Lichen Sclerosus

Synonyms: lichen sclerosus et atrophicus, balanitis xerotica obliterans, lichen albus, white spot disease, Csillag's disease, kraurosis vulvae

Lichen sclerosus (LS) is a chronic inflammatory dermatosis which usually affects the skin of the anogenital region in women, and the glans penis and foreskin in men (balanitis xerotica obliterans). It occurs less commonly in extra-genital areas. It does not cause any systemic disease outside the skin.

The cause is unknown:

• There is some evidence for autoimmune mechanisms in the aetiology.
• There is an association with other auto-immune diseases.
• Borrelial infection may contribute - this is uncertain.
• One reported case was linked to treatment with imatinib mesylate.¹

There are no reliable figures for population incidence or prevalence, but it is probably a relatively common disease.

• Women: there are peaks of incidence in prepubertal girls and post-menopausal women.
• Men: incidence is lower in males; the incidence peaks in young boys and again in adults.

The lesions are white thickened patches (porcelain-white papules and plaques). These may progress to crinkled white patches (like cigarette paper). Active lesions may have areas of ecchymosis, hyperkeratosis or bullae.

Anogenital LS³

Women

Symptoms:

• Itch - can be severe and disturb sleep.
• Pain can occur if there are fissures or erosions, leading to dyspareunia.
• Perianal lesions are common (about 30%) and may cause constipation.
• May be asymptomatic and found incidentally.

Signs:

• White lesions as above. These may be patchy, or in a figure-of-eight area around the vulva and anus.
• Destructive scarring may cause shrinking of the labia, narrowing of the introitus, or the clitoris may be obscured by adhesions. The vagina is not affected.
• In girls, the signs may be mistaken for sexual abuse.

Men⁵

Lesions are usually on the prepuce, gland penis and coronal sulcus.
Symptoms:

• Itching, soreness, haemorrhagic blisters.
• Dyspareunia, painful erections due to phimosis.
• If there is meatal scarring, poor urinary stream or dysuria.

Signs:

• White patches on the glans or prepuce.
• Haemorrhagic vesicles or purpura.
• Rarely, blisters or ulcers.
• If scarring has occurred - phimosis, wasting of the prepuce, meatal narrowing/thickening.
• Perianal disease is rare.
• In boys, may present as phimosis. Perianal involvement rarely (if ever) occurs.
• May be asymptomatic.

Other sites

• LS elsewhere is unusual; it is most often on the upper trunk, axillae, buttocks and lateral thighs.
• Oral lesions are extremely rare, but can affect sites where there is cornified stratified squamous epithelium, e.g. tongue, gingiva and hard palate. A case involving the tonsils was recently reported.⁶

• The diagnosis is usually made clinically.
• Biopsy:
  • Is indicated when there is diagnostic uncertainty or suspected malignancy.
  • Is not always practical, e.g. in children - it may be preferable to start treatment and to monitor response.
  • Is essential if lesions do not respond to adequate treatment.
• Skin swab to exclude infection, particularly if lesions are excoriated.³
• Blood tests:
  • Consider autoimmunity screen and thyroid function tests.

• In children, signs may mimic those of child sexual abuse. Note that a diagnosis of LS does not automatically exclude sexual abuse.²
• Various other skin, genital or mucosal conditions, including:
  • Vitiligo
  • Localised scleroderma
  • Lichen planus
  • Leukoplakia
  • Vulval intraepithelial neoplasia
  • Bowen's disease (squamous cell carcinoma in situ; if on the penis, this is Queyrat's erythroplasia)
  • Graft-versus-host disease

Where should patients be treated and followed up?²

• LS can be managed by a GP, dermatologist or gynaecologist, depending on local expertise and protocols. Referral is usually required to confirm the diagnosis.
• Specialist advice on treatment may be needed. Vulval clinics and urology clinics have a role, particularly if there are complications.
  The British Society of Dermatologists suggests an initial consultation plus 2 follow-up visits at 3 and 6 months, to ensure response to treatment and that patients are confident managing their condition.
• Long-term follow-up is needed for patients with poorly controlled LS.
• Patients who respond well to treatment and need only small amounts of topical steroids, should be reviewed annually - this can be in primary care. Give patients clear instructions to report immediately any persistent new lumps, skin changes, erosions or ulcers.

