Lichen Sclerosus

Lichen Sclerosus

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Lichen Sclerosus is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • 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

  • None

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.

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

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.

Resources

National Vulvodynia Association

P.O. Box 4491

Silver Spring,, MD 20914-4491

USA

Tel: (301)299-0775

Fax: (301)299-3999

Email: mate@nva.org

Internet: http://www.nva.org



National Women's Health Resource Center

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Suite 106

Red Bank, NJ 07701

Tel: (732)530-3425

Fax: (732)530-3347

Tel: (877)986-9472

Email: gcassidy@healthywomen.org

Internet: http://www.healthywomen.org



American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



National Women's Health Network

1413 K Street, NW

4th Floor

Washington, DC 20005

USA

Tel: (202)682-2640

Fax: (202)682-2648

Email: nwhn@nwhn.org

Internet: http://www.womenshealthnetwork.org



NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



International Scleroderma Network

7455 France Ave So #266

Edina, MN 55435-4702

Tel: (952)583-5735

Tel: (800)564-7099

Email: isn@sclero.org

Internet: http://www.sclero.org



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

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