Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

initial presentation

1st line – topical corticosteroid

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ACUTE
|
initial presentation
1st line – 

topical corticosteroid

The first-line treatment for anogenital lichen sclerosus (LS) is a very-high potency topical corticosteroid (e.g., clobetasol, ulobetasol, betamethasone dipropionate augmented).[34][35]​​​[56][61]​​​​

Treatment of extragenital LS should be overseen by a specialist who will individualise the approach according to the site involved, typically only initiating therapy if symptoms are bothersome to the patient. A very-high potency corticosteroid or a high potency topical corticosteroid (e.g., mometasone, triamcinolone) may be an appropriate initial therapy in these patients.[35][56]​ Treatment should be individualised according to the site involved.

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][83]

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]

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][85]​​​ 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 anatomical location and adverse effects are minimal when used as directed. 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).[86]​ 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.

  • 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.

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

  • Treatment should be individualised to the patient, based on their response.

  • 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).[82]

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 individualised 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][94]​​​ 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.[94]

Once initial disease control is achieved, maintenance therapy (usually with a topical corticosteroid) may be appropriate for prevention of flares.[82]

If initial treatment fails, it is important to rethink the diagnosis and consider a biopsy (if not obtained previously).[36][61]​​

Doses of topical corticosteroids presented here reflect licensed doses. Different doses may be used in practice for the treatment of LS (e.g., reduced dose frequency, treatment durations beyond the approved maximum duration of 2 weeks, lower maximum doses). Consult your local guidelines for more information.

Primary options

Very-high potency corticosteroid

clobetasol topical: (0.05% ointment) children ≥12 years of age and adults: apply to the affected area(s) twice daily for up to 2 weeks, maximum 50 g/week

OR

Very-high potency corticosteroid

ulobetasol topical: (0.05% ointment) children ≥12 years of age and adults: apply to the affected area(s) once or twice daily for up to 2 weeks, maximum 50 g/week

OR

Very-high potency corticosteroid

betamethasone dipropionate augmented topical: (0.05% ointment) children ≥13 years of age and adults: apply to the affected area(s) once or twice daily for up to 2 weeks, maximum 50 g/week

Secondary options

mometasone topical: (0.1% ointment) children ≥2 years of age and adults: apply to the affected area(s) once daily

OR

triamcinolone topical: (0.5% ointment) children and adults: apply to the affected area(s) two to four times daily

Plus – lifestyle measures and supportive care

Back
ACUTE
|
initial presentation
Plus – 

lifestyle measures and supportive care

Treatment recommended for ALL patients in selected patient group

​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.

Consider referring patients with pain, voiding issues, sexual dysfunction, functional impairment, or psychological distress to specialist services for supportive care (e.g., psychological therapy, analgesia, pelvic floor physiotherapy, surgery).[35][56]

Consider – emollient

Back
ACUTE
|
initial presentation
Consider – 

emollient

Additional treatment recommended for SOME patients in selected patient group

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]

2nd line – topical calcineurin inhibitor

Back
ACUTE
|
initial presentation
2nd line – 

topical calcineurin inhibitor

Refer patients with anogenital LS who have an allergy or contraindication to topical corticosteroids to a clinician who specialises 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]

  • Topical calcineurin inhibitors are not recommended during pregnancy or breastfeeding, but a specialist may choose to use these treatments if the benefits outweigh the risks.

  • 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 paediatric LS for further management.

  • There is a theoretical risk of malignancy in patients using topical calcineurin inhibitors.[84]​ The Food and Drug Administration (FDA) recognises 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.

Topical calcineurin inhibitors are not typically recommended for extragenital disease.

Once initial disease control is achieved, maintenance therapy may be appropriate for prevention of flares.[82]

If treatment fails, it is important to rethink the diagnosis and consider a biopsy (if not obtained previously).[36][61]

Primary options

tacrolimus topical: (0.03%) children ≥2 years of age and adults: apply to the affected area(s) twice daily; (0.1%) adults: apply to the affected area(s) twice daily

OR

pimecrolimus topical: 1%) children ≥2 years of age and adults: apply to the affected area(s) twice daily

Plus – lifestyle measures and supportive care

Back
ACUTE
|
initial presentation
Plus – 

lifestyle measures and supportive care

Treatment recommended for ALL patients in selected patient group

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.

