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Vitiligo
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KEY
WORDS
Antibodies:
protective proteins produced by the body's immune
system to fight infectious agents (such as bacteria
or viruses) or other "foreign" substances.
Occasionally abnormal antibodies develop that can
attack a part of the body and cause an "autoimmune"
disease. These abnormal antibodies are called autoantibodies.
Pigment:
a coloring matter in the cells and tissues of the
body.
Pigmentation:
coloring of the skin, hair, mucous membranes, and
retina of the eye.
Depigmentation:
loss of color in the skin, mucous membranes, hair,
or retina of the eye.
Melanin:
a yellow, brown, or black pigment that determines
skin color. Melanin also acts as a sunscreen and protects
the skin from ultraviolet light.
Melanocytes:
special cells that produce melanin.
Ultraviolet
light A (UVA): one type of radiation that is part
of sunlight and reaches the earth's surface. Exposure
to UVA can cause the skin to tan. Ultraviolet light
is also used in a treatment called phototherapy for
certain skin conditions, including vitiligo.
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What Is Vitiligo?
Vitiligo (vit-ill-eye-go)
is a pigmentation disorder in which melanocytes (the cells that
make pigment) in the skin, the tissues that line the inside
of the mouth and nose and genital and rectal areas (mucous membranes),
and the retina of the eyes are destroyed. As a result, white
patches of skin appear on different parts of the body. The hair
that grows in areas affected by vitiligo may turn white.
The cause of vitiligo
is not known, but doctors and researchers have several different
theories. One theory is that people develop antibodies that
destroy the melanocytes in their own bodies. Another theory
is that melanocytes destroy themselves. Finally, some people
have reported that a single event such as sunburn or emotional
distress triggered vitiligo; however, these events have not
been scientifically proven to cause vitiligo.
Who Is Affected
by Vitiligo?
About 1 to 2 percent
of the world's population, or 40 to 50 million people, have
vitiligo. In the United States, 2 to 5 million people have the
disorder. Ninety-five percent of people who have vitiligo develop
it before their 40th birthday. The disorder affects all races
and both sexes equally.
Vitiligo seems to
be more common in people with certain autoimmune diseases (diseases
in which a person's immune system reacts against the body's
own organs or tissues). These autoimmune diseases include hyperthyroidism
(an overactive thyroid gland), adrenocortical insufficiency
(the adrenal gland does not produce enough of the hormone called
corticosteroid), alopecia areata (patches of baldness), and
pernicious anemia (a low level of red blood cells caused by
failure of the body to absorb vitamin B12). Scientists
do not know the reason for the association between vitiligo
and these autoimmune diseases. However, most people with vitiligo
have no other autoimmune disease.
Vitiligo may also
be hereditary, that is, it can run in families. Children whose
parents have the disorder are more likely to develop vitiligo.
However, most children will not get vitiligo even if a parent
has it, and most people with vitiligo do not have a family history
of the disorder.
What Are the Symptoms
of Vitiligo?
People who develop
vitiligo usually first notice white patches (depigmentation)
on their skin. These patches are more common in sun-exposed
areas, including the hands, feet, arms, face, and lips. Other
common areas for white patches to appear are the armpits and
groin and around the mouth, eyes, nostrils, navel, and genitals.
Vitiligo generally
appears in one of three patterns. In one pattern (focal pattern),
the depigmentation is limited to one or only a few areas. Some
people develop depigmented patches on only one side of their
bodies (segmental pattern). But for most people who have vitiligo,
depigmentation occurs on different parts of the body (generalized
pattern). In addition to white patches on the skin, people with
vitiligo may have premature graying of the scalp hair, eyelashes,
eyebrows, and beard. People with dark skin may notice a loss
of color inside their mouths.
Will the Depigmented
Patches Spread?
There is no way to
predict if vitiligo will spread. For some people, the depigmented
patches do not spread. The disorder is usually progressive,
however, and over time the white patches will spread to other
areas of the body. For some people, vitiligo spreads slowly,
over many years. For other people, spreading occurs rapidly.
Some people have reported additional depigmentation following
periods of physical or emotional stress.
How Is Vitiligo
Diagnosed?
If a doctor suspects
that a person has vitiligo, he or she usually begins by asking
the person about his or her medical history. Important factors
in a person's medical history are a family history of vitiligo;
a rash, sunburn, or other skin trauma at the site of vitiligo
2 to 3 months before depigmentation started; stress or physical
illness; and premature graying of the hair (before age 35).
