Approach

Early diagnosis and intervention with treatment is important to prevent complications. Lichen sclerosus (LS) is usually a chronic condition with a relapsing and remitting course. Due to the chronic relapsing nature of anogenital LS, with scarring and risk of transformation into squamous cell carcinoma (SCC) when left untreated, lifelong maintenance therapy and follow-up of patients with anogenital disease is a key consideration; follow-up frequency and duration should be tailored to the individual patient.[84]​ Extragenital LS neither scars nor transforms into SCC. 

In most cases of LS, the goals of treatment are to:[56]

  • Decrease clinical signs of disease and inflammation, and improve the patient’s quality of life

  • Reduce disease flares

  • Reduce the risk of scarring, and reduce progression to SCC.

The first-line treatment for LS in all patients with anogenital disease is a very-high potency topical corticosteroid.​[34][35]​​​​​[56][61]​​​​

While very-high potency topical corticosteroids are only licensed for use in children ≥12 years of age, they are used in younger children under specialist guidance.[7][85]​​​​

All patients with LS should be managed by a clinician who is confident in treating the condition. Treatment of extragenital LS should be overseen by a specialist who will individualize the approach according to the site involved, typically only initiating therapy if symptoms are bothersome to the patient.

Recommended treatment frequency and duration varies between international guidelines and should be individualized to the patient, based on their response to treatment.

Once initial disease control is achieved, maintenance therapy (usually with a topical corticosteroid) may be appropriate for prevention of anogenital disease flares.[84]​ Patients with extragenital LS will usually not require maintenance therapy unless they find their symptoms to be troublesome. If maintenance therapy is required for extragenital LS, suitable therapy options should be guided by a specialist.

In genital LS affecting men and boys, intervention with circumcision, especially when carried out early, is often curative. However, a trial of a topical corticosteroid may be effective in the first instance.[56]

Anogenital lichen sclerosus: initial treatment

Women and girls

Commence treatment with a very-high potency topical corticosteroid (e.g., clobetasol, halobetasol, betamethasone dipropionate augmented), which should initially be used daily.[34][35]​​[36][56]

  • While very-high potency topical corticosteroids are only licensed for use in children ≥12 years of age, they are used in younger children under specialist guidance.[7][85]

  • Ointments are generally preferred to creams as creams may contain alcohol or preservatives that can cause burning or stinging of the affected skin in LS. Ointments provide better penetration and an increased barrier effect.[35]

Almost all patients with LS will have significant improvement in clinical signs and symptoms with a very-high potency topical corticosteroid. If initial treatment fails, it is important to rethink the diagnosis and consider a biopsy (if not obtained previously).[36][61]​​

  • Individual patients may require slightly different lengths of initial treatment regimen depending on the severity of their disease.

    • Exact treatment duration and frequency varies between international guidelines; some guidelines recommend daily treatment for 3 months; others recommend daily treatment for the first month, followed by tapering therapy such as alternate day therapy for a further 2 months.​[29][35][45][56]

    • There is often an improvement in symptoms within the first few weeks of treatment; however, the patient will need to continue treatment until signs of disease have resolved (which may take several more weeks).[84]

  • Refer patients who have an allergy or contraindication to topical corticosteroids to a clinician who specializes in inflammatory skin disease, specifically genital dermatoses, for consideration of other therapies such as a topical calcineurin inhibitor (e.g., tacrolimus, pimecrolimus), depending on patient comorbidities, disease severity, and patient preference.[56]

    • Note that topical calcineurin inhibitors are not typically recommended for use in children <2 years of age, but some specialists may use them in this age group. These patients should be referred to a specialist with expertise in pediatric LS for further management.

    • There is a theoretical risk of malignancy in patients using topical calcineurin inhibitors.[86]​ The Food and Drug Administration (FDA) recognizes that a causal relationship has not been established, while advising that the long-term safety of these drugs has not been established, and recommends limiting their use to affected areas and avoiding long-term use when possible.

Counsel the patient on the use of topical corticosteroids.

  • Demonstrate how much to use, which will differ depending on the extent of body surface area covered (e.g., a lentil or half pea-size amount may be appropriate in an adult woman with anogenital disease), and where to apply it - the use of photographs or having patients use a mirror during the consultation can be helpful.

  • Allay any fears stemming from corticosteroid phobia, which is frequently encountered in patients with LS.[42][87]​​​ Before initiation of topical corticosteroids, reassure the patient that:

    • Topical corticosteroids are safe and highly effective when an appropriate amount is used in the correct anatomic location and adverse effects are minimal when used as directed.

    • Using the correct amount will decrease symptoms as well as help to prevent future scarring; good control of the disease should decrease the chance of developing squamous cell carcinoma.

