$Unique_ID{BRK03939} $Pretitle{} $Title{Lichen Planus} $Subject{Lichen Planus Csillag's Disease Guttate Morphea Guttate Scleroderma Lichen Planus Sclerosus Atrophicus Zambusch's Disease von Zambusch's Disease White Spot Disease Hallopeau's Disease I Lichen Ruber Planus } $Volume{} $Log{} Copyright (C) 1986, 1989, 1990 National Organization for Rare Disorders, Inc. 229: Lichen Planus ** IMPORTANT ** It is possible the main title of the article (Lichen Planus) is not the name you expected. Please check the SYNONYMS listing the find the alternate names and disorder subdivisions covered by this article. Synonyms Csillag's Disease Guttate Morphea Guttate Scleroderma Lichen Planus Sclerosus Atrophicus Zambusch's Disease, also known as von Zambusch's Disease White Spot Disease Hallopeau's Disease I Lichen Ruber Planus General Discussion ** REMINDER ** 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 Planus is a recurrent, itchy, inflammatory eruption of the skin which is characterized by small separate, angular spots that may coalesce into rough scaly patches. It is often accompanied by oral lesions. Women are most commonly affected by the disorder. Symptoms Onset of Lichen Planus may be abrupt or gradual. The initial attack persists for weeks or months, and intermittent recurrences may be noted for years. The primary spots are 2 to 4 mm in diameter with angular borders, a violet color and a distinct sheen in cross-lighting. Rarely, blisters may develop. Moderate to severe itching may be present, and it often does not respond to treatment. The lesions are usually distributed symmetrically, most commonly on the joint surfaces of the wrists and on the legs, trunk, glans penis, and mucous membrane of the mouth and vagina. Lesions are occasionally generalized, but the face is rarely involved. The lesions may become large, scaly and warty (Hypertrophic Lichen Planus), particularly on the lower legs. During the acute phase, new spots may appear along a site of minor skin injury such as a superficial scratch. Hyperpigmentation and sometimes atrophy may develop as lesions persist. Rarely, a patchy scarring baldness of the scalp is present. The mucous membrane of the mouth is involved in approximately 50% of patients with Lichen Planus, often before skin lesions develop. The mucous membrane of the cheek, tongue margins and in areas without teeth show asymptomatic ill-defined bluish-white linear lesions; these lesions may be net-like at first and increase in size in an angular configuration. An erosive form may occur in which the patient complains of shallow, often painful recurrent ulcerations of the mouth. Chronic increases in severity and remissions are common. Causes The cause of Lichen Planus is not known. Some metals such as arsenic, bismuth, gold, or exposure to certain chemicals used in developing color- photography may cause an eruption indistinguishable from Lichen Planus. Quinacrine taken for long period of time may produce hypertrophic Lichen Planus of the lower legs as well as other dermatological and systemic disturbances. Affected Population Lichen Planus affects 6 to 7 times as many women as men. Onset usually occurs around 40 to 50 years of age. Children are rarely affected. Related Disorders Candidiasis is a chronic infection of the skin, nails, scalp, and mucous membranes. (For more information on this disorder, choose "Candidiasis" as your search term in the Rare Disease Database. Erythema Multiforme is an inflammatory eruption characterized by symmetric red, swollen or blistery lesions of the skin and mucous membranes. Therapies: Standard Asymptomatic Lichen Planus does not require treatment. If a drug or chemical is suspected to be the cause, its use should be discontinued. In symptomatic Lichen Planus, antihistamines may decrease itching. Localized itchy or hypertrophic areas may be treated with triamcinolone acetonide suspension diluted with saline and superficially injected into the lesion, or with occlusive corticosteroid therapy. Tretinoid solution may also be beneficial in treating Lichen Planus. For erosive oral lesions, viscous lidocaine mouthwashes before meals and triamcinolone acetonide in emollient dental paste may be helpful. Erosive oral lesions and widespread severely itchy skin lesions often require a systemic corticosteroid such as oral prednisone. Unfortunately, skin lesions may return after systemic prednisone has been stopped. In this case, continued low dosage of a systemic corticosteroid may be tried. Therapies: Investigational In a 1989 study of thirteen patients with Lichen Planus who had not responded to other therapies, the investigational drug Temarotene appeared to help most patients after four to six months of therapy. Temarotene (RO-15-0778) is manufactured by Hoffman LaRoche. More research is needed to determine the safety and effectiveness of this drug. Scientists are studying the use of Cyclosporine (a drug that suppresses the immune system, normally used by organ transplant patients) on Lichen Planus. Rubbed into the skin three times each day, preliminary tests indicate that cyclosporine may be an effective treatment in Lichen Planus. More study is needed, however, to determine long-term safety and effective treatment for this disorder. Cyclosporine is also being investigated as a mouth wash treatment for patients with oral Lichen Planus. Preliminary studies suggest that this drug may be an effective treatment for oral symptoms. It is also used experimentally on certain autoimmune disorders since modification of the immune system may stop antibodies from attacking normal tissue. The drug is manufactured by Sandoz drugs. This disease entry is based upon medical information available through August 1990 . 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 Planus, 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 THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 2286. CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 2326-8. EFFECT OF TOPICAL CYCLOSPORINE RINSE ON ORAL LICHEN PLANUS, A Double-Blind Analysis, Drore Eisen, et al.; N Eng J Med (August 2, 1990, issue 323 ( )). Pp. 290-2394.