In addition, the doctor will need to know whether the patient
or anyone in the patient's family has had any autoimmune diseases
and whether the patient is very sensitive to the sun.
The doctor will then
examine the patient to rule out other medical problems. The
doctor may take a small sample (biopsy) of the affected skin.
He or she may also take a blood sample to check the blood-cell
count and thyroid function. For some patients, the doctor may
recommend an eye examination to check for uveitis (inflammation
of part of the eye). A blood test to look for the presence of
antinuclear antibodies (a type of autoantibody) may also be
done. This test helps determine if the patient has another autoimmune
disease.
How Can People Cope
With the Emotional and Psychological Aspects of Vitiligo?
The change in appearance
caused by vitiligo can affect a person's emotional and psychological
well-being and may create difficulty in getting or keeping a
job. People with this disorder can experience emotional stress,
particularly if vitiligo develops on visible areas of the body,
such as the face, hands, arms, feet, or on the genitals. Adolescents,
who are often particularly concerned about their appearance,
can be devastated by widespread vitiligo. Some people who have
vitiligo feel embarrassed, ashamed, depressed, or worried about
how others will react.
Several strategies
can help a person cope with vitiligo. First, it is important
to find a doctor who is knowledgeable about vitiligo and takes
the disorder seriously. The doctor should also be a good listener
and be able to provide emotional support. Patients need to let
their doctor know if they are feeling depressed because doctors
and other mental health professionals can help people deal with
depression. Patients should also learn as much as possible about
the disorder and treatment choices so that they can participate
in making important decisions about medical care.
Talking with other
people who have vitiligo may also help a person cope. The National
Vitiligo Foundation can provide information about vitiligo and
refer people to local chapters that have support groups of patients,
families, and physicians. Family and friends are another source
of support.
Some people with
vitiligo have found that cosmetics that cover the white patches
improve their appearance and help them feel better about themselves.
A person may need to experiment with several brands of concealing
cosmetics before finding the product that works best.
What Treatment Options
Are Available?
The goal of treating
vitiligo is to restore the function of the skin and to improve
the patient's appearance. Therapy for vitiligo takes a long
time--it usually must be continued for 6 to 18 months. The choice
of therapy depends on the number of white patches and how widespread
they are and on the patient’s preference for treatment.
Each patient responds differently to therapy, and a particular
treatment may not work for everyone.
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TREATMENT
OPTIONS FOR VITILIGO
Medical
Therapies
- Topical
steroid therapy
- Topical
psoralen photochemotherapy
- Oral
psoralen photochemotherapy
- Depigmentation
Surgical
Therapies
- Autologous
skin grafts
- Skin
grafts using blisters
- Micropigmentation
(tattooing)
- Autologous
melanocyte transplants
Adjunctive
Therapies
- Sunscreens
- Cosmetics
- Counseling
and support
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Current treatment
options for vitiligo include medical, surgical, and adjunctive
therapies (therapies that can be used along with surgical or
medical treatments).
Medical Therapies
- Topical
Steroid Therapy
- Steroids may be
helpful in repigmenting (returning the color to white patches)
the skin, particularly if started early in the disease. Corticosteroids
are a group of drugs similar to the hormones produced by the
adrenal glands (such as cortisone). Doctors often prescribe
a mild topical corticosteroid cream for children under 10
years old and a stronger one for adults. Patients must apply
the cream to the white patches on their skin for at least
3 months before seeing any results. It is the simplest and
safest treatment but not as effective as psoralen photochemotherapy
(see below). The doctor will closely monitor the patient for
side effects such as skin shrinkage and skin striae (streaks
or lines on the skin).
- Psoralen
Photochemotherapy
- Psoralen photochemotherapy
(psoralen and ultraviolet A therapy, or PUVA) is probably
the most beneficial treatment for vitiligo available in the
United States. However, it is time-consuming and care must
be taken to avoid side effects, which can sometimes be severe.
Psoralens are drugs that contain chemicals that react with
ultraviolet light to cause darkening of the skin. The treatment
involves taking psoralen by mouth (orally) or applying it
to the skin (topically). This is followed by carefully timed
exposure to ultraviolet A (UVA) light from a special lamp
or to sunlight. Patients usually receive treatments in their
doctors' offices so they can be carefully watched for any
side effects. Patients must minimize exposure to sunlight
at other times. The goal of PUVA therapy is to repigment the
white patches.
