Patient education: Vulvar lichen sclerosus (Beyond the Basics)

INTRODUCTION — Lichen sclerosus (LS) is a skin disorder that causes the skin to become thin, whitened, and wrinkled and can cause itching or pain.

LS can develop on any skin surface, but in females, it most commonly occurs near the clitoris, on the labia (the inner and outer genital lips), and in the anal region (figure 1). This is called "vulvar LS." In a minority of patients, LS lesions develop on other skin surfaces, such as the thighs, breasts, wrists, shoulders, or neck.

Vulvar LS usually occurs in postmenopausal females, although premenopausal females and children may be affected. It is not clear exactly how many people have vulvar LS. Estimates for vulvar LS vary from 1 in 30 older adult females seen in general gynecology offices to 1 in 300 to 1000 patients referred to dermatologists.

CAUSES AND RISK FACTORS — The cause of vulvar lichen sclerosus (LS) is not clear; health care providers suspect that a number of factors may be involved.

●Genetic factors – LS seems to be more common in some families. However, the role of genetics in LS is not fully understood.

●Disorders of the immune system – LS in females may be an autoimmune disorder, in which the body's immune system mistakenly attacks and injures the skin. Adult females with LS are at greater risk of developing other autoimmune disorders, such as some types of thyroid disease, anemia, diabetes, alopecia areata, and vitiligo.

●Infections – Researchers have not been able to clearly demonstrate any relationship between infections and LS. LS is not contagious.

●Hormones – Vulvar LS is more common in prepubertal females and postmenopausal females, 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 LS.

●Skin injury – People with vulvar LS sometimes develop symptoms in places where the skin has been injured, such as from physical trauma or sexual abuse.

●Urine – There is evidence that urine may contribute to genital LS in males, in that microscopic droplets of urine may pool between the glans penis and the foreskin, contributing to LS in uncircumcised males. Further research is needed to determine whether urine contributes to more severe or difficult-to-control vulvar LS in females.

SIGNS AND SYMPTOMS

Features of vulvar lichen sclerosus — Some people with vulvar lichen sclerosus (LS) feel dull, painful discomfort in the vulva, while others have no symptoms. The most common symptoms include:

●Vulvar itching – The most common symptom of vulvar LS is itching. It may be so severe that it interferes with sleep.

●Anal itching, fissures, bleeding, and pain – (See "Patient education: Anal fissure (Beyond the Basics)".)

●Painful sexual intercourse (dyspareunia) – This can occur as a result of repeated cracking of the skin (fissuring) or from narrowing of the vaginal opening due to scarring.

Typically, females with vulvar LS have thin, white, wrinkled skin on the labia, often extending down and around the anus (figure 1). Sometimes, however, the skin can actually appear thickened and rough, like a callus. Purple-colored areas of bruising below the skin may be seen. Cracks (also known as fissures) may form in the skin in the area around the anus, the labia, and the clitoris. Relatively minor rubbing or sex may lead to bleeding due to the fragility of the involved skin.

Vulvar LS may progress and change the appearance of the genital area as the outer and inner lips of the vulva fuse (stick together) and cover the clitoris. The opening of the vagina can become narrowed, and cracks, fissures, and thickened, scarred skin in the genital and anal area can make sexual intercourse or genital examination painful. LS does not affect the inner reproductive organs, such as the vagina and uterus.

DIAGNOSIS — Providers typically use the following methods to diagnose vulvar lichen sclerosus (LS).

History and physical examination — A medical history and physical examination of the vulvar and anal areas will be done, looking for the signs and symptoms of vulvar LS. A general skin examination may also be performed to exclude LS elsewhere on the body.

Biopsy — A skin biopsy can be taken to confirm a suspected diagnosis of vulvar LS; however, this is not always required. A small piece of the affected skin will be removed and sent to a pathologist to be examined with a microscope.

Excluding other conditions — An examination or tests may be done to exclude other conditions that could cause symptoms similar to those of vulvar LS, such as:

●Lichen planus – Lichen planus is a skin disease that can also cause itching, pain, and fusing of genital skin. Lichen planus can occur together with LS, when it is called lichen sclerous/lichen planus overlap.

●Low estrogen level – Vulvar changes related to a low estrogen level can occur alongside vulvar LS as both may develop in peri- or postmenopausal females. A lack of the hormone estrogen can contribute to painful intercourse and, in rare cases, can cause fusing of genital skin. (See "Patient education: Vaginal dryness (Beyond the Basics)".)

●Vitiligo – Vitiligo is a disorder that can cause white skin patches similar to those of LS. Vitiligo can occur together with LS.

●Vulvar dermatitis – Vulvar dermatitis is an itchy skin disorder that can cause severe itch, fissuring, thickening of the skin, and lightening or darkening of the skin. However, vulvar dermatitis does not cause white skin patches or scarring.

●Pemphigoid – Pemphigoid is a blistering skin disorder that also causes scarring of the vulva. This condition is rare.

●Infections – Infections can cause pruritus or discomfort but usually do not cause the typical skin changes of LS. However, infection can occur together with LS.

VULVAR LICHEN SCLEROSUS AND CANCER — Adults with vulvar lichen sclerosus (LS) are at a slightly increased risk for developing squamous cell skin cancer of the vulva.

Diagnosing vulvar LS early, treating it effectively, and biopsying any abnormal areas may help to reduce the risk of developing or missing a diagnosis of skin cancer. A once-yearly examination of the skin of the vulva is recommended, and women should examine themselves regularly (for example, monthly) for lumps or sores that do not heal. A biopsy should be performed if there are areas that do not improve with treatment. There is evidence that suggests that good control of vulvar LS may reduce the risk of vulval cancer.

