Information on the following diseases can be found in the Related Disorders section of this report:

• Scleroderma
• Lichen Planus
• Carcinoma of the Vulva
• Hyperplastic Dystrophy
• Paget's Disease of the Vulva
• .

Lichen sclerosus usually affects the external genitalia (i.e. vulva, vagina, or penis) and/or the area around the anus (perianal region). Sometimes, it is accompanied by intense (intractable) itching. If the disease is severe, even minor abrasions or chaffing can cause bleeding, tearing, and blistering. For this reason, many affected women and men avoid sexual activity, tight undergarments, and such activities as riding bicycles.

For children with lichen sclerosus affecting the perianal region, constipation may be among the first signs of the presence of the disease. Lichen sclerosus is much more likely to affect males that have not been circumcised than males that have been.

Skin tissue often becomes thin, shiny, wrinkled and parchment-like. Fissures, cracks, and purplish patches (ecchymoses) appear frequently. An eruption of bluish-white pimples, either separate or joined together, containing a central depression or a black plug of hard skin occurs. Swelling of the skin, overgrowth of skin (epidermal hyperkeratosis), loss of skin tissue, (atrophy), and inflammation are accompanied by soreness and itching. Shrinkage of the skin of the vagina and vulva, often accompanied by a chronic inflammation in the deeper tissues (kraurosis vulvae), may also occur.

In males, lichen sclerosus most commonly affects the foreskin of the penis, although it may affect other areas of the body. The opening at the end of the foreskin may become narrow and scarred. Discoloration and skin changes may also occur. Symptoms also include itching, soreness, and painful erections.

Thick, white patches of skin (leukoplakia) that are slightly raised may develop on the penis or vulva.

In some rare cases, skin lesions may also develop in the mouth. The lesions consist of bluish-white flat patchy areas on the inside of the cheeks and/or palate. The tongue may also be involved.

The exact cause of lichen sclerosus is not known. It may be due to an autoimmune process, or an injury, or may follow radiation therapy. Autoimmune disorders arise when the body’s natural defenses against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. Some cases of lichen sclerosus may be linked to formation of certain antibodies (e.g. a thyroid protein (thyroglobulin), or certain cells that line the walls of organs.

Some scientists believe that a genetic predisposition to lichen sclerosus exists. A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease.

Lichen sclerosus affects mostly females, usually between the ages of 40 and 60 years. Cases involving younger females and males have also been identified in the medical literature in the United States, and cases have been reported that involve female children under the age of thirteen. Females are six times as likely to be affected as are males.

Symptoms of the following disorders can be similar to those of Lichen Sclerosus. Comparisons may be useful for a differential diagnosis:

Lichen Planus is a rare disorder associated with recurrent, itchy, inflammatory eruptions of the skin. Which are usually small separate, angular spots that may merge into rough scaly patches. Lichen Planus is often accompanied by lesions in the mouth (oral). Females are most commonly affected by the disorder. (For more information on this disorder, choose "Lichen Planus" as your search term in the Rare Disease Database.)

Carcinoma of the Vulva is a malignant disease characterized by abnormal cancerous changes in the skin of the vulva. Changes can resemble those of severe Lichen Sclerosus.

Hyperplastic Dystrophy of Vulva represents an skin response to injury and is usually accompanied by itching. It may be caused by ingestion of foods with high acid content or contact with a chemical such as a laundry detergent, body soap, hygiene sprays, dye in toilet paper, or other various substances that come in contact with the skin. Some fabrics or unusually tight clothing may cause this condition. In some cases, there may be no apparent cause. Corticosteroid cream often clears up the skin symptoms. This medication may be used as a continued maintenance treatment in patients who experience recurring symptoms.
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Diagnosis
Lichen sclerosus is diagnosed in advanced cases by looking at the skin affected. Milder cases require a thorough clinical evaluation, identification of characteristic physical features, and a detailed patient history. In order to be sure of the diagnosis in mild cases, a skin biopsy is almost invariably undertaken.

Treatment
Patients with non-genital lichen sclerosus should remain watchful about the course of the disease but may not need to treat it. However, even mild symptoms of genital or perianal lichen sclerosus should be treated in order to avoid the scarring that can lead to the narrowing of vaginal or anal openings.