- Topical
Psoralen Photochemotherapy
- Topical psoralen
photochemotherapy often is used for people with a small number
of depigmented patches (affecting less than 20 percent of
the body). It is also used for children 2 years old and older
who have localized patches of vitiligo. Treatments are done
in a doctor's office under artificial UVA light once or twice
a week. The doctor or nurse applies a thin coat of psoralen
to the patient's depigmented patches about 30 minutes before
UVA light exposure. The patient is then exposed to an amount
of UVA light that turns the affected area pink. The doctor
usually increases the dose of UVA light slowly over many weeks.
Eventually, the pink areas fade and a more normal skin color
appears. After each treatment, the patient washes his or her
skin with soap and water and applies a sunscreen before leaving
the doctor's office.
- There are two
major potential side effects of topical PUVA therapy: (1)
severe sunburn and blistering and (2) too much repigmentation
or darkening of the treated patches or the normal skin surrounding
the vitiligo (hyperpigmentation). Patients can minimize their
chances of sunburn if they avoid exposure to direct sunlight
after each treatment. Hyperpigmentation is usually a temporary
problem and eventually disappears when treatment is stopped.
- Oral Psoralen
Photochemotherapy
- Oral PUVA therapy
is used for people with more extensive vitiligo (affecting
greater than 20 percent of the body) or for people who do
not respond to topical PUVA therapy. Oral psoralen is not
recommended for children under 10 years of age because of
an increased risk of damage to the eyes, such as cataracts.
For oral PUVA therapy, the patient takes a prescribed dose
of psoralen by mouth about 2 hours before exposure to artificial
UVA light or sunlight. The doctor adjusts the dose of light
until the skin areas being treated become pink. Treatments
are usually given two or three times a week, but never 2 days
in a row.
- For patients who
cannot go to a PUVA facility, the doctor may prescribe psoralen
to be used with natural sunlight exposure. The doctor will
give the patient careful instructions on carrying out treatment
at home and monitor the patient during scheduled checkups.
- Known side effects
of oral psoralen include sunburn, nausea and vomiting, itching,
abnormal hair growth, and hyperpigmentation. Oral psoralen
photochemotherapy may increase the risk of skin cancer. To
avoid sunburn and reduce the risk of skin cancer, patients
undergoing oral PUVA therapy should apply sunscreen and avoid
direct sunlight for 24 to 48 hours after each treatment. Patients
should also wear protective UVA sunglasses for 18 to 24 hours
after each treatment to avoid eye damage, particularly cataracts.
- Depigmentation
- Depigmentation
involves fading the rest of the skin on the body to match
the already white areas. For people who have vitiligo on more
than 50 percent of their body, depigmentation may be the best
treatment option. Patients apply the drug monobenzylether
of hydroquinone (monobenzone or Benoquin*) twice
a day to pigmented areas until they match the already depigmented
areas. Patients must avoid direct skin-to-skin contact with
other people for at least 2 hours after applying the drug.
- The major side
effect of depigmentation therapy is inflammation (redness
and swelling) of the skin. Patients may experience itching,
dry skin, or abnormal darkening of the membrane that covers
the white of the eye. Depigmentation is permanent and cannot
be reversed. In addition, a person who undergoes depigmentation
will always be abnormally sensitive to sunlight.
Surgical Therapies
All surgical therapies
must be viewed as experimental because their effectiveness and
side effects remain to be fully defined.
- Autologous
Skin Grafts
- In an autologous
(use of a person’s own tissues) skin graft, the doctor
removes skin from one area of a patient's body and attaches
it to another area. This type of skin grafting is sometimes
used for patients with small patches of vitiligo. The doctor
removes sections of the normal, pigmented skin (donor sites)
and places them on the depigmented areas (recipient sites).
There are several possible complications of autologous skin
grafting. Infections may occur at the donor or recipient sites.
The recipient and donor sites may develop scarring, a cobblestone
appearance, or a spotty pigmentation, or may fail to repigment
at all. Treatment with grafting takes time and is costly,
and most people find it neither acceptable nor affordable.