Consider referring patients with pain, voiding issues, sexual dysfunction, functional impairment, or psychological distress to specialist services for supportive care (e.g., psychological therapy, analgesia, pelvic floor physical therapy, surgery).​[35][56]

Consider – emollient

Back
ACUTE
|
initial presentation
Consider – 

emollient

Additional treatment recommended for SOME patients in selected patient group

​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]

Consider – referral to urologist and consider circumcision

Back
ACUTE
|
initial presentation
|
men and boys with genital disease
Consider – 

referral to urologist and consider circumcision

Additional treatment recommended for SOME patients in selected patient group

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]

Consider – phototherapy or systemic therapy

Back
ACUTE
|
initial presentation
|
extragenital disease
Consider – 

phototherapy or systemic therapy

Additional treatment recommended for SOME patients in selected patient group

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 individualise the approach according to the site involved, typically only initiating therapy if symptoms are bothersome to the patient.

In patients with extragenital disease, a specialist may consider phototherapy (taking into account carcinogenicity and practicality), or a systemic therapy such as methotrexate or an oral retinoid (e.g., acitretin), although other systemic therapies may be used in practice.[35]

​Based on experience in practice, extragenital LS is typically more refractory to treatment than anogenital LS. However, extragenital disease is usually less symptomatic and there is no known association with progression to squamous cell carcinoma.

Primary options

acitretin: adults: consult specialist for guidance on dose

OR

methotrexate: adults: consult specialist for guidance on dose

Consider – intravaginal oestrogen

Back
ACUTE
|
initial presentation
|
pregnant or breastfeeding
Consider – 

intravaginal oestrogen

Additional treatment recommended for SOME patients in selected patient group

In women with anogenital LS who breastfeed, consider the use of an intravaginal oestrogen (e.g., estradiol) to offset the symptoms of oestrogen deficiency (such as dryness and irritation) that may be present following pregnancy and during the breastfeeding period.

Primary options

estradiol vaginal: (0.01%) adults: insert 2-4 g into the vagina once daily for 2 weeks, gradually reduce dose over 1-2 weeks to maintenance dose of 1 g once to three times weekly

ONGOING

response to initial treatment

1st line – individualised maintenance therapy

Back
ONGOING
|
response to initial treatment
1st line – 

individualised maintenance therapy

​Consider individualised 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][82]​​​ 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][82]​ 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 for anogenital LS is intended to reduce risks of long-term morbidity arising from scarring or development of squamous cell carcinoma (SCC).[82]

Extragenital LS is typically less symptomatic than anogenital LS and there is no known association with progression to SCC. 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, this should be guided by a specialist.

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 of corticosteroids when stopping treatment (topical corticosteroid withdrawal reactions).[86]​ 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.

Topical calcineurin inhibitors are not recommended during pregnancy or breastfeeding, but a specialist may choose to use these treatments if the benefits outweigh the risks. Topical calcineurin inhibitors are also not typically recommended for use in children <2 years of age. These patients should be referred to a specialist with expertise in paediatric LS for further management. There is a theoretical risk of malignancy in patients using topical calcineurin inhibitors.[84]​ The US Food and Drug Administration (FDA) recognises 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.

Plus – lifestyle measures and supportive care

Back
ONGOING
|
response to initial treatment
Plus – 

lifestyle measures and supportive care

Treatment recommended for ALL patients in selected patient group

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.

Consider referring patients with pain, voiding issues, sexual dysfunction, functional impairment, or psychological distress to specialist services for supportive care (e.g., psychological therapy, analgesia, pelvic floor physiotherapy, surgery).[35][56]​

Consider – emollient

Back
ONGOING
|
response to initial treatment
Consider – 

emollient

Additional treatment recommended for SOME patients in selected patient group

The use of emollients (e.g., white petrolatum) is recommended for affected areas in all patients with LS and may be continued following the acute phase to maintain improved condition of the skin.[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]

Consider – intravaginal oestrogen

Back
ONGOING
|
response to initial treatment
|
pregnant or breastfeeding
Consider – 

intravaginal oestrogen

Additional treatment recommended for SOME patients in selected patient group

In women with anogenital LS who breastfeed, consider the use of an intravaginal oestrogen (e.g., estradiol) to offset the symptoms of oestrogen deficiency (such as dryness and irritation) that may be present following pregnancy and during the breastfeeding period.

