What is lichen sclerosus?
Lichen sclerosus is chronic skin disorder that most often affects genital and perianal areas. It may cause no symptoms but it is often very itchy and sometimes sore. Lichen sclerosus usually persists for years, and can cause permanent scarring.
Lichen sclerosus (LS) was previously known as lichen sclerosus et atrophicus, and historically was also called kraurosis vulvae.
Who gets lichen sclerosus?
Lichen sclerosus is ten times more common in women than in men. It can start at any age, although it is most often diagnosed in women over 50. Pre-pubertal girls can also be affected.
It is estimated that around 15% of patients know of a family member that has been affected by lichen sclerosus.
People with lichen sclerosus often have a personal or family history of other autoimmune conditions such as thyroid disease (about 20% of patients), pernicious anaemia, or alopecia areata.
What causes lichen sclerosus?
The cause of lichen sclerosus is not fully understood and may include genetic, hormonal, irritant and infectious components. It is classified as an autoimmune disease. Autoimmune diseases are associated with antibodies to a specific protein. Extracellular matrix protein-1 (ECM-1) antibodies have been detected in 60–80% of women with vulval lichen sclerosus. Antibodies to other unknown proteins may account for other cases, explaining differing presentations of lichen sclerosus and response to treatment.
Lichen sclerosus can develop after an injury to the affected area. Male genital lichen sclerosus is rare in men circumcised at birth. It has been suggested that it may be caused by chronic, intermittent damage by urine.
What are the clinical features of lichen sclerosus?
Lichen sclerosus presents as white crinkled or thickened patches of skin that have a tendency to scar.
Vulval lichen sclerosus
In women, lichen sclerosus can be localised to one small area or extensively involve the perineum, labia majora, labia minora, fourchette and clitoris. Lichen sclerosus never affects mucosal tissue within the vagina itself.
Vulval skin affected by lichen sclerosus can be unbearably itchy (the symptom known as pruritus vulvae) and/or sore. Sometimes bruises, blood blisters and ulcers appear, after scratching, or from minimal friction (eg tight clothing, sitting down).
Sexual intercourse can be very uncomfortable and may result in painful splitting, also called fissuring of the posterior fourchette—this is the skin at the base of the vagina. This is because lichen sclerosus causes adhesions and scarring: sometimes the clitoris is buried (phimosis), the labia minora (inner lips) can shrink and the entrance to the vagina tightens (introital stenosis).
The skin around the anus (perianal skin) is also be affected by lichen sclerosus in about 50% of affected women. It may cause discomfort or bleeding when passing bowel motions, and aggravate any tendency to constipation, particularly in children.
Penile lichen sclerosus
In men, lichen sclerosus usually affects the tip of the penis (glans), which becomes firm and white (also called balanitis xerotica perstans). The urethra may narrow such that it is difficult to pass urine (urinary obstruction with meatal stenosis), resulting in a thin or crooked stream. The foreskin may become difficult to retract due to scarring (phimosis). Sexual function may be affected, because of painful erections or embarassment.
Other skin sites
Lichen sclerosus may also affect non-genital areas and does so in 10% of patients with vulval disease. This is called extragenital lichen sclerosus. Six percent of men and women with extragenital lichen sclerosus do not have genital lichen sclerosus.
One or more white dry plaques may be found on the inner thigh, buttocks, lower back, abdomen, under the breasts, neck, shoulders and armpits. Extragenital lichen sclerosus is described as looking like cigarette paper, with a wrinkled surface and waxy thickened feel. The hair follicles may appear prominent, containing dry plugs of keratin. Less often the plaques are scaly, bruised-looking, blistered or ulcerated. Extragenital lichen sclerosus is generally not itchy.
What are the complications of lichen sclerosus?