    • However, it is important to note that topical corticosteroids can rarely cause serious adverse effects including skin thinning, adrenal suppression, or Cushing syndrome, especially with prolonged use and use of very-high potency corticosteroids. Rarely, skin reactions have been reported by long-term users when stopping treatment (topical corticosteroid withdrawal reactions).[88]​ However, there are no documented cases of topical corticosteroid withdrawal reactions associated with vulval use. Advise patients to contact their care provider if they develop any noticeable adverse effects associated with topical corticosteroids.

Once disease signs are under control, consider the requirement for maintenance therapy with topical corticosteroids (see Anogenital lichen sclerosus: maintenance therapy, below).[35][61]​​​​​

Men and boys

In genital LS affecting men and boys, intervention with circumcision, especially when carried out early, is often curative. However, a trial of a topical corticosteroid may be effective in the first instance.[56]

Men or boys with LS involving the urethra who have issues related to voiding or sexual dysfunction should be referred for expert urology assessment and consideration for surgical intervention.[56]

Anogenital lichen sclerosus: refractory disease

Patients with biopsy-confirmed LS who have persisting signs and symptoms following initial treatment (typically 8-12 weeks) with topical therapies are considered to have refractory disease and should be referred to a clinician who specializes in inflammatory skin disease, specifically genital dermatoses.​[35][56][61]​​ The specialist will further investigate why the patient has not responded to initial therapies and may initiate second-line therapies if appropriate for the individual patient.

Women

In women with refractory anogenital disease, a specialist may consider:

  • A topical calcineurin inhibitor (e.g., tacrolimus, pimecrolimus)[56]

    • Frequently used as a second-line therapy or in addition to topical corticosteroids if corticosteroids alone are insufficient in maintaining disease control.

    • There is a theoretical risk of malignancy in patients using topical calcineurin inhibitors.[86]​ The FDA recognizes that a causal relationship has not been established, while advising that the long-term safety of these drugs has not been established, and recommends limiting their use to affected areas and avoiding long-term use when possible.

  • Intralesional triamcinolone injections[56][61]​​

  • An oral retinoid (e.g., isotretinoin, acitretin)[56][61]​​

    • Systemic retinoids are for use in adults only by physicians experienced with their use, and they may only be available through a restricted distribution program in some countries.

    • Systemic retinoids are teratogenic and are contraindicated in women who are or may become pregnant. All women of childbearing age should have pregnancy excluded before initiating treatment, and use effective contraception before, during, and after therapy (duration of contraceptive use after therapy depends on the retinoid used).[56][89]

    • Adverse effects can be severe. Severe headaches, decreased night vision, and signs of adverse psychiatric events necessitate prompt discontinuation.

    • Elevated serum cholesterol, triglycerides, and transaminases have been reported. A lipid panel and liver function tests should be monitored before and regularly during therapy.[56]

  • Methotrexate[56]

    • Methotrexate is teratogenic and is contraindicated in women with non-neoplastic diseases who are or may become pregnant. All women of childbearing age should have pregnancy excluded before initiating treatment, and use effective contraception during and after therapy.

    • Adverse effects include hepatotoxicity, nephrotoxicity, gastrointestinal toxicity, pulmonary toxicity, skin reactions, and myelosuppression.

Girls

In girls with refractory anogenital disease, a specialist may consider:[56][90][91][92]​​

  • A topical calcineurin inhibitor second-line or in addition to topical corticosteroids if corticosteroids alone are insufficient in maintaining disease control.

    • Topical calcineurin inhibitors are not recommended for use in children <2 years of age. These patients should be referred to a specialist with expertise in pediatric LS for further management.

Specific systemic therapies may be used in children with severe/refractory LS. However, the use of these therapies must be decided on a case-by-case basis after discussion with the patient and their family about the benefits and risks of these treatments. A discussion of these specific therapies is beyond the scope of this topic.

Men and boys

In men or boys with refractory anogenital disease, a specialist may consider:[56]

  • Frenuloplasty in combination with intralesional triamcinolone injections

  • Complete circumcision

    • Complete circumcision is usually curative in early male penile disease.[48]

  • A topical calcineurin inhibitor.

    • Topical calcineurin inhibitors are not recommended for use in children <2 years of age. These patients should be referred to a specialist with expertise in pediatric LS for further management.

In men, an oral retinoid or methotrexate may be considered.[56]

Specific systemic therapies may be used in children with severe/refractory LS. However, the use of these therapies must be decided on a case-by-case basis after discussion with the patient and their family about the benefits and risks of these treatments. A discussion of these specific therapies is beyond the scope of this topic.

Anogenital lichen sclerosus: interdisciplinary management

Consider referral for specialist services as indicated. In particular, refer patients who may require assistance with signs and symptoms of:[35][56]​​

  • Functional impairment due to scarring (i.e., sexual dysfunction or urinary tract symptoms due to narrowing of the introitus, anterior fusion, or posterior fourchette shelf), for consideration of other interventions (e.g., surgical lysis of adhesions in addition to topical therapies)

  • Psychological distress, for psychological support

  • Sexual dysfunction

    • Refer for pelvic floor physical therapy If the patient has or develops secondary pelvic floor dysfunction due to dyspareunia.[93][94][95]

    • Consider referral for psychosexual support.

