Vitiligo
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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.
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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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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.
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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.
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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.
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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.
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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.
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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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Medical Therapies
- Topical steroid therapy
- Topical psoralen photochemotherapy
- Oral psoralen photochemotherapy
- Depigmentation
Surgical Therapies
- Skin grafts from a person's own tissues (autologous)
- Skin grafts using blisters
- Micropigmentation (tattooing)
- Autologous melanocyte transplants
Adjunctive Therapies
- Sunscreens
- Cosmetics
- Counseling and support
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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.
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.
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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For more than a decade, research on how melanocytes play a
role in vitiligo has greatly increased. This includes research on
autologous melanocyte transplants. At the University of Colorado, NIAMS
supports a large collaborative project involving families with vitiligo
in the United States and the United Kingdom. To date, over 2,400
patients are involved. It is hoped that genetic analysis of these
families will uncover the location--and possibly the specific gene or
genes--conferring susceptibility to the disease. Doctors and researchers
continue to look for the causes of and new treatments for vitiligo.
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American Academy
of Dermatology
National Vitiligo
Foundation
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