You use Therapeutics in Dermatology on a regular basis and you are pleased with it ?
Then help us improve it by

make a donation now!



MENU
Therapeutics in Dermatology
A reference textbook in dermatology

Lichen sclerosus

5 March 2019, by RODRIGUES A.

1 - ACKNOWLEDGEMENTS

Chapter written with the help of the EADV, the Fondation René Touraine and the Therapeutics in Dermatology

2 - OVERVIEW

Lichen sclerosus is an uncommon skin condition that causes the skin to become thin, whitened, and wrinkled, and can cause itching and pain. Untreated it can lead to permanent scarring of the areas affected.

It is a long-term problem that can present in women, men and children at any age. It is more frequent in women than men. Women after menopause have the highest risk, followed by young girls. It is difficult to know exactly how many people have the disease. The signs and symptoms may improve at puberty, but they will still need monitoring for disease activity.

Lichen sclerosus can develop on any skin surface, but it most often affects the genital and anal areas. In women it most commonly occurs in the vulva near the clitoris or on the labia (the inner and outer genital lips), and in the anal region. In men, it most commonly occurs on the glans (head) of the penis and the foreskin.

In about 15% of patients lichen sclerosus lesions develop outside the anogenital region, such as the thighs, breasts, wrists, shoulders and neck. It almost never appears on the face or hands.

3 - SYMPTOMS

Lichen sclerosus symptoms can range from very mild to quite severe. In mild forms of lichen sclerosus patients can have no symptoms, but what usually makes the person notice the disease is itching in the affected areas (usually the genital and anal regions). Itching can be severe and interfere with sleep. Other symptoms include soreness, pain, bleeding and changes in the appearance of the affected skin. Regarding the skin alterations, early in the disease small white spots can be noted and over time these spots grow into bigger patches. The skin becomes thin and crinkles, tearing and bruising easily, causing severe itching, with some patients also experiencing pain and bleeding. Without treatment the affected skin becomes scarred, this can ultimately alter the structure of the genital region.

In women, the most common symptom is vulvar itching. Due to the fragility of the involved skin, relatively minor rubbing or sexual intercourse may lead to bleeding. Often the lesions extend down and around the anus. When perianal skin is involved, tightening of the skin around the anus may cause discomfort when passing bowel motions and aggravate any tendency to constipation, especially in children. Other symptoms in the anal region include anal itching, fissures and bleeding. In the vulva, when cracking of the skin (fissuring) occurs repeatedly or the lesions progress to scarring, there can be a change in the normal appearance, with the outer and inner lips of the vulva fusing (sticking together) and covering the clitoris. This scarring can lead to narrowing of the vaginal opening and result in painful sexual intercourse and difficulties with urination. Lichen sclerosus is a skin disease only and does not affect the inner reproductive organs, such as the vagina and uterus.

In men, lichen sclerosus usually affects the tip of the penis and foreskin, with the development of withe spots that can be sore. Men who develop lichen sclerosus are usually uncircumcised (they have not had the foreskin of the penis removed). With time, the foreskin can become tight, shrunken, and scarred over the head of the penis. These changes may cause difficulty in retracting the foreskin and in passing urine. Erections may become painful.

Lichen sclerosus can also cause lesions outside the anal and genital areas, especially the upper body, breasts, and upper arms. The lesions look like small pearly white areas on the skin. Sometimes they occur in people who also have genital lichen sclerosus. Sometimes they occur without genital problems. Away from the genital area, patches of lichen sclerosus usually do not cause itch or other symptoms.

4 - CAUSES

No one knows what exactly causes lichen sclerosus. There is a type of inflammation within affected skin which causes changes to the structure of the affected skin, but it is not clear why this happens.

It is not due to an infection, which means the disease is not contagious and cannot be spread through contact, including sexual intercourse. It is not known if lichen sclerosus is hereditary, but it rarely occurs in relatives. Most likely it has a genetic and immune basis. People who are genetically predisposed to lichen sclerosus may develop symptoms after experiencing trauma, injury, or sexual abuse.