In females, vulval lichen sclerosus is associated with an increased risk of vulval cancer or anal cancer (squamous cell carcinoma, SCC). Cancer presents as a slowly growing lump, or a sore that fails to heal. It may affect up to 5% of patients with vulval lichen sclerosus and can be quite aggressive. Lichen sclerosus may also give rise to differentiated or usual-type (HPV-associated) vulval intraepithelial neoplasia (VIN).
Extragenital lichen sclerosus does not appear to predispose to cancer.
How is lichen sclerosus diagnosed?
Often a dermatologist or gynaecologist makes the diagnosis after a careful clinical examination. A skin biopsy is frequently recommended to confirm the diagnosis, as there are characteristic histopathological findings in lichen sclerosus. One or more biopsies may be taken to rule out other possible explanations for the skin condition such as dermatitis, lichen planus, vulval intraepithelial neoplasia or vulval cancer. Sometimes these disorders may co-exist with lichen sclerosus.
During follow-up, a specialist may decide to perform another biopsy to evaluate areas of concern or explain poor response to treatment.
What is the treatment for lichen sclerosus?
Patients are advised to become familiar with the location and appearance of their lichen sclerosus. It may be necessary for women to use a mirror to inspect the vulva. Many clinicians use photographs to monitor the activity of lichen sclerosus, and may provide their patients with copies, so that they are clear where to apply topical treatment. Patients should be advised to:
- Wash gently in a shower or bath with plain water alone or with a non-soap cleanser.
- Try to avoid rubbing and scratching.
- Apply emollients to relieve dryness and itching, and to protect sensitive skin, particularly in genital and anal areas.
- Follow instructions carefully and attend follow-up appointments regularly.
Topical steroid ointment
Topical steroids (especially clobetasol propionate ointment) are the main treatment for lichen sclerosus. Creams are used less often than ointments, as they are more likely to sting or to cause contact dermatitis. The topical steroid should be applied very accurately as a thin smear rubbed into the affected areas. Over-use of topical corticosteroids can result in skin thinning, red skin and discomfort; this is uncommon when applied accurately to lichen sclerosus. It is most important to follow instructions carefully and to attend follow-up appointments regularly.
Most patients will be told to apply the steroid ointment once a day initially. The doctor should reassess the treated area after a few weeks, as the response to treatment is quite variable.
The itch often settles within a few days but it takes weeks to months for the appearance to return to normal. Once lichen sclerosus has resolved, the topical steroid should be applied less often. It may need to be continued on a regular basis (perhaps once or twice a week) to prevent the lichen sclerosus recurring or to control on-going symptoms. In general, after initial more generous treatment, one 30-g tube is expected to last about 6 to 12 months.
If the first topical steroid is not well tolerated or is ineffective, another one should be used.
Other topical therapay
In most cases, occasional application of topical steroid is all that is required to control lichen sclerosus long term. But in a few patients, they are poorly tolerated or ineffective. Other topical treatments used for lichen sclerosus include:
Topical oestrogen cream or pessaries may be prescribed for atrophic vulvovaginitis (dry, thinned and sensitive vulval and vaginal tissues due to hormonal deficiency), and to prevent fissuring. Oestrogen can also reduce the itching and soreness of lichen sclerosus.
Topical calcineurin inhibitors tacrolimus ointment and pimecrolimus cream can be effective in lichen sclerosus on their own or in addition to topical steroids, but may be difficult to use because they tend to cause burning (at least for the first few days). There is also concern that these medications may have the potential to accelerate skin cancer growth in the presence of oncogenic human papilloma virus (the cause of genital warts and many cases of vulval and penile intraepithelial neoplasia), but this is unproven.
Topical retinoid cream is not well tolerated on genital skin but may be applied to other sites affected by lichen sclerosus. It reduces scaling and dryness.
When very lichen sclerosus is very severe, acute, and not responding to topical therapy, systemic steroids may be prescribed for a few weeks. These are usually effective, but have important side effects, so are not used long term for lichen sclerosus.
Other oral medicines reported to be successful in the treatment of lichen sclerosus include:
- Phototherapy has been reported to be effective for extragenital lichen sclerosus.