Primary options

estradiol vaginal: (0.01%) adults: insert 2-4 g into the vagina once daily for 2 weeks, gradually reduce dose over 1-2 weeks to maintenance dose of 1 g once to three times weekly

refractory disease

1st line – topical or systemic therapy + consider surgery (men or boys only)

Back
ONGOING
|
refractory disease
1st line – 

topical or systemic therapy + consider surgery (men or boys only)

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

​Patients with biopsy-confirmed anogenital 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 specialises 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.  

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

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

  • Intralesional triamcinolone injections​[56][61]

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

  • Methotrexate.[56]

In girls with refractory anogenital disease, a specialist may consider:[56][88][89][90]​​

  • A topical calcineurin inhibitor.[56]

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

  • Frenuloplasty in combination with intralesional triamcinolone injections

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

  • A topical calcineurin inhibitor[56]

  • An oral retinoid or methotrexate (in men only).

Topical calcineurin inhibitors may frequently be used for refractory disease either as monotherapy, or in addition to topical corticosteroids if corticosteroids alone are insufficient in maintaining disease control.

  • These agents are not recommended during pregnancy or breastfeeding, but a specialist may choose to use these treatments if the benefits outweigh the risks.

  • They are also not recommended for use in children <2 years of age. These patients should be referred to a specialist with expertise in paediatric LS for further management.

  • There is a theoretical risk of malignancy in patients using topical calcineurin inhibitors.[84]​ The US Food and Drug Administration (FDA) recognises that a causal relationship has not been established, while advising that the long-term safety of these drugs has not been established, and recommending limiting their use to affected areas and avoiding long-term use when possible. 

Systemic retinoids are for use in adults only by physicians experienced with their use, and they may only be available through a restricted distribution programme 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][87]

  • 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 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 of methotrexate include hepatotoxicity, nephrotoxicity, gastrointestinal toxicity, pulmonary toxicity, skin reactions, and myelosuppression.

Based on experience in practice, extragenital LS is typically more refractory to treatment than anogenital LS. However, extragenital disease is typically less symptomatic than anogenital LS and there is no known association with progression to squamous cell carcinoma. If extragenital LS is refractory to initial treatment and the patient finds their symptoms to be bothersome, ongoing therapy options (e.g., methotrexate) should be guided by a specialist.

Primary options

tacrolimus topical: (0.03%) children ≥2 years of age and adults: apply to the affected area(s) twice daily; (0.1%) adults: apply to the affected area(s) twice daily

OR

pimecrolimus topical: (1%) children ≥2 years of age and adults: apply to the affected area(s) twice daily

Secondary options

triamcinolone acetonide: children and adults: consult specialist for guidance on intralesional dose

OR

isotretinoin: adults: consult specialist for guidance on dose

OR

acitretin: adults: consult specialist for guidance on dose

OR

methotrexate: adults: consult specialist for guidance on dose

Plus – lifestyle measures and supportive care

Back
ONGOING
|
refractory disease
Plus – 

lifestyle measures and supportive care

Treatment recommended for ALL patients in selected patient group

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.

Consider referring patients with pain, voiding issues, sexual dysfunction, functional impairment, or psychological distress to specialist services for supportive care (e.g., psychological therapy, analgesia, pelvic floor physiotherapy, surgery).[35][56]

Consider – emollient

Back
ONGOING
|
refractory disease
Consider – 

emollient

Additional treatment recommended for SOME patients in selected patient group

The use of emollients (e.g., white petrolatum) is recommended for affected areas in all patients with LS and may be continued following the acute phase to maintain improved condition of the skin.[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]

Consider – intravaginal oestrogen

Back
ONGOING
|
refractory disease
|
pregnant or breastfeeding
Consider – 

intravaginal oestrogen

Additional treatment recommended for SOME patients in selected patient group

In women with anogenital LS who breastfeed, consider the use of an intravaginal oestrogen (e.g., estradiol) to offset the symptoms of oestrogen deficiency (such as dryness and irritation) that may be present following pregnancy and during the breastfeeding period.

Primary options

estradiol vaginal: (0.01%) adults: insert 2-4 g into the vagina once daily for 2 weeks, gradually reduce dose over 1-2 weeks to maintenance dose of 1 g once to three times weekly

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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