Patient information: Lichen sclerosus

INTRODUCTION

Lichen sclerosus (LS) is a skin disorder that causes the skin to become thin, whitened, wrinkled, and can cause itching and pain (picture 1). LS usually occurs in postmenopausal women, although men, children, and premenopausal women may be affected. It can develop on any skin surface, but in women it most commonly occurs on the labia (the inner and outer genital lips) and anal region (figure 1). In 15 to 20 percent of patients, LS lesions develop on other skin surfaces, such as the thighs, breasts, wrists, shoulders, neck, and even the inside the mouth.

Although it is not clear exactly how many people have LS, it is estimated that between 1 in 300 to 1000 patients referred to dermatologists have LS.

LICHEN SCLEROSUS CAUSES AND RISK FACTORS

The cause of LS is not clear; healthcare providers suspect that a number of factors may be involved.

Genetic factors — LS seems to be more common in some families. People who are genetically predisposed to LS may develop symptoms after experiencing trauma, injury, or sexual abuse.

Disorders of the immune system — People with LS are at a greater risk of developing autoimmune disorders, which develop when the body's immune system mistakenly attacks and injures normal body tissues. These may include some types of thyroid disease, anemia, diabetes, alopecia areata, and vitiligo.

Infections — Researchers have tried to identify an infectious organism as a cause of LS, but no clear data have shown that there is an infectious source [1]. LS is not contagious.

LICHEN SCLEROSUS SIGNS AND SYMPTOMS

Feature of LS in women — Some women with LS feel dull, painful discomfort in the vulva, while other women with LS have no symptoms. The most common symptoms include:

• Vulvar itching — The most common symptom of LS is itching. It may be so severe that it interferes with sleep.
• Anal itching, fissures, bleeding, and pain (see "Patient information: Anal fissure")

• 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, women with LS have thin, white, wrinkled skin on the labia, often extending down and around the anus (figure 1). Purple-colored areas of bruising 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.

If lichen sclerosus is not treated, it may progress and change the appearance of the genital area as the outer and inner lips of the vulva fuse (stick together) and bury the clitoris (picture 2). 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.

LS may also cause lesions to occur in areas outside the genitals, especially the upper body, breasts, and upper arms. These lesions tend to be white, flat or raised, and are not as itchy as the affected skin of the genitals and anus.

Features of LS in men — In men, lichen sclerosus may appear on the head of the penis. Men who develop lichen sclerosus are usually uncircumcised (they have not had the foreskin of the penis removed), and the foreskin can become tight, shrunken, and scarred over the head of the penis. Men with lichen sclerosus may also have problems pulling back the foreskin and may experience decreased sensation at the tip of the penis, painful erections, or problems with urination [2].

LICHEN SCLEROSUS DIAGNOSIS

Providers typically use the following methods to diagnose lichen sclerosus.

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 lichen sclerosus.

Biopsy — To confirm a suspected diagnosis of lichen sclerosus, a biopsy is recommended. A small piece of the affected skin will be removed and sent to a pathologist to be examined with a microscope.

Excluding other conditions — Tests may be done to exclude other conditions that could cause symptoms similar to those of lichen sclerosus, such as:

• Lichen planus (a skin disease that can also cause itching and fusing of genital skin). Lichen planus can occur together with lichen sclerosus.
• Low estrogen level (a lack of the hormone estrogen can rarely cause fusing of genital skin but is often the cause of painful intercourse). Vaginal estrogen may be recommended to treat this condition. (See "Patient information: Postmenopausal hormone therapy", in the section on vaginal dryness).
• Vitiligo (a disorder that can cause white skin patches similar to those of lichen sclerosus). Vitiligo can occur together with lichen sclerosus.
• Pemphigoid (a blistering skin disorder that also causes scarring of the vulva) is extremely rare.
• Hemorrhoids (which can also cause cracks in the skin of the anus)

LS and cancer — Women with LS affecting the vulva are at increased risk for developing squamous cell skin cancer. However, it is not clear if women who are treated for LS are at the same risk as women who are not treated.

Diagnosing 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 lumps or sores that do not heal. A biopsy should be performed if there are areas that do not improve with treatment.

LS lesions outside the genital area do not have an increased risk of cancer. Men with LS that affects the skin of the penis have an increased risk of squamous cell skin cancer of the penis.

LICHEN SCLEROSUS TREATMENT

The goals of treatment of 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.

Most patients with LS, even those without noticeable symptoms, need to use medication on a regular and ongoing basis.

