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Lichen Sclerosus

Synonyms of Lichen Sclerosus

  • Csillag's Disease (Sclerosus)
  • Guttate Morphea (Sclerosus)
  • Guttate Scleroderma, Lichen Sclerosus Type
  • Hallopeau I Disease
  • Lichen Sclerosus et Atrophicus
  • Von Zambusch Disease
  • White-Spot Disease

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Lichen sclerosus is a chronic skin disorder that most commonly affects post-menopausal women. However, it is sometimes identified among pre-menopausal women, and, even more rarely, among males. When found in males, the disease is known as balanitis xerotica obliterans.

Lichen sclerosus is characterized by skin changes of the external genitalia (i.e., vulva, head of the penis), although other parts of the body may also be affected. Intense itching often accompanies attacks of lichen sclerosus. The disorder is not contagious nor is it a sexually transmitted disease. In the recent past, a genetic component for lichen sclerosus has been recognized. In addition, many clinical researchers believe that it is a disorder of the immunological system. The understanding of the causes of this disorder is still incomplete. Lichen sclerosus can develop concurrently with other conditions.

Symptoms

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.

Causes

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.

Affected Populations

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.

Related Disorders

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.

Standard Therapies

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).

Investigational Therapies

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.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Lichen Sclerosus

References

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 .

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.

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.
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.

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.

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.

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

FROM THE INTERNET
Lichen Sclerosis. Center for Vulvar Diseases. Department of Ob/Gyn, University of Michigan Health System. 2003. 2pp.
www.med.umich.edu/obgyn/vulva/lichens.htm

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. Dept of 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

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.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2004/04/23 00:00:00 GMT+0

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