Lichen sclerosis (lichen sclerosis et atrophicus, balanitis xerotica obliterans — lichen sclerosis of the penis) is an idiopathic inflammatory scarring dermatosis that affects the skin and mucosa, especially genital skin. It can be extremely itchy with scarring that may lead to significant functional impairment.

Lichen sclerosis (LS) affects all ages and races. It’s more common in females. The distribution is bimodal, with peaks in prepubertal children and post-menopausal women. The anogenital area is affected in 85% of cases, with extragenital LS being relatively rare. In patients with vulvar disease, LS is often the underlying cause.

LS is an idiopathic disease but may have some element of autoimmunity. In affected patients, circulating serum IgG autoantibodies to extracellular matrix protein-1 (ECM-1) have been detected. Some consider LS to be on a spectrum with morphea- localized scleroderma. Autoimmune diseases such as thyroid disease may be associated with LS.

It’s controversial whether genital LS should be called a “pre-malignant condition,” but it’s certainly amongst the commonest dermatoses associated with squamous cell carcinoma (SCC) of the genital skin — up to 5% of genital LS may lead to SCC. Although not a true pre-cancerous lesion, scarring in a moist environment is thought to predispose to malignancy.

Clinical findings

Genital LS can be very uncomfortable for patients with severe itch, dryness and burning pain. Initially, lesions are erythematous and partially eroded, and can appear “bruise-like.” These evolve into white atrophic plaques, with purpura, ecchymosis, erosions and sometimes blisters. In females, there’s often a “figure 8” configuration around the anus and vulva. In males, the glans and foreskin are affected, with sparing of the perianal area. Genital LS may present as phimosis (non-retractable foreskin) in males.

Extragenital LS is often asymptomatic but can present with itch and dryness. Patients may be concerned about the appearance. The trunk and upper limbs are favoured sites. Lesions begin as white, ivory, shiny papules that coalesce into plaques, becoming scar-like with atrophy. Sometimes hemorrhagic blisters, and telangiectasias are seen.

Complications

In genital LS, scarring is an important concern. In women, there can be burying of the clitoris, loss of labia minora, and narrowing of the introitus. In young girls, watch for constipation due to pain from defecation. In men, there can be meatal stenosis and phimosis. Urethral obstruction is rare.

Post-inflammatory pain syndromes sometimes follow clinical clearance of lesions. These syndromes can be challenging to manage as they don’t respond to topical anti-inflammatory treatment.

There’s also a risk of malignancy with genital LS, particularly when scarring is present.

Differential diagnoses

Genital LS:

• lichen planus
• squamous cell carcinoma in situ
• Zoon’s balanitis
• sexual abuse (in young girls and boys)

Extragenital LS:

• morphea

Diagnosis

A biopsy of the affected skin can confirm LS. Consider screening for other autoimmune conditions on history and physical, as well as laboratory tests — e.g. thyroid function tests.

Management

LS tends to come and go. The majority of patients less than 50 years will go into remission after 2-3 years, but in those over 70, complete remission is rare.

Educate patients to watch for changes suggestive of malignancy, and examine the site yearly in the office, unless there’s very little scarring. Consider stool softeners in young girls if there’s concern of constipation. Consider involving urology if there are concerns about urethral obstruction.

The goals of treatment are to reduce symptoms such as pruritus, and prevent complications. Emollients and barrier creams help prevent irritation, especially if there’s urinary incontinence. Tell patients to avoid harsh soaps and other irritants.

Highly potent topical corticosteroids are the mainstay of treatment. Clobetasol propionate 0.05% ointment is the strongest agent and can be applied nightly for 4 weeks, then tapering down to twice weekly over the next 2 months.

Response is noted with improvement in itch, and resolution of fissuring, erosions and bruising. White colouration and scarring will likely persist. Intralesional corticosteroids are sometimes used in more challenging cases.

Topical immunomodulators (e.g. pimecrolimus and tacrolimus) can provide some benefit, with the advantage of less atrophy compared to corticosteroids, but use with caution in LS because of the potential for squamous cell carcinoma in this condition.

For disease not responsive to topical therapies, and/or if the scarring is severe, surgical options are available. Circumcision can be curative in males.