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$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.