  • Pain

    • If the patient has vulvodynia (vulval pain with no obvious underlying cause), refer to a pain specialist or vulval specialist.

  • Voiding issues

    • Refer to a urologist or urogynecologist.

Anogenital lichen sclerosus: maintenance therapy

Consider individualized maintenance therapy, usually with a topical corticosteroid, once disease control has been achieved in female patients with anogenital LS (and men who do not achieve control of the condition with circumcision), depending on the severity of disease, comorbidities, and patient preference.[35][84]​​ A topical calcineurin inhibitor may be an alternative option. The choice of maintenance therapy may depend on which treatment was used initially. It is recommended that patients requiring maintenance therapy are regularly reviewed by a specialist. See Monitoring.

Treatment should be reduced to the minimum potency and frequency required to control patient symptoms and clinical signs of disease.[35][61][84]​ For example, a very-high potency topical corticosteroid may be used at a reduced frequency of three times weekly, or a high-potency topical corticosteroid may be used on a daily basis. Lower potency topical corticosteroids may also be used in select patients depending on their response. Seek specialist advice regarding appropriateness, type and duration of maintenance therapy in children.​

Maintenance therapy is intended to reduce risks of long-term morbidity arising from scarring or development of squamous cell carcinoma.[84]

Extragenital lichen sclerosus

Owing to the scarcity of evidence to guide management of extragenital LS, optimal treatment is not well established. Treatment should be overseen by a specialist who will individualize the approach according to the site involved, typically only initiating therapy if symptoms are bothersome to the patient.

In patients with extragenital disease, options a specialist may consider include:

  • A very-high potency topical corticosteroid, or a high potency topical corticosteroid (e.g., mometasone, triamcinolone).[56][35]

  • Phototherapy, taking into account carcinogenicity and practicality.[35]

  • Methotrexate.[35]

  • An oral retinoid (e.g., acitretin).[35]

Specific systemic therapies may be used in children with severe/refractory LS. However, the use of these therapies must be decided on a case-by-case basis after discussion with the patient and their family about the benefits and risks of these treatments. A discussion of these specific therapies is beyond the scope of this topic.

Based on experience in practice, extragenital LS is typically more refractory to treatment than anogenital LS. However, extragenital disease is usually symptomatic and there is no known association with progression to squamous cell carcinoma. Therefore, patients with extragenital LS will usually not require maintenance therapy unless they find their symptoms to be troublesome. If maintenance therapy is required for extragenital LS or extragenital LS is refractory to initial treatment, suitable therapy options should be guided by a specialist.

Management in pregnancy

There are few reports regarding the course of LS during pregnancy, but in general LS rarely worsens during pregnancy and patients often find that their symptoms improve during the course of the pregnancy.[18]

If LS is first diagnosed during pregnancy, the authors of this topic recommend treatment with a very-high potency topical corticosteroid (high potency topical corticosteroids may also be used in some patients, with strength of the corticosteroid individualized to the patient, depending on the severity and extent of disease). Limited amounts of topical corticosteroid use during pregnancy have been shown to be safe.[34][96]​​​​ One study of 33 women treated with topical corticosteroids for LS during pregnancy found no corticosteroid-related adverse effects in any of the mothers or children.[96]

Continue maintenance therapy in pregnant patients as required with a high potency topical corticosteroid, with the minimum frequency required to control the diseases.[56]

Follow up in the postpartum period to ensure treatment is continued as appropriate.[56]

In women with anogenital LS who breast-feed, consider the use of intravaginal estrogen to offset the symptoms of estrogen deficiency (such as dryness and irritation) that may be present following pregnancy and during the breast-feeding period.

Note that topical calcineurin inhibitors are not recommended during pregnancy or breast-feeding, but a specialist may choose to use these treatments if the benefits outweigh the risks. Oral retinoids are contraindicated during pregnancy due to severe teratogenic effects.

Self management

Advise patients to avoid irritant and fragranced products.[35]

  • Instruct patients with anogenital disease to clean affected areas with only water if they can tolerate this. If the patient’s preference is to use a soap, counsel them to use a gentle formulation without allergens or irritants, and to apply this with their hands rather than with a washcloth.

    • Soaps, if used, should be entirely rinsed with water.[35][56]

    • Advise women and girls that soap and other cleansers should not be used internally.

The use of emollients (e.g., white petrolatum) is recommended for affected areas in all patients with LS as an adjunct treatment alongside standard therapies.[35][56]

  • Emollients may provide additional symptom relief, improve the skin barrier and provide resistance to external irritants.[56]

  • Note that emollients and topical corticosteroids should not be applied simultaneously, because doing so may dilute the agents.[56]

  • Emollients may be used as soap substitutes when washing extragenital areas.[35]

  • Use of emollients may be continued following the acute phase to maintain improved condition of the skin.[35][56]

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