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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 mucous membranes (tissues that line the inside of the
mouth and nose and genital and rectal areas), and the retina (inner layer
of the eyeball) 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 usually turns 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.
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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, antibodies develop that can attack a part of the body
and cause an "autoimmune" disease. These 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, hair, mucous membranes, 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
skin 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. |
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 B-12). 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 (before age 35) graying
of the hair. 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 doctors 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. Current treatment
options for vitiligo include medical, surgical, and adjunctive therapies
(therapies that can be used along with surgical or medical treatments).
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Treatment Options for Vitiligo
Medical Therapies
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Topical steroid therapy
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Topical psoralen photochemotherapy
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Oral psoralen photochemotherapy
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Depigmentation
Surgical Therapies
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Skin grafts from a person's own
tissues (autologous)
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Skin grafts using blisters
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Micropigmentation (tattooing)
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Autologous melanocyte transplants
Adjunctive Therapies
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Sunscreens
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Cosmetics
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Counseling and support
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Medical Therapies
Topical Steroid
Therapy
Steroids may be helpful in
repigmenting the skin (returning the color to white patches),
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. The
goal of PUVA therapy is to repigment the white patches. 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.
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
bodies, 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.
* Brand names included in
this booklet 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.
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 normal 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.
Additional
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 cover depigmented patches with stains, makeup, or self-tanning
lotions. These cosmetic products can be particularly effective for
people whose vitiligo is limited to 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
Groups
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. In
addition, it may be helpful to attend a vitiligo support group.
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