Patients who are diagnosed with LS should talk to their clinician about:

• The lifelong and potentially progressive nature of LS; appropriate treatment can stop the condition from worsening
• Ways to manage the condition
• The increased risk of vulvar cancer and the need for ongoing monitoring
• How to keep the genital area healthy and avoid scratching (table 1)

Depending on the severity of the condition, a healthcare provider may recommend one or more of the following treatments:

• Steroid ointments are recommended to reduce inflammation and itching. Steroid creams are not recommended because they contain ingredients that irritate the skin.
• Steroid injections, especially if steroid ointments are not effective
• Oral or topical tricyclic antidepressants (TCAs) are sometimes recommended for vulvar pain that persists despite steroids. The dose of TCAs is typically much lower than that used for treating depression. It is believed that these drugs reduce pain perception when used in low doses, although it is not clear how TCAs work.

TCAs commonly used for pain management include amitriptyline, desipramine, and nortriptyline. Patients beginning TCAs commonly experience fatigue; this is not always an undesirable side effect since it can help improve sleep when TCAs are taken in the evening. TCAs are generally started in low doses and increased gradually. Their full effect may not be seen for weeks to months.

Surgery is not routinely used to treat LS because lichen sclerosus tends to recur after the skin heals. However, patients whose genital tissues have grown together may have surgery to separate the fused tissues. Recurrence of the scarring occurs frequently.

Men who have lichen sclerosus are generally treated with circumcision, which removes the foreskin of the penis. After circumcision, LS does not usually come back.

WHAT TO EXPECT

The good news for patients who have been diagnosed with lichen sclerosus is that treatments such as topical steroid ointments are very effective. In one study of women and girls who were treated, 96 percent of patients showed improvement in their LS symptoms, and 66 percent of patients had relief of all LS symptoms. Thus, early treatment of LS with topical steroids can prevent scarring. Follow up is important throughout the lifetime.

SUMMARY

• Lichen sclerosus (LS) is a chronic skin problem that can develop on any skin surface, but most often affects the genital and anal regions. It is most common in postmenopausal women, but it can also occur in younger women, children, and men.
• Although the cause of LS is unclear, it seems to be more common in certain families. LS is not caused by an infection and it is not contagious. (See 'Lichen sclerosus causes and risk factors' above.)

• Symptoms of LS in women may include pain or itching in the vulvar and/or anal area, and the development of thin, white, wrinkled skin on and around the labia. Bruising or cracks (fissures) may also appear, and the fragile skin may bleed with rubbing or sexual activity. In men, LS can develop on the head of the penis, and usually affects the foreskin of uncircumcised males. (See 'Lichen sclerosus signs and symptoms' above.)

• If LS is not treated, it may worsen and cause permanent changes to the genitals.
• To diagnose LS, a healthcare provider conducts a medical history and physical examination; a biopsy can confirm the diagnosis and rule out other conditions that cause similar symptoms. Patients with LS affecting the genitals are at increased risk for developing vulvar cancer, and should be examined regularly for changes. (See 'Lichen sclerosus diagnosis' above.)

• Treatment can reduce symptoms and prevent the condition from progressing; most patients find that their symptoms improve with treatment over time. Steroid ointments or injections can help reduce inflammation and itching; pain may be treated with an oral medication. (See 'Lichen sclerosus treatment' above.)

WHERE TO GET MORE INFORMATION

Your healthcare provider is the best source of information for questions and concerns related to your medical problem. Because no two people are exactly alike and recommendations can vary from one person to another, it is important to seek guidance from a provider who is familiar with your individual situation.

This discussion will be updated as needed every four months on our web site (www.uptodate.com/patients). Additional topics as well as selected discussions written for healthcare professionals are also available for those who would like more detailed information.

Some of the most pertinent include:

Patient Level Information:
Patient information: Anal fissure
Patient information: Postmenopausal hormone therapy

Professional Level Information:
Dermatitis of the vulva
Differential diagnosis of vulvar lesions
Epidemiology and clinical features of cutaneous squamous cell carcinoma
Vulvar cancer: Clinical manifestations, diagnosis, and pathology
Vulvar intraepithelial neoplasia
Vulvar lichen planus
Vulvar lichen sclerosus
Vulvovaginal complaints in the prepubertal child

A number of web sites have information about medical problems and treatments, although it can be difficult to know which sites are reputable. Information provided by the National Institutes of Health, national medical societies and some other well-established organizations are often reliable sources of information, although the frequency with which they are updated is variable.

• National Institute of Arthritis and Musculoskeletal and Skin Diseases

      (www.niams.nih.gov/Health_Info/Lichen_Sclerosus/default.asp)

• National Lichen Sclerosus Support Group

      (www.lichensclerosus.org)

• National Vulvodynia Association

      (www.nva.org)

[2-5]