Health Topics
Publication Date: March 2000
Questions and Answers About Lichen
Sclerosus
This fact sheet contains general information on lichen
sclerosus. It describes what lichen sclerosus is and how it is diagnosed
and treated. If you have further questions after reading this fact sheet,
you may wish to discuss them with your doctor.
What Is Lichen Sclerosus?
Lichen sclerosus (LIKE-in skler-O-sus) is a skin disorder
that can affect men, women, or children, but is most common in women.
It usually occurs on the vulva (the outer genitalia or sex organ) in
women, but sometimes develops on the head of the penis in men. Occasionally,
lichen sclerosus is seen on other parts of the body, especially the
upper body, breasts, and upper arms.
The symptoms are the same in children and adults. Early
in the disease, small, subtle white spots appear. These areas are usually
slightly shiny and smooth. As time goes on, the spots develop into bigger
patches, and the skin surface becomes thinned and crinkled. As a result,
the skin tears easily, and bright red or purple discoloration from bleeding
inside the skin is common. More severe cases of lichen sclerosus produce
scarring that may cause the inner lips of the vulva to shrink and disappear,
the clitoris to become covered with scar tissue, and the opening of
the vagina to narrow.
Lichen sclerosus of the penis occurs almost exclusively
in uncircumcised men (those who have not had the foreskin removed).
The foreskin can scar, tighten, and shrink over the head of the penis.
Skin on other areas of the body affected by lichen sclerosus usually
does not experience scarring.
How Common Is It?
Although definitive data are not available, lichen sclerosus
is considered a rare disorder that can develop in people of all ages.
It primarily affects the vulva. Fewer than 1 in 20 women who have vulvar
lichen sclerosus have the disease on other skin surfaces. The disease
is much less common in childhood. In boys, it is a major cause of tightening
of the foreskin, which requires circumcision. Otherwise, it is very
uncommon in men.
What Are the Symptoms?
Symptoms vary depending on the area affected. Patients
experience very different degrees of discomfort. When lichen sclerosus
occurs on parts of the body other than the genital area, most often
there are no symptoms, other than itching. If the disease is severe,
bleeding, tearing, and blistering caused by rubbing or bumping the skin
can cause pain.
Very mild lichen sclerosus of the genital area may cause
itching, but often causes no symptoms at all. If the disease worsens,
itching is the most common symptom. Rarely, lichen sclerosus of the
vulva may cause extreme itching that interferes with sleep and daily
activities. Rubbing or scratching to relieve the itching can create
painful sores and bruising, so that many women must avoid sexual intercourse,
tight clothing, tampons, riding bicycles, and other common activities
that involve pressure or friction. Urination can be accompanied by burning
or pain, and bleeding can occur, especially during intercourse. When
lichen sclerosus develops around the anus, the discomfort can lead to
constipation. This is particularly common in children.
Most men with genital lichen sclerosus have not been circumcised.
They sometimes experience difficulty pulling back the foreskin and have
decreased sensation in the tip of the penis. Occasionally, erections
are painful, and the urethra (the tube through which urine flows) can
become narrow or obstructed.
What Causes Lichen Sclerosus?
The cause is unknown, although an overactive immune system
may play a role. Some people may have a genetic tendency toward the
disease, and studies suggest that abnormal hormone levels may also play
a role. Some scientists believe that an infectious bacterium, called
a spirochete, may cause the changes in the immune system that lead to
lichen sclerosus.
Is It Contagious?
No, lichen sclerosus is not contagious.
How Is It Diagnosed?
Doctors can diagnose an advanced case by looking at the
skin. However, early or mild disease often requires a biopsy (removal
and examination of a small sample of affected skin). Because other diseases
of the genitalia can look like lichen sclerosus, a biopsy is advised
whenever the appearance of the skin is not typical of lichen sclerosus.
How Is It Treated?
Patients with lichen sclerosus of nongenital skin often
do not need treatment because the symptoms are very mild and usually
go away over time. (The amount of time involved varies from patient
to patient.)
However, lichen sclerosus of the genital skin should be
treated, even when it is not causing itching or pain, because it can
lead to scarring that may narrow openings in the genital area and interfere
with either urination or sexual intercourse or both. There is also a
very small chance that cancer may develop.
In uncircumcised men, circumcision is the most widely
used therapy for lichen sclerosus. This procedure removes the affected
skin, and the disease usually does not recur.
Prescription medications are required to treat vulvar
lichen sclerosus, nongenital lichen sclerosus that is causing symptoms,
and lichen sclerosus of the penis that is not cured by circumcision.
The treatment of choice is an ultrapotent topical corticosteroid. Daily
use of these creams or ointments can stop itching within a few days
and restore the skin's normal texture and strength after several months.
However, treatment does not reverse the scarring that may have already
occurred.
