Figure 1. Lichen sclerosus of the glans and surrounding penile skin.

Lichen sclerosus (LS) is a chronic and progressive inflammatory dermatologic condition that predominantly affects the genital and anal regions of both males and females. In men, genital LS may involve the glans and skin of the prepuce or penile shaft. Men may present with porcelain-like skin changes; presence of plaques, fissures, or erosions of the skin; pruritus; and pain with erections due to stretching and tearing of skin adhesions (Figure 1). This progressive scar-forming process may result in a spectrum of findings, ranging from superficial skin changes to phimosis and adult acquired buried penis (Figure 2). The exact etiology of LS remains unknown; however, numerous proposed theories exist linking the mechanism of LS to an infectious, autoimmune, traumatic, or genetic etiology. What is clearly understood is that chronic inflammation and irritation play a fundamental role in disease development and propagation.

Figure 2. Lichen sclerosus in setting of adult acquired buried penis.

In males, LS can result in the development of LS-induced urethral stricture disease (LS-USD). LS is believed to be the cause of USD in approximately 14%-29% of cases.¹ In addition to the physical stigmata of LS, patients with LS-USD will present with the characteristic symptoms of USD, including dysuria, obstructive voiding, splitting of the urinary stream, and painful ejaculation. Men with LS-USD range in severity from meatal stenosis to panurethral involvement (Figures 3 and 4). The classically described course of LS-USD is a distal-to-proximal stricture progression of the urethra up to the bulbomembranous junction. Although this describes the typical presentation of LS, isolated bulbar USD secondary to LS has also been described.² Such findings reinforce the notion that a clear understanding of the pathophysiological mechanisms of LS and LS-USD remains unidentified.

Figure 3. Lichen sclerosus of the glans with evidence of meatal stenosis.

The standard management of LS-USD spans from observation, topical treatments, urethral dilation, intermittent self-catheterization (ISC), and surgical intervention in the form of urethroplasty (single or multi staged with substitution grafting or perineal urethrostomy). Although urethroplasty has demonstrated success in treating non–LS-USD, surgery is fraught with intraoperative complexity, postoperative complications, and high recurrence rates ranging from 9%-71%.^3,4 Therefore, many patients and providers seek alternative approaches in the management of LS-USD.

Figure 4. Panurethral stricture disease of a patient with lichen sclerosus.

Given the inflammatory and scar-forming tendencies of LS, a cornerstone of conservative treatment is ISC with or without topical corticosteroids, most notably clobetasol propionate 0.05%. The ultimate benefit of corticosteroids remains to be determined. Treatment duration recommendations range from 6 weeks to 3 months. Alternatives such as betamethasone 0.05% and hydrocortisone 2.5%-10% have also been employed. Based on the postulated mechanism of a distal-to-proximal disease progression, past investigations have examined whether early and aggressive treatment of LS with topical corticosteroids may hinder disease progression and reduce the need for more invasive future treatments. Tausch et al reviewed male LS patients over 10 years, reporting that 7/17 men with mild meatal stenosis and distal preputial involvement were treated successfully with topical clobetasol ointment alone, while 6/10 patients experienced recurrence despite topical treatment. All 10 of these patients received circumcision and/or meatotomy and had no recurrence at a mean follow-up of 38.2 months.⁵

Potts et al examined the success of twice-daily ISC with intra urethral clobetasol.⁶ Overall success, which was defined as not requiring an escalation of care, was seen in 25/28 patients (89.3%) with a mean follow-up of 24.8 months. Only 3/28 patients were able to completely discontinue ISC. No patients went on to require urethroplasty. Although these findings demonstrate that continued ISC with corticosteroids is a favorable alternative to upfront urethroplasty, they do reinforce the progressive nature of LS and LS-USD and that ISC with corticosteroids remains only a temporizing measure.

Similarly, Rozanski et al retrospectively reviewed 112 patients with LS-USD who underwent conservative management with a mean follow-up of 30 months and a mean stricture length of 12 cm.⁷ Balloon dilation was performed in 100/112 (89%) patients. Intermittent self-catheterization was performed in 51/112 (46%) patients, of which 31% performed ISC with intraurethral steroids. Treatment escalation was avoided in 94/112 (84%) patients. Eighteen patients (16%) failed conservative management, of which, 16/18 underwent urethroplasty. Patients who performed ISC with and without intraurethral corticosteroid were also compared: 5/16 (31%) patients failed ISC with corticosteroids versus 8/35 (23%) patients failed ISC without corticosteroids. The authors also reported a significant improvement in patient-reported outcomes between their first and last clinic appointments while being managed conservatively. Lastly, the authors identified clinical characteristics associated with conservative management failure, such as a history of UTI, urosepsis, or acute urinary retention. Such information may aid providers in stratifying patients who require closer follow-up or escalation of care.

Aside from corticosteroids, topical calcineurin inhibitors, such as tacrolimus 0.1% ointment, have been used to treat genital LS, primarily for cutaneous manifestations of LS. Tacrolimus has been shown to benefit pediatric patients with LS and meatal involvement, improving urinary symptoms and resolving meatal stenosis 3 weeks following circumcision, but has not been well studied in the adult population.⁸

Novel treatments such as high-intensity focused ultrasound therapy, adipose-derived stem cells, and platelet-rich plasma have been utilized in the treatment of vulvar LS but have yet to find clinical application or utility in the treatment of male anogenital LS or LS-USD. Although these treatments may be limited to the external genitalia, they may prevent the propagation and progression of LS and the ultimate development of LS-USD.

Due to the lack of reliable conservative treatments for LS-USD, understanding its pathophysiology is critical to developing better nonsurgical strategies. Recent publications, such as Levy et al, have begun establishing the molecular pathophysiology of LS.⁹ Their investigations noted higher expression of inflammatory markers CD8, CCL-4, TNF-α, and IgG-4 in men with LS-USD. Furthermore, Cohen et al demonstrated that the urinary microbiomes of patients with LS and non-LS USD are different.¹⁰ The authors identified a trend towards greater microbiome diversity in patients with LS-USD compared to patients with non-LS USD. These investigations may lay the groundwork for future conservative interventions.

The challenges patients and providers face in the treatment of LS and LS-USD are well established. Although conservative and surgical interventions have shown success in the treatment of LS-USD, they are not without the risk of complication and failure. Further investigations are needed to identify more effective conservative interventions in the treatment of LS-USD.