilection of LS in women compared with men is well established. Urethral involvement of LS is largely seen in men, however. The extent of urethral disease may be limited to the urethral meatus or progress to panurethral involvement.
In this article, the authors describe the presentation, pathogenesis, epidemiology, and their current management algorithm for male patients with LS.
Presentation
Genital involvement with LS may present with local pruritus, dysuria, phimosis, paraphimosis, fissures, whitish skin, and bothersome lower urinary tract symptoms (LUTS) when the urethra is involved ( Fig. 1 ). Riddell and colleagues reported common symptoms in patients diagnosed with LS. Tight foreskin was noted in 25.8%, pruritus in 18%, painful erections in 13.6%, and cracking and bleeding in 9.1%. Up to 19.7% of patients reported difficulty passing urine, which raises concern for either meatal or urethral involvement. Chronic inflammatory changes may be associated with genital ulceration and superimposed infections. Significant scarring and genital deformation may be noted as a consequence.
Pathogenesis
LS is characterized microscopically by the presence of hyperkeratosis, thinning of the epidermis, loss of rete pegs, and collagen deposition in the dermis ( Fig. 2 ). A histiocytic or lymphocytic infiltrate is also noted and has led to the theory of an inflammatory cause. A variety of precipitating factors, including autoimmune processes, infections, and trauma, have also been suggested to contribute to the development of LS.
Autoimmunity
Immune-related dysregulation has been suggested as the cause of LS. Histopathologic findings of abnormal T-cell clones in the lymphocytic infiltrate of tissue affected by LS argue for autoimmune dysregulation as the underlying factor leading to pathogenesis. Attempts at identification of a putative antigen suggest that extracellular matrix protein 1 (ECM1) may play a role. Initially, this was suggested by the overlapping dermatologic clinical and histologic findings between lipoid proteinosis and LS. Lipoid proteinosis is an autosomal-recessive genetic disorder leading to loss of function of ECM1. Circulating antibodies to ECM1 have been noted in a higher proportion of patients with LS than controls. Debate still remains whether these circulating antibodies are due to exposure of the site-specific antigens from another inciting event, or whether circulating antibodies are the cause of the genital skin changes.
In addition, other studies have also suggested a genetic susceptibility and autoimmune basis to LS. Bjekic and colleagues, in a case control study of 73 patients with LS, noted an association between the presence of LS and prior genital injury, vitiligo, family history of alopecia areata, and thyroid gland disease. Azurdia and colleagues compared a cohort of biopsy-proven LS patients with controls and found an increased frequency of class II HLA antigens DR11, DR12, and DQ7, suggesting an immunopathogenesis for this disease.
Infection
An infectious pathogen has been suggested as a potential driving factor for LS based on histologic evaluation of skin biopsies. A few studies have linked spirochetes, Borrelia burgdorferi , and acid-fast bacilli to LS. However, others have attempted to confirm these findings and have failed to demonstrate a correlation in specimens of LS. At the current time, the authors think that the evidence does not support infection as the cause of LS.
Trauma
The Koebner phenomenon is described as dermal lesions arising from trauma and has been suggested as a cause for LS. Various reports suggest the development of LS after circumcision at suture lines as well as following sunburns and radiation therapy. It remains unclear if these events herald the development of the immunopathogenesis resulting in LS in susceptible patients.
Degeneration to Malignancy
Importantly, LS is a relapsing and progressive disease with reported degeneration to squamous cell carcinoma (SCC). However, a direct causation has not been reliably described. Most reports have identified LS changes in the background of SCC. No published prospective cohort of patients diagnosed with LS has been reported to then ascertain the incidence of subsequent SCC.
Depasquale and colleagues reported 522 men surgically treated for LS and noted a 2.3% rate of associated SCC. In this cohort, the indication for surgery was SCC and LS was a secondary finding. Given that many of the patients were referred for surgical treatment of SCC, these data do not necessarily suggest LS as a precursor to SCC. There are other reports associating LS with SCC. Barbagli and colleagues reported 130 men with surgically treated LS and noted an 8.4% rate of premalignant or malignant histopathological features on re-review of the pathologic specimens. Similarly, of 20 patients with confirmed penile carcinoma, Powell and colleagues noted that 50% of those patients had SCC in a background of LS. Last, Velazquez and Cubilla reported 68 patients with known SCC and found LS in 33% of the specimens. The authors have longitudinally followed a cohort of men with biopsy-proven LS. Of 68 patients with biopsy-proven LS followed for a mean of 36 months, there were no instances of development of SCC or premalignant lesions. To the authors’ knowledge, this is the only report to date that has longitudinally followed patients with biopsy-proven LS.
The current data are unclear regarding the role of LS in the subsequent development of SCC. Although the authors’ experience has not demonstrated degeneration to malignancy, longer follow-up interval with a larger number of patients will provide further insight into this critical question.
Epidemiology
The true prevalence of LS in men is likely underreported, because many affected individuals will have minimally symptomatic disease. In children presenting with phimosis for circumcision, pathologic analysis has shown that LS may be present in up to 20% to 30% of patients. An estimated 28% of men seen in an outpatient clinic diagnosed with LS by physical examination were asymptomatic. In 1971, Wallace reported an estimated prevalence of LS between 1 in 300 and 1 in 1000 in a cohort of men referred to a community-based dermatology clinic. The age of presentation has been reported highest in the third and fourth decade of life. However, in a large cohort of Department of Defense beneficiaries, the age distribution was more than double in the fourth and fifth decade of life compared with the first 3 decades. The highest prevalence was seen in men greater than 61 years old.
Management of urogenital LS is predicated on the extent of disease, and because of its variable presentation, this ranges from conservative therapy to surgical intervention. Depasquale and colleagues reported 428 men with LS as the primary disease process. In this cohort, 70.1% of patients had LS involving only the foreskin and glans, 4.9% the urethral meatus, and 20% the urethra. In patients with disease limited to the foreskin or glans circumcision, topical therapies may be sufficient. However, in cases of severe glanular, meatal, or urethral involvement, more aggressive surgical therapies are necessary.
Evaluation for penile/urethral disease
Although the diagnosis of LS is often from history and physical examination (see Fig. 1 ; Fig. 3 ), several skin disorders, such as scleroderma, penile intraepithelial neoplasia (previously known as erythroplasia of Queyrat and Bowen disease), leukoplakia, and Zoon balanitis, may present with similar signs and symptoms. Therefore, the authors think a confirmatory biopsy is imperative to rule out malignant and premalignant penile lesions and further guide therapy. Moreover, because the external manifestations of LS do not accurately predict the degree of urethral involvement, they recommend a LUTS evaluation in all patients.
In those patients with self-reported LUTS or with an elevated American Urological Association Symptom Index score, a retrograde urethrogram is the study of choice to evaluate for urethral involvement ( Fig. 4 ). In addition, cystoscopy can be performed, usually revealing a narrowed urethral lumen with pale mucosa. The presence of atypical cystoscopic findings, such as significant desquamation, focal nodular narrowing, and ulcerated or bleeding mucosa, may signify malignant transformation and warrant a biopsy.
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Cost-effective Strategies for the Management and Treatment of Urethral Stricture Disease
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