$Unique_ID{BRK03940} $Pretitle{} $Title{Lichen Sclerosus} $Subject{Lichen Sclerosus Lichen Sclerosus et Atrophicus White-spot disease Csillag's disease Hallopeau I Disease Guttate Morphea Guttate Scleroderma Von Zambusch Disease Scleroderma Lichen Planus Carcinoma of the Vulva Hyperplastic Dystrophy Paget's Disease of the Vulva } $Volume{} $Log{} Copyright (C) 1986, 1988, 1989 National Organization for Rare Disorders, Inc. 252: Lichen Sclerosus ** IMPORTANT ** It is possible the main title of the article (Lichen Sclerosus) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Lichen Sclerosus et Atrophicus White-spot disease Csillag's disease Hallopeau I Disease Guttate Morphea Guttate Scleroderma Von Zambusch Disease 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 General Discussion ** IMPORTANT ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Lichen Sclerosus is a chronic skin disorder that most commonly affects women between forty and sixty years of age, although cases have been identified among younger females as well. This disorder is characterized by skin changes of the vulva, although other body sites can be affected as well. In some very rare cases, males have also been affected. Lichen Sclerosus can develop concurrently with other skin abnormalities. Symptoms Lichen Sclerosus usually affects the vulva in women. It is characterized by abnormal changes in the external genitalia and usually occurs between the ages of forty and sixty. Skin tissue often becomes thin, shiny, and parchment-like. Fissures, cracks, and purplish patches (ecchymoses) appear frequently. An eruption of white pimples, either separate or joined together, containing a central depression or a black plug of hard skin (visible microscopically) occurs. Swelling of the skin, overgrowth of the superficial skin layer (epidermal hyperkeratosis), atrophy, and mid-dermal inflammation are accompanied by soreness and itching. Atrophy and shrinkage of the skin of the vagina and vulva, often accompanied by a chronic inflammatory reaction in the deeper tissues (kraurosis vulvae) may also occur. Lichen Sclerosus is not a premalignant disease. In males, Lichen Sclerosus is characterized as disease of the foreskin of the penis although it may occur in other areas of the body. The opening in the end of the foreskin narrows and may become scarred. Discoloration and skin changes also occur. Causes The exact cause of Lichen Sclerosus is not known. It may be due to an autoimmune process, an injury, or may follow radiation therapy. Autoimmune disorders are caused when the body's natural defenses against foreign substances (antibodies) suddenly begin to attack healthy tissue. Some cases of Lichen Sclerosus may be linked to formation of antibodies to a thyroid protein (thyroglobulin), organ wall (parietal) cells, adrenal cells, or the thyroid. Some scientists believe that a genetic predisposition to Lichen Sclerosus exists. Affected Population Lichen Sclerosus predominantly affects females, usually between the ages of forty and sixty. Cases involving younger females and males have also been identified in the medical literature in the United States. Females are affected at a ratio of six to seven times more often than males. Related Disorders Symptoms of the following disorders can be similar to those of Lichen Sclerosus. Comparisons may be useful for a differential diagnosis: Scleroderma refers to a group of chronic disorders characterized by fibrosis, degenerative changes, vascular abnormalities, and excess collagen in the skin. Scleroderma is the chronic hardening and shrinking of the connective tissues of any part of the body, although the term literally means "hardening of the skin". Lichen Sclerosus can occur concurrently with Scleroderma. (For more information on this disorder, choose "Scleroderma" as your search term in the Rare Disease Database). Lichen Planus is a recurrent, itchy, inflammatory eruption of the skin which is characterized by small separate, angular spots that may merge into rough scaly patches. It is often accompanied by oral lesions. Women 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 neoplasm associated with a number of disorders and is characterized by abnormal changes in skin layer cells of the vulva. Changes can resemble those of Lichen Sclerosus in very serious cases, but will continue to progress or may occur in other areas of the body as well. Cases of Lichen Sclerosus occurring in conjunction with this condition have been identified. Hyperplastic Dystrophy affecting the vulva represents an epithelial cell response to an 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 tight clothing may cause this condition, or there may be no apparent cause in some cases. Corticosteroid cream often clears up the skin symptoms. This medication may be used as a continued maintenance treatment in patients who have recurrences when applications are stopped. Therapies: Standard Treatment of Lichen Sclerosus consists of applications of testosterone to the affected areas in females. Surgical removal of affected skin layers may be of benefit in very severe cases. Therapies: Investigational Research on the use of the drug Etretinate (Tigason) as a treatment for Lichen Sclerosus is underway. However, more tests to determine safety and effectiveness are necessary before this drug can be recommended for use in all but the most severe cases of Lichen Sclerosus. This disease entry is based upon medical information available through February 1988. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Lichen Sclerosus, please contact: For more information on Lichen Sclerosis et Atrophicus, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W.B. Saunders Co., 1988. P. 1419, 2341.