- •
Lichen sclerosis is a chronic inflammatory skin disease with a variable presentation commonly affecting the anogenital area in both men and women.
- •
Management of urogenital lichen sclerosis is predicated on the extent of disease.
- •
Most patients can be treated with conservative therapies consisting of minimally invasive surgical techniques and potent topical steroids.
- •
Surgical intervention may be indicated when the disease process is extensive or recalcitrant to conservative therapy.
- •
Perineal
Effect of Lichen Sclerosis on Success of Urethroplasty
Section snippets
Key points
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 colleagues5 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
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.6, 7 A variety of precipitating factors, including autoimmune processes, infections, and trauma, have also been suggested to contribute to the development of LS.
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.23, 24, 25 An estimated 28% of men seen in an outpatient clinic diagnosed with LS by physical examination were asymptomatic.5 In 1971, Wallace26 reported an estimated prevalence of LS between 1 in 300 and 1 in 1000 in a cohort
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.4 Moreover, because the external
Management of genital lichen sclerosis
The 3 overarching goals of management are alleviation of symptoms, prevention and treatment of urethral stricture disease, and prevention and detection of malignant transformation. In their practice, the authors have developed 3 additional goals to also address improving quality of life:
- 1.
Unobstructed voiding
- 2.
Painless intercourse
- 3.
Adequate cosmesis32
As a result, the authors have shifted their paradigm in the management of LS. Most patients can be treated with minimally invasive therapies, including
Two-stage repair
Extensive cases of urethral stricture disease due to LS with an inadequate native urethral plate for primary one-stage repair represent a reconstructive challenge. In these cases, a staged surgical approach often yields better outcomes than single-stage reconstruction.41
The first stage of this approach, as described by Barbagli, through a midline penile incision, involves complete excision of all affected urethra and full opening of the glans. Buccal mucosa is harvested and grafted to the
Recurrence after 2-stage repairs
The success rates for 2-stage repairs are traditionally lower when compared with uncomplicated urethral reconstruction, due to a variety of factors, including the extensive nature of preoperative urethral stricture disease and the recurrent nature of LS. Kulkarni and colleagues43 demonstrated a recurrence rate of 27% at a mean follow-up time of 43 months for 2-stage buccal urethral reconstruction.
Peterson and colleagues44 reported a series of 63 patients with LS, 19 who underwent first-stage
Perineal urethrostomy
Perineal urethrostomy (PU) is a recognized alternative for proximal diversion in the nonsalvageable urethra. PU as the final point in management of an obstructed lower urinary tract is becoming more acceptable in men with LS and is an attractive option for patients with extensive urethral LS who may be unwilling or medically unfit to proceed with a 2-stage repair. Prior reports suggest patient satisfaction with a PU is acceptable.41, 44 Barbagli and colleagues45 recently reported their
Recurrence with perineal urethrostomy
There is a wide range of reported recurrence for PU (72%–100%).43, 44 In his series of 173 patients, Barbagli reports a 70% success rate. Even though some patients needed up to 5 additional procedures, nearly all (97%) were satisfied with PU and would choose it again.45 Peterson and colleagues44 found that almost half of planned 2-stage repairs (8/19, 42%) elected to not proceed to the second stage, rendering them with a functional PU without significantly affecting quality of life.
Morey and
Summary
LS is a progressive disease with a varied presentation and can be a challenging problem to manage. Localized disease to the foreskin and glans can be treated with potent topical steroids or circumcision. Urethral involvement occurs in 20% of patients. The extent of urethral involvement ranges from meatal only to panurethral. Intraurethral steroids with self-calibration may provide symptomatic relief to a large proportion of patients. Urethroplasty in this population has a higher risk of
References (46)
- et al.
Lichen sclerosus
Lancet
(1999) - et al.
Lichen sclerosus: review of the literature and current recommendations for management
J Urol
(2007) Immune dysregulation in lichen sclerosus
Eur J Cell Biol
(2005)- et al.
Autoantibodies to extracellular matrix protein 1 in lichen sclerosus
Lancet
(2003) - et al.
Penile carcinoma in patients with genital lichen sclerosus: a multicenter survey
J Urol
(2006) - et al.
Lichen sclerosus et atrophicus causing phimosis in boys: a prospective study with 5-year followup after complete circumcision
J Urol
(1994) - et al.
Lichen sclerosus: epidemiological distribution in an equal access health care system
J Urol
(2011) - et al.
Lichen sclerosus and isolated bulbar urethral stricture disease
J Urol
(2014) - et al.
Intraurethral steroids are a safe and effective treatment for stricture disease in patients with biopsy-proven lichen sclerosus
J Urol
(2016) - et al.
Early aggressive treatment of lichen sclerosus may prevent disease progression
J Urol
(2012)
Fossa navicularis reconstruction: impact of stricture length on outcomes and assessment of extended meatotomy (first stage Johanson) maneuver
J Urol
Buccal mucosa graft urethroplasty for anterior urethral stricture repair: evaluation of the impact of stricture location and lichen sclerosus on surgical outcome
J Urol
Outcomes for management of lichen sclerosus urethral strictures by 3 different techniques
Urology
Heroic measures may not always be justified in extensive urethral stricture due to lichen sclerosus (balanitis xerotica obliterans)
Urology
Clinical outcome and quality of life assessment in patients treated with perineal urethrostomy for anterior urethral stricture disease
J Urol
The “7-flap” perineal urethrostomy
Urology
New nomenclature for vulvar disease
Obstet Gynecol
Balanitis xerotica obliterans–a review
World J Urol
Clinical features of lichen sclerosus in men attending a department of genitourinary medicine
Sex Transm Infect
Lichen sclerosus in adult men: a study of HLA associations and susceptibility to autoimmune disease
Br J Dermatol
Lichen sclerosus et atrophicus and related disorders
AMA Arch Derm Syphilol
Risk factors for genital lichen sclerosus in men
Br J Dermatol
Histologic observations of pleomorphic, variably acid-fast bacteria in scleroderma, morphea, and lichen sclerosus et atrophicus
Int J Dermatol
Cited by (0)
Disclosure Statement: There are no conflicts of interest. No sources of external funding to be reported.