Treatment consists of the use of one or another of the ultrapotent corticosteroids that are available by prescription as a cream or ointment. Ultrapotent corticosteroids available in the United States include: betamethasone diproprionate, clobetasol propionate, diflorasone diacetate, and halobetasol propionate. These drugs may stop the itching within days or a couple of weeks. Within a few months of regular use, they may make it possible for skin to regain its strength and texture, but they cannot affect scarring or changes in skin color that may already have taken place.

In very severe cases, surgical removal of affected skin layers may be of benefit. In males, circumcision may be helpful (if the foreskin is involved).

Clinical trials of low-dose ultraviolet light therapy, either alone or in combination with drug therapy, have been reported. More studies are needed to determine the efficacy and safety of this treatment for patients with lichen sclerosus, as well as the potential duration of relief from symptoms.

McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Lichen Sclerosus et atrophicus. Entry Number; 151590: Last Edit Date; 12/19/1994.

TEXTBOOKS
Goldman BD. Lichen Sclerosis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:126.

Champion RH, Burton JL, Ebling FJG, eds. Textbook of Dermatology. 5th ed. Blackwell Scientific Publications. London, UK; 1992:2269-74; 2813-14.

Jones III H, Wentz AC, Burnett LS, eds. Novak’s Textbook of Gynecology. 11th ed. William & Wilkins, Baltimore, MD; 1988:587-88 .

REVIEW ARTICLES
Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol. 2003;28:128-33.

Neill SM, Tatnall FM, Cox NH. British Association of Dermatologists. Guidelines for the management of lichen sclerosus. Br J Dermatol. 2002;147:640-49.

Wong YW, Powell J. Lichen sclerosus. A review. Minerva Med. 2002;93:95-99.

Fischer GO. Vulval disease in pre-pubertal girls. Australas J Dermatol. 2001;42:225-34.

Neill SM, Ridley CM. Management of anogenital lichen sclerosus. Clin Exp Dermatol. 2001;26:637-43.

Meffert JL, Davis BM, Grimwood RE. Lichen sclerosis. J Amer Acad Dermatol. 1995;32:393-416.

JOURNAL ARTICLES
Kizer Ws, Prarie T, Morey AF. Balanitis xerotica obliyerans: epidemiologic distribution in an equal access health care system. South Med J. 2003;96:9-11.

Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus. The course after puberty. J Reprod Med. 2002;47:706-09.

Rolfe KJ, Crow JC, Reid WM, et al. The effect of topical corticosteroids on Ki67 and p53 expression in vulval lichen sclerosus. Br J Dermatol. 2002;147:503-08.

Hagedorn M, Buxmeyer B, Schmitt Y, et al. Survey of genital lichen sclerosus in women and men. Arch Gynecol Obstet. 2002;266:86-91.

Powell J, Robson A, Cranston D, et al. High incidence of lichen sclerosus in patients with squamous cell carcinoma of the penis.Br J Dermatol. 2001;145:85-89.

FROM THE INTERNET
Questions and Answers About Lichen Sclerosus. NIAMS. Publication Date: March 2000. 5pp.
www.niams.nih.gov/hi/topics/lichen/lichen.htm

Hicks R. Lichen sclerosus. BBC Health. nd. 2pp.
www.bbc.co.uk/health/features/lichen_sclerosus.shtml

What is Lichen Sclerosus? Symptoms? Diagnosis? Treatment? National Lichen Sclerosus Support Group. nd. 3pp.
www.lichensclerosus.org

Vulvar/Anal Lichen Sclerosis. Univerisity of Iowa Healh Care. Dep’t Ob/Gyn. Peer Review Date: 1997. 2pp.
http://obgyn.uihc.uiowa.edu/patinfo/Vulvar/lichensclerosis.htm

Lichen Sclerosus: What is it? Center for Vulvar Pain. nd. 2pp.
www.vulvarhealth.org/LS/summary.htlm
Current Remedies for Lichen Sclerosus. Center for Vulvar Pain. nd. 3pp.
www.vulvarhealth.org/LS/remedies.htlm
Possible Causes of Lichen Sclerosus. Center for Vulvar Pain. nd. 2pp.
www.vulvarhealth.org/LS/causes.htlm

Lichen Sclerosis. Center for Vulvar Diseases. Department of Ob/Gyn, University of Michigan Health System. Ó2003. 2pp.
www.med.umich.edu/obgyn/vulva/lichens.htm