LS lesions outside the genital area do not have an increased risk of cancer.

PAINFUL SEXUAL INTERCOURSE — Vulvar lichen sclerosus (LS) can lead to constriction of the vaginal opening and pain during sexual intercourse. People who experience pain during sex first require treatment to suppress any active disease.

Once the disease is controlled, some clinicians may recommend an estrogen cream to help to soften the skin around the vaginal opening. Devices called vaginal dilators, which patients can use at home, also may be used to slowly stretch the skin. Self-massage and dilator therapy may help to reduce pain with sexual intercourse. Rarely, surgical treatment is needed.

Pain with intercourse can also occur from other causes. Patients who notice pain during intercourse should discuss their symptoms with their health care providers.

LICHEN SCLEROSUS TREATMENT — The goals of treatment of vulvar lichen sclerosus (LS) are to relieve bothersome symptoms and to prevent the condition from worsening. A clinician may recommend medication for the physical symptoms and may refer the patient for support and therapy for other issues associated with the condition, such as problems with sex.

All patients with vulvar LS, even those without noticeable symptoms, need to use medication on a regular and ongoing basis. Patients also should see a health care provider for re-evaluation of the disease at least once or twice yearly.

Disease education — Patients who are diagnosed with vulvar LS should talk to their clinician about:

●The lifelong and potentially progressive nature of vulvar LS; appropriate treatment can stop the condition from worsening.

●Ways to manage the condition.

●The slightly increased risk of vulvar cancer and the need for ongoing monitoring.

●How to keep the genital area healthy and avoid scratching (table 1).

●Persistent pain with intercourse.

●Good vulval hygiene, including avoidance of irritant products (eg, soaps, douches, and body washes) and the use of a bland emollient (moisturizer).

Medications — Depending on the severity of the condition, a health care provider may recommend one or more of the following treatments for vulvar LS.

Topical steroids and steroid injections — Steroid ointments reduce inflammation and itching. They are the treatment of choice for genital LS. Strong steroid ointments (eg, clobetasol propionate) are the mainstay of treatment for vulvar LS and are effective in most patients.

Initial treatment usually requires daily application of the steroid ointment for a few months to resolve the symptoms and reduce inflammation. After the initial course, most patients require "maintenance" therapy with less frequent application of a steroid ointment.

Although there may be warnings on the steroid product about the use of topical steroids on genital skin, it is important to use an adequate amount to bring the disease under control. The health care provider will provide guidance about the amount to use and frequency of application.

Steroid injections are sometimes helpful when steroid ointments are not effective.

Other medications — Another class of topical medications are the calcineurin inhibitors (eg, tacrolimus or pimecrolimus), which are sometimes prescribed for patients who respond poorly to steroids or cannot tolerate steroid treatment.

An oral medication called acitretin has also been used for the treatment of LS. Because it has many side effects, the drug is used primarily in patients who have not been helped by other treatments. Acitretin can cause severe birth defects, and people treated with acitretin should not get pregnant during treatment or for three years after taking the drug. For this reason, acitretin usually is not recommended for people who can become pregnant.

Surgery — Some people with vulvar LS may develop abnormal fusion of the labia and/or scarring. Vaginal dilators can be used in this situation to stretch the skin to help restore normal function. Surgery may also be used in this situation. It is important to continue medical treatment (with steroid ointments) and dilators after surgery to prevent the recurrence of scarring.

WHAT TO EXPECT — The good news for patients who have been diagnosed with vulvar lichen sclerosus (LS) is that treatments such as topical steroid ointments are very effective. Thus, early treatment of vulvar LS with topical steroids can prevent scarring. Follow-up is important throughout the patient's lifetime.

WHERE TO GET MORE INFORMATION — Your health care provider is the best source of information for questions and concerns related to your medical problem.

This article will be updated as needed on our website (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for health care professionals, are also available. Some of the most relevant are listed below.

Patient level information — UpToDate offers two types of patient education materials.

The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.

Patient education: Lichen sclerosus (The Basics)
Patient education: Vulvar itching (The Basics)
Patient education: Lichen planus (The Basics)
Patient education: Vulvar pain (The Basics)

Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.

Patient education: Anal fissure (Beyond the Basics)
Patient education: Menopausal hormone therapy (Beyond the Basics)

Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.

Vulvar dermatitis
Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers
Cutaneous squamous cell carcinoma: Epidemiology and risk factors
Vulvar cancer: Epidemiology, diagnosis, histopathology, and treatment
Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia)
Vulvar lichen planus
Vulvar lichen sclerosus: Clinical manifestations and diagnosis
Vulvar pain of unknown cause (vulvodynia): Treatment
Overview of vulvovaginal conditions in the prepubertal child
Cutaneous squamous cell carcinoma (cSCC): Clinical features and diagnosis
Extragenital lichen sclerosus: Clinical features and diagnosis

The following organizations also provide reliable health information.

●National Institutes of Health

     (https://rarediseases.info.nih.gov/diseases/6905/lichen-sclerosus)

●National Vulvodynia Association

     (www.nva.org)

●The International Society for the Study of Vulvovaginal Disease

     (www.issvd.org)

●CareDownThere

     (https://www.caredownthere.com.au/)

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ACKNOWLEDGMENTS — The UpToDate editorial staff acknowledges Elizabeth G Stewart, MD, and Susan M Cooper, MB ChB, MRCGP, FRCP, MD, who contributed to earlier versions of this topic review.