Female anogenital lichen sclerosus³

A reducing course of clobetasol propionate is the usual treatment:

• The usual regimen is - once-daily use for one month, alternate days for one month, then twice-weekly for one month with review at 2-3 months.
• If the patient's symptoms return during reduction of treatment, go back up to the frequency that was effective.
• A 30 g tube of clobetasol propionate should last 12 weeks; the patient is then reviewed.
• If the treatment has been successful, the hyperkeratosis, ecchymoses, fissuring and erosions should have resolved but the atrophy and colour change remain.
• Maintenance treatment may be required - either with less potent steroid preparations, or less frequent use of very potent steroids. For example, 30-60 g annually of clobetasol propionate is commonly required.²
• Oestrogen or testosterone creams should not be used to treat lichen sclerosus.⁹ Testosterone is no better than petroleum jelly and there may be adverse effects.¹⁰ It must not be used in children.
• Ointment bases are less allergenic, but the choice of base will depend on patient preference.
• For children, betamethasone dipropionate has been used successfully for vulval LS.²

Male anogenital lichen sclerosus⁵

• Use potent topical steroids, e.g. clobetasol propionate or betamethasone valerate, applied once-daily until remission, then gradually reduced.
• May need intermittent use, e.g. once weekly, to maintain remission.
• Topical mometasone furoate has been used successfully in children with LS of the glans penis.

Other treatments for anogenital lichen sclerosus^3,5

• Treat any secondary infection.
• Advice to patients:
  • Wash with bland emollients, e.g. aqueous cream; avoid topical irritants and tight clothing; use lubricants if necessary; give details of support groups.
  • Warn patients to seek medical attention if there are possible signs of malignancy, i.e. if the area develops a persistent lump, change in texture of the skin (such as thickening) or a non-healing ulcer/erosion (see Complications and their treatment below).
  • If relevant, advise which creams/ointments may be used with condoms.
• If there is apparent treatment failure, consider:
  • Compliance: for example, patients may be deterred by side-effect warnings on steroid preparations; elderly patients may have difficulty applying the creams.
  • Is the diagnosis correct? There may be an additional problem, e.g. infection or allergy to the preparation.
  • Is there a complication? (See Complications and their treatment below.)
• Research and unlicensed treatments (for use by specialists only):³
  • Topical calcineurin inhibitors may be effective, but there are safety concerns.
  • Oral retinoids may have a role in complex or severe cases (pregnancy is an absolute contra-indication).
  • Cryotherapy is sometimes helpful.²
  • UVA1 phototherapy has been successful in a few cases.
  • Intralesional steroids.⁴

Extragenital lichen sclerosus

Clobetasol propionate is first-line treatment,² e.g. once-daily for 2-3 weeks and repeated as required.¹¹

Asymptomatic patients

Treatment is recommended if patients have features of active disease, e.g. ecchymosis, hyperkeratosis or progressive atrophy.²

• Scarring:
  • This is not uncommon (see Presentation above).
  • Scarring may cause urinary symptoms or sexual dysfunction.
  • Renal failure due to obstructive uropathy has been reported.¹²
  • May require surgery, e.g. circumcision, meatal dilatation or vulval surgery.
• Constipation due to perianal fissures - prescribe softening laxatives.
• Squamous cell carcinoma (SCC):
  • There is a small risk of SCC of the vulva (≤5% lifetime risk²), and there may be an increased risk of SCC of the penis. Long-term follow-up is advisable.
  • Warn patients about signs of malignancy and biopsy any suspicious lesions.
  • Extra-genital lesions do not appear to have any increased risk.
• Dysaesthesia:
  • Vulvodynia or penile dysaesthesia can occur following inflammatory conditions of the genitalia. This is a neuropathic type of pain. Treat with xylocaine ointment or amitriptyline.
• Sexual dysfunction:
  • Be aware this may be a complication; offer referral.

• Some patients have complete remission after a course of treatment.
• In males, a course of steroids may prevent the need for circumcision.
• However, in some cases LS recurs. More complex cases may be an overlap syndrome with lichen planus, and may be more difficult to treat.