Lichen Sclerosus

What is Lichen Sclerosus?

Lichen sclerosus is a chronic inflammatory skin condition of the skin and mucus membranes that mainly affects the genital area including around the anus. The condition is not contagious. Treatment is essential to control symptoms, and prevent tissues from scarring, destruction, wasting, and a loss of function. Generally, lichen sclerosus is a lifelong condition, although it may resolve on its own in children. Moreover, quality of life is affected by, for example, itching, embarrassment, painful sex, and fear of skin cancer.

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Who gets Lichen Sclerosus?

Lichen sclerosus affects the genital area in about 85% of cases, other (extragenital) areas in 15–20% of cases, and in both genital and extragenital areas in less than 10% of cases. The prevalence of lichen sclerosus is estimated to be 0.1% to 0.3% of all individuals, although it is thought to be under-estimated. It occurs in more females than males (between one male in every 3 to 10 females), in both children and adults (mainly adult females), and may first occur at any age. The condition mainly starts in children immediately before puberty, in women post-menopause, and in men aged 30–40 years.

What causes Lichen Sclerosus?

The cause of lichen sclerosus is also unknown. Of the different factors previously implicated, genetic and autoimmune dispositions (particularly in females) appear to be most likely. The importance of hormonal factors is less favoured, and cases of infection are inconsistent.

Who is at risk of Lichen Sclerosus?

Several factors have been suggested to increase the risk of lichen sclerosus, including:

• Children just before puberty
• Post-menopausal women
• Men aged 30–40 years
• A family history of lichen sclerosus
• One or more autoimmune diseases
• Men who have not been or incompletely circumcised
• Covered moist skin environments
• Previous skin trauma
• Skin contact with urine, which is an irritant
• Women using oral contraceptives containing both oestrogen and progesterone.

What are the signs and symptoms of Lichen Sclerosus?

Lichen sclerosus in the genital and anal region is initially itchy, and may cause soreness, pain, redness, painful sexual intercourse, burning/stinging, difficulty urinating, and constipation (in children). The condition appears as whitish lesions that can merge to form plaques. Ulcers, blisters, cracks and tears, noticeable fine blood vessels, bruising, and/or bleeding may occur later. Skin becomes fragile, thickened and wrinkled. Genital lichen sclerosus can lead to skin cancer, which appears as lumps, ulcers and crusty areas.

In females, a figure-of-eight pattern is often seen when both genitals and anus are affected. Progression of the condition can lead to scarring, shrinkage of external genital parts, and genital structural changes. In males, lichen sclerosus usually affects the edge of the foreskin or head of the penis but can affect the penis shaft and skin of the testicles. It can cause difficulties in or inability to retract the foreskin.

Extragenital lichen sclerosus is commonly found on the upper body, under the breasts, neck, shoulders, armpits, wrists, and thighs. This form often arises in areas of skin that are pressure points, previous trauma sites, and surgical scars. Extragenital lichen sclerosus may initially appear with no symptoms or be only slightly itchy, with papules that merge together. Blisters, noticeable blood vessels, ulcers, erosions, and bleeding, developing into white plaques of scar tissue.

How is Lichen Sclerosus diagnosed?

The diagnosis of lichen sclerosus is usually based on symptoms and by examining the skin. Sometimes, a biopsy (skin sample) is needed to view under the microscope. This may be because lichen sclerosus can be difficult to diagnose, to check it has not become cancerous, or because it has not responded to initial treatment.

Lichen sclerosus can be confused with other disorders, mainly vitiligo, lichen planus, lichen simplex chronicus, and other conditions causing blistering. In girls pre-puberty, lichen sclerosus has been misidentified as sexual abuse.

How is Lichen Sclerosus treated?

Treatment of lichen sclerosus is important to relieve symptoms such as itch, and stop the skin from shrinking and becoming scarred, distortion of the genitals, and the affected skin from turning cancerous. However, treatment does not reverse any scarring or structural changes.

Treatment includes general skin care, by using of soap substitutes and moisturising emollients to relieve itchiness and provide a barrier against skin contact with urine.

Lichen sclerosus is generally treated successfully with topical potent and ultrapotent corticosteroids that supress the inflammation. If the condition does not respond to corticosteroids or the individual cannot use them, then medicines known as topical calcineurin inhibitors are used. Other treatments such as phototherapy (particularly for extragenital lichen sclerosus), retinoids, and methotrexate are only used in difficult to treat cases where corticosteroids and calcineurin inhibitors have failed.

Generally, males respond to circumcision when the condition does not respond to corticosteroids or there has been structural changes to the penis. In uncomplicated early-stage cases of lichen sclerosus, complete circumcision can result in permanent remission and cure.

Surgery may be required to treat the complications associated with lichen sclerosus. Reasons for surgery may be to stop any scar tissue affecting normal functioning (i.e., difficulties with urination and sexual intercourse) or address any malignancy.

How can a person with Lichen Sclerosus help themselves?

Self help measures include:

• General skin care, by using soap substitutes, other non-irritant skin products, and moisturising emollients
• Reduce skin contact with urine by drying the genital area after passing urine, and by applying moisturiser as a barrier between skin and urine.
• Avoid the use of rough or moist toilet paper, abrasive flannels and towels, and rubbing the area.
• Avoid wearing tight clothing and chaffing of the skin.
• Use lubricants if sexual intercourse is painful.
• Check the skin for new patches of lichen sclerosus and, especially if the condition has been present for years, for changes that might indicate cancer.
• Tell the doctor if the patches of lichen sclerosus are not responding to corticosteroid treatment or there are any adverse changes in the skin.

References

Arif T, Fatima R, Sami M. Extragenital lichen sclerosus: A comprehensive review. Australas J Dermatol. 2022 Nov;63(4):452-462. doi: 10.1111/ajd.13890.

De Luca DA, Papara C, Vorobyev A, Staiger H, Bieber K, Thaçi D, Ludwig RJ. Lichen sclerosus: The 2023 update. Front Med (Lausanne). 2023 Feb 16;10:1106318. doi: 10.3389/fmed.2023.1106318

Chamli A, Souissi A. Lichen Sclerosus. [Updated 2023 Jul 31]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK538246/

Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013 Feb;14(1):27-47. doi: 10.1007/s40257-012-0006-4.

Kirtschig G. Lichen Sclerosus-Presentation, Diagnosis and Management. Dtsch Arztebl Int. 2016 May 13;113(19):337-43. doi: 10.3238/arztebl.2016.0337.