- Skin Grafts
Using Blisters
- In this procedure,
the doctor creates blisters on the patient's pigmented skin
by using heat, suction, or freezing cold. The tops of the
blisters are then cut out and transplanted to a depigmented
skin area. The risks of blister grafting include the development
of a cobblestone appearance, scarring, and lack of repigmentation.
However, there is less risk of scarring with this procedure
than with other types of grafting.
- Micropigmentation
(Tattooing)
- Tattooing implants
pigment into the skin with a special surgical instrument.
This procedure works best for the lip area, particularly in
people with dark skin; however, it is difficult for the doctor
to match perfectly the color of the skin of the surrounding
area. Tattooing tends to fade over time. In addition, tattooing
of the lips may lead to episodes of blister outbreaks caused
by the herpes simplex virus.
- Autologous
Melanocyte Transplants
- In this procedure,
the doctor takes a sample of the patient's normally pigmented
skin and places it in a laboratory dish containing a special
cell culture solution to grow melanocytes. When the melanocytes
in the culture solution have multiplied, the doctor transplants
them to the patient's depigmented skin patches. This procedure
is currently experimental and is impractical for the routine
care of people with vitiligo.
Adjunctive Therapies
- Sunscreens
- People who have
vitiligo, particularly those with fair skin, should use a
sunscreen that provides protection from both the UVA and UVB
forms of ultraviolet light. Sunscreen helps protect the skin
from sunburn and long-term damage. Sunscreen also minimizes
tanning, which makes the contrast between normal and depigmented
skin less noticeable.
- Cosmetics
- Some patients
with vitiligo camouflage depigmented patches with stains,
makeup, or self-tanning lotions. These cosmetic products can
be particularly effective for people whose vitiligo is limited
on exposed areas of the body. Dermablend, Lydia O'Leary, Clinique,
Fashion Flair, Vitadye, and Chromelin offer makeup or dyes
that patients may find helpful for covering up depigmented
patches.
- Counseling
and Support
- Many people with
vitiligo find it helpful to get counseling from a mental health
professional. People often find they can talk to their counselor
about issues that are difficult to discuss with anyone else.
A mental health counselor can also offer patients support
and help in coping with vitiligo.
What Research Is
Being Done on Vitiligo?
Over the past 10
years, research on how melanocytes play a role in vitiligo has
greatly increased. This includes research on autologous melanocyte
transplants. Doctors and researchers continue to look for the
causes of and new treatments for vitiligo.
Where Can People
Get More Information About Vitiligo?
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National
Vitiligo Foundation
P.O. Box 6337
Tyler, TX 75711
903/534-2925
World Wide Web address: http://www.nvfi.org/
This nonprofit organization
stimulates, coordinates, and sponsors scientific research on
vitiligo. In addition, the Foundation educates the public about
vitiligo and assists in making referrals for treatment. The
Foundation holds annual and regional meetings and sponsors symposia
open to both professionals and the public. It publishes a newsletter
two times a year and can provide free brochures on vitiligo.
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American
Academy of Dermatology
P.O. Box 4014
Schaumburg, IL 60168-4014
847/330-0230
Fax: 847/330-0050
World Wide Web address: http://www.aad.org
This national organization
for dermatologists publishes a six-page brochure on vitiligo.
A single copy is free with a self-addressed stamped envelope.
The Academy also can provide referrals to dermatologists.
Acknowledgments
The NIAMS gratefully
acknowledges the assistance of Jean-Claude Bystryn, M.D., of
the New York University; Rebat M. Halder, M.D., of Howard University,
Washington, DC; and James J. Nordlund, M.D., of the University
of Cincinnati in the preparation and review of this fact sheet.
* Brand names
included in this fact sheet are provided as examples only, and
their inclusion does not mean that these products are endorsed
by the National Institutes of Health or any other Government
agency. Also, if a particular brand name is not mentioned, this
does not mean or imply that the product is unsatisfactory.
The National Arthritis
and Musculoskeletal and Skin Diseases Information Clearinghouse
(NAMSIC) is a public service sponsored by the NIAMS that provides
health information and information sources. The NIAMS, a component
of the National Institutes of Health (NIH), leads and coordinates
the Federal medical effort in arthritis, musculoskeletal, bone,
muscle, and skin diseases by conducting and supporting research
projects, research training, clinical trials, and epidemiological
studies, and by disseminating information on research initiatives
and research results.
AMT 10/96
Updated 1/97
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