- Photodynamic therapy using methyl aminolevulinate has also been reported to be of benefit, but the procedure may be very painful.
- Experimentally, fat injections and platelet-rich plasma injected into affected areas have been reported to result in regeneration of normal skin.
Surgery for lichen sclerosus
In females with lichen sclerosus, a narrowed vaginal opening may need gentle stretching using vaginal dilators.
Surgical release of vulval and vaginal adhesions and scarring from vulval lichen sclerosus may occasionally be performed to reduce urination difficulties and allow intercourse. Procedures may include:
- Simple perineotomy (division of adhesions)
- Fenton procedure (an incision that is repaired transversely)
- Perineoplasty (excison of involved tissue and vaginal mucosal advancement)
Unfortunately, lichen sclerosus sometimes closes up the vaginal opening again after surgery has initially appeared successful. It can be repeated.
In males with penile lichen sclerosus, surgery is indicated if topical corticosteroids are ineffective, if there is scarring/phimosis, meatal stenosis, urethral stricture, carcinoma in situ or squamous cell carcinoma.
What is the outlook for lichen sclerosus?
There is no known cure for lichen sclerosus, although most people are substantially improved and quite comfortable with treatment. Active disease may go into remission, although it may recur at a later date. Extragenital lichen sclerosus is more likely to clear than genital disease.
Long-term specialist follow-up is recommended to assess the disease, optimise treatment and ensure early diagnosis of cancer.
- Bunker CB, Shim TN. Male genital lichen sclerosus. Indian J Dermatol. 2015 Mar-Apr;60(2):111-7. doi: 10.4103/0019-5154.152501. Review. PubMed PMID: 25814697; PubMed Central PMCID: PMC4372901.
- New Surgical Approach to Lichen Sclerosus of the Vulva: The Role of Adipose-Derived Mesenchymal Cells and Platelet-Rich Plasma in Tissue Regeneration. Casabona F, Priano V, Vallerino V, Cogliandro A, Lavagnino G. Plastic & Reconstructive Surgery. 126(4):210e-211e, October 2010. doi: 10.1097/PRS.0b013e3181ea9386
- British Association of Dermatologists' guidelines for the management of lichen sclerosus 2010, SM Neill, FM Lewis, FM Tatnall, NH Cox, BJD, Vol. 163, No. 4, October 2010 (p672-682) – British Association of Dermatologists
- Kirtschig G, Becker K, Günthert A, Jasaitiene D, Cooper S, Chi CC, Kreuter A, Rall KK, Aberer W, Riechardt S, Casabona F, Powell J, Brackenbury F, Erdmann R, Lazzeri M, Barbagli G, Wojnarowska F. Evidence-based (S3) Guideline on (anogenital) Lichen sclerosus. J Eur Acad Dermatol Venereol. 2015 Jul 22. doi: 10.1111/jdv.13136. [Epub ahead of print] PubMed PMID: 26202852.
On DermNet NZ:
- Lichen sclerosus pathology
- Genital skin conditions
- Pruritus vulvae
- Vulval cancer
- vulval intraepithelial neoplasia or VIN
- Lichen planus
- Erosive lichen planus
- Lichen sclerosus et atrophicus – Medscape Reference
- UK National Lichen Sclerosus Support Group
- Lichen Sclerosus (females) – British Association of Dermatologists
- Lichen Sclerosus (males) – British Association of Dermatologists
- Lichen sclerosus: the unspoken pain on Vimeo – Dr Andrew Goldstein talks about lichen sclerosus in a video presentation (1 hour 20 mins)
- Worldwide Lichen Sclerosus Support
- Supportgroep & Stichting Lichen Sclerosus Nederland
- The V Book: A Doctor's Guide to Complete Vulvovaginal Health
- The Vulvodynia Survival Guide: How to Overcome Painful Vaginal Symptoms & Enjoy an Active Lifestyle