Lichen sclerosus

INTRODUCTION — Lichen sclerosus (LS) is a chronic, progressive skin disorder characterized by thin, white, wrinkled skin that is itchy and often painful (show picture 1). LS can develop on any skin surface, but usually is localized to the labia minora and/or labia majora (the inner and outer genital lips) and anal region (show 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.

Females are most often affected by LS before puberty and during the peri- or postmenopausal period. Men can also develop LS. Although it is not clear exactly how many people have LS, it is estimated between 1 out of 300 and 1 out of 1000 patients referred to dermatologists have LS.

POTENTIAL 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.

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. Several common symptoms of LS 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 minora and/or labia majora, often extending down and around the anus (show 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. The vaginal opening may appear yellow and waxy. 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 and structure of the genital area as the outer and inner lips of the vulva fuse (stick together) and bury the clitoris (show 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, and problems with urination [2].

DIAGNOSIS — Providers typically use the following methods to diagnose lichen sclerosus.

History and physical examination — A medical history and 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 always recommended. A small piece of the affected skin will be removed and 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) 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 vulvar cancer. In some cases, women may be diagnosed with both LS and cancer at the same time, even though they had no symptoms. For this reason, the skin of patients with vulvar LS should be examined at least yearly, but women should examine themselves regularly for lumps or sores that do not heal. Biopsy should be performed if there are thickened areas that fails to thin with treatment or there are persistently open or nonresolving lesions. LS lesions outside the genital area are not associated with an increased risk of cancer.

Men with LS that affects the skin of the penis have an increased risk of penile cancer.

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 sexual functioning.

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 progression 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 (show table 1)

Depending on the severity of the condition, a healthcare provider may recommend one or more of the following treatments: Topical steroid ointments reduce inflammation and itching. Steroid creams should be avoided because they contain ingredients that irritate the skin. Generic formulations are less desirable due to variations in the ingredients that may be less effective and more irritating. Steroid injections, especially if topical steroids are not effective Short-term retinoid treatments, which are derived from vitamin A, reduce scarring and inflammation, but are reserved for complicated cases. Tacrolimus ointment and/or pimecrolimus cream can be used to reduce itching, inflammation, and redness, but these medications have not been extensively studied and are not proven to stop scarring [3]. Oral or topical tricyclic antidepressants (TCAs) are sometimes recommended for vulvar pain that persists despite topical steroids. When used orally, 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, but the exact mechanism of their benefit is unknown.

TCAs commonly used for pain managment 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 inreased 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. Limited local excision (removal of the affected tissues) can cure vulvar cancer if it is detected early when the area is small.

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 PATIENTS CAN 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 377 patients (74 girls and 253 women), 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 could prevent scarring. However, the likelihood of LS symptom improvement depended upon the patient's age; younger patients have a greater chance for relief of symptoms [4,5]. Regardless of age, follow up in necessary throughout the lifetime.

SUMMARY Lichen sclerosus (LS) is a chronic skin disorder that can develop on any skin surface, but most often affects the genital and anal regions. Although the cause of LS is unclear, the disorder seems to be more common in certain families, suggesting a genetic component. LS is not caused by an infection and it is not contagious. (See "Potential 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 "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 cancer, and should be examined regularly for changes. (See "Diagnosis" above). The goals of therapy of LS are to 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 medication. (See "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 patients 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.patients.uptodate.com). Additional topics as well as selected discussions written for healthcare professionals are also available for those who would like more detailed information.

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. American Academy of Dermatology

(www.aad.org)
American Urological Association

(www.auanet.org)
National Institute of Arthritis and Musculoskeletal and Skin Diseases

(www.niams.nih.gov)
National Library of Medicine

(www.nlm.nih.gov/medlineplus/healthtopics.html)
National Lichen Sclerosus Support Group

(www.lichensclerosus.org)
National Vulvodynia Association

(www.nva.org)


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Use of UpToDate is subject to the Subscription and License Agreement. REFERENCES 1. Funaro, D. Lichen sclerosus: a review and practical approach. Dermatol Ther 2004; 17:28.
2. Questions and Answers about Lichen Sclerosus. National Institute of Arthritis and Musculoskeletal and Skin Diseases 2004. www.niams.nih.gov/hi/topics/lichen/lichen.htm (Accessed 8/4/06).
3. Kunstfeld, R, Kirnbauer, R, Stingl, G, Karlhofer, FM. Successful treatment of vulvar lichen sclerosus with topical tacrolimus. Arch Dermatol 2003; 139:850.
4. Cooper, SM, Gao, XH, Powell, JJ, Wojnarowska, F. Does treatment of vulvar lichen sclerosus influence its prognosis?. Arch Dermatol 2004; 140:702.
5. Renaud-Vilmer, C, Cavelier-Balloy, B, Porcher, R, Dubertret, L. Vulvar lichen sclerosus: effect of long-term topical application of a potent steroid on the course of the disease. Arch Dermatol 2004; 140:709.