Disorders of the immune system, with an overactive immune system may play a role. People with lichen sclerosus are at greater risk of developing other autoimmune disorders, such as some types of thyroid disease, anemia, diabetes, alopecia areata, and vitiligo. There is a particular, association with autoimmune thyroid disease. Up to 3 in 10 people with lichen sclerosus also have an underactive thyroid gland. But so far lichen sclerosus has not been proven to be an autoimmune condition in itself.

The disease is more common in prepubertal girls and postmenopausal women, suggesting that hormonal changes influence the disease. However, treatments such as hormone replacement therapy or the application of testosterone or progesterone have not been shown to be effective for females with the disease.

5 - TREATMENT

Currently there is no cure for lichen sclerosus. The disease may be lifelong and potentially progressive. Appropriate treatment can stop the condition from worsening and prevent its complications. The goals of treatment are to relieve bothersome symptoms and to prevent the condition from worsening.

Lesions in the genital skin should be treated, even if they aren’t painful or itchy. Untreated, the lesions can scar, causing problems with urination or sexual intercourses. There is also a very small chance that skin cancer may develop in the lesions.

For genital lichen sclerosus, the best treatment is using enough steroid ointment to prevent all symptoms (such as itch or soreness) and suppress active disease. Even if there have never been symptoms, treatment is still necessary.

Strong steroid ointments (e.g., clobetasol propionate) are the mainstay of treatment for genital lichen sclerosus and are effective in the majority of women. They are used to stop the inflammation, soften the affected skin, reduce the itching and help to keep the disease from coming back. Irritation tends to ease after two weeks or so, but the skin may take about three months of treatment to look and feel better. Initial treatment has a duration of one to three months with daily application of the ointment for it to be effective. Even after the initial course, most women require lifelong "maintenance" therapy with either less frequent application of the strong steroid ointment or a switch to a less potent steroid, with or without symptoms. It is important to understand that although there may be warnings on the product about the use of topical steroids on genital skin, it is important to use an adequate amount to bring the disease under control and prevent its complications.

The skin may return to normal if lichen sclerosus is diagnosed and treated with a topical steroid at an early stage. If the appearance of the skin has already changed a lot, the changes may not reverse much with topical steroid treatment even though symptoms of itch and soreness are often relieved.

In women with abnormal fusion of the labia and/or scarring vaginal dilators can be used to stretch the skin to help restore normal function. Surgery may also be used in this situation. It is important to note that topical corticosteroids and dilators should be maintained after surgery to prevent the recurrence of scarring.

In cases where vulvar pain persists despite treatment with topical steroids could be addressed with self-massage and dilator therapy to reduce pain with sexual intercourse. Sometimes oral medication, such as tricyclic antidepressants or anticonvulsants, may be prescribed. The doses are typically much lower than those used for treating depression or other conditions.

Boys and men who have genital lichen sclerosus are generally treated with circumcision, which removes the foreskin of the penis. After circumcision, the disease does not usually come back.

Lichen sclerosus lesions outside the genital and anal regions usually go away after time without treatment.

If the disease starts in childhood, it usually goes away around the time of puberty.

Patients with lichen sclerosus should maintain a consult every six to 12 months in order to follow and treat any skin changes.

6 - TIPS FOR MANAGING

The good news for women who have been diagnosed with lichen sclerosus is that treatment with topical steroid ointments are very effective and if used early, prevent scarring. Maintenance treatment should be maintained even without symptoms. Follow-up at least once a year is important to detect skin changes due to the small risk of vulvar cancer. It is important to avoid irritants in the skin (irritating creams, unnecessary medications, soaps, and over-washing) and urine, as well as use an emollient to help control dryness in the genital region. Some irritants, such as sweat in overweight people, and urine in incontinent people, can be hard to avoid. Otherwise, the most common irritant is rubbing and scratching that should be controlled with topical steroid treatment. Patients should use cotton underwear. For boys and men circumcision is usually curative.

× N.B. : This limited content is for the general public. If you are a health professional, click here to register for free and gain access to a dedicated deeper content.
If you already have an account, log in!

Follow us

Newsletter

  Health professionals