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$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.