Because ultrapotent corticosteroid creams and ointments
are very strong, frequent evaluation by a doctor is necessary to check
the skin for side effects when the medication is used every day. Once
the symptoms are gone and the skin has regained its strength, medication
can be used less frequently, although use must continue indefinitely,
several times a week, to keep vulvar lichen sclerosus in remission.
Ultrapotent Corticosteroids Available
by Prescription in the United States
|
Young girls may not require lifelong treatment, since
lichen sclerosus can sometimes, but not always, disappear permanently
at puberty. Scarring and changes in skin color, however, may remain
even after the symptoms have disappeared.
Because ultrapotent topical corticosteroids are so effective,
other therapies are rarely prescribed. The previous standard therapy
was testosterone ointment or cream, but this has recently been proven
to produce no more benefit than a placebo (inactive) cream. Another
hormone cream, progesterone, was previously used to treat the disease,
but also has little beneficial effect. Retinoids, or vitamin A-like
medications, may be helpful for patients who cannot tolerate or are
not helped by ultrapotent topical corticosteroids.
Patients who need medication should ask their doctor how
it works, what side effects it might have, and why it is the best treatment
for lichen sclerosus.
For women and girls, surgery to remove the affected skin
is not an acceptable option. Surgery may be useful for scarring, but
only after lichen sclerosus is controlled with medication.
Sometimes, people do not respond to the ultrapotent topical
corticosteroid. Other factors, such as low estrogen levels that cause
vaginal dryness and soreness, a skin infection, or irritation or allergy
to the medication, can keep symptoms from clearing up. Your doctor may
need to treat these factors as well. If you feel that you are not improving
as you would expect, talk to your doctor.
Can People With Lichen Sclerosus
Have Sexual Intercourse?
Women with severe lichen sclerosus may not be able to
have sexual intercourse because of pain or scarring that narrows the
entrance to the vagina. However, proper treatment with an ultrapotent
topical corticosteroid should restore normal sexual ability, unless
severe scarring has already narrowed the vaginal opening. In this case,
surgery may be needed to correct the problem, but only after the disease
has been controlled.
Is Lichen Sclerosus Related
to Cancer?
Lichen sclerosus does not cause skin cancer. However,
skin that is scarred by lichen sclerosus is more likely to develop skin
cancer. About 1 in 20 women with untreated vulvar lichen sclerosus develops
skin cancer. The frequency of skin cancer in men with lichen sclerosus
is not known. It is important for people who have the disease to receive
proper treatment and to see their doctor every 6 to 12 months, so that
he or she can monitor and treat any changes that might signal skin cancer.
What Kind of Doctor Treats
Lichen Sclerosus?
Lichen sclerosus is treated by dermatologists (skin doctors)
and by gynecologists if the female genitalia are involved. Urologists
and primary care health providers with a special interest in genital
diseases also treat this disease. To find a doctor who treats lichen
sclerosus, ask your family doctor for a referral, call a local or State
department of health, look in the local telephone directory, or contact
a local medical center. The American Academy of Dermatology also provides
referrals to dermatologists in your area, and the American College of
Obstetricians and Gynecologists can refer you to a gynecologist. The
Directory of Medical Specialists, available at most public libraries,
lists dermatologists, gynecologists, and urologists in your area.
Where Can People Find More
Information on Lichen Sclerosus?
This clearinghouse, a public service sponsored by the
NIAMS, provides information on arthritis and musculoskeletal and skin
diseases. The clearinghouse distributes patient and professional education
materials and also refers people to other sources of information.
This national professional association for dermatologists
provides patient information and referrals to dermatologists.
This professional association provides referrals to gynecologists
and has patient education materials.
This professional association provides physician referrals
to urologists through its Web site. Many public libraries now provide
access to the World Wide Web. Ask a librarian for assistance.
This association provides information and support to women
who have vulvovaginal pain, including pain caused by lichen sclerosus.
The NVA maintains a network of support groups or support individuals
throughout the United States, in Canada, and in Europe. The association
also publishes a newsletter.
Acknowledgments
The NIAMS gratefully acknowledges the assistance of
Alan Moshell, M.D., NIAMS, NIH; Libby Edwards, M.D., Wake Forest University
School of Medicine; and Harriet O’Connor, National Vulvodynia Association,
in the preparation and review of this fact sheet.
The National Institute of Arthritis and Musculoskeletal
and Skin Diseases (NIAMS), a part of the National Institutes of Health
(NIH), leads the Federal medical research effort in arthritis and musculoskeletal
and skin diseases. The NIAMS supports research and research training
throughout the United States, as well as on the NIH campus in Bethesda,
MD, and disseminates health and research information. The National Institute
of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
is a public service sponsored by the NIAMS that provides health information
and information sources.