Aaron Lerner, M.D. and Gisela Moellmann, Ph.D., Department of Dermatology, Yale University
Patients with vitiligo develop white spots on the skin that vary in size and location. The spots occur when pigment cells, or melanocytes, are destroyed and the pigment melanin can no longer be produced. Melanocytes normally occur throughout the skin, as well as in the hair follicles, mouth, and eyes. In vitiligo, pigment cells can be lost in any of these areas. Common sites of pigment loss are:
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- Exposed areas: hands, face, upper part of the chest
- Around body openings: eyes, nostrils, mouth, nipples, belly button, genitalia
- Body folds: arm pits, groin
- Sites of injury: cuts, scrapes, burns
- Hair: early graying of hair of the scalp, beard or other areas
- Area immediately surrounding pigmented moles
- Back of the eye
Vitiligo affects approximately 1% of the population worldwide. About 5 million people in the U.S. live with vitiligo. About half of the people who develop this skin disorder experience some pigment loss before the age of 20, and about one third of all vitiligo patients say that other family members also have this condition. Though its more noticeable on darker skin tones, vitiligo does not discriminate based on race, gender, socioeconomics or any other basis. Patients are usually not born with vitiligo. Though the average age for onset is in the mid 20's, onset can occur at any age. Even though most people with vitiligo are in good general health, they face a greater risk of having hyperthyroidism or hypothyroidism (increased or decreased thyroid function), pernicious anemia (vitamin B12 deficiency), Addison's disease (decreased adrenal function), alopecia areata (round patches of hair loss), and/or uveitis (inflammation of the eyes).
Melanin is the substance that normally determines the color of skin, hair, and eyes. This pigment is produced in cells called melanocytes. If melanocytes cannot form melanin or if their number decreases, skin color will become lighter or completely white as in vitiligo.
Leukoderma is a general term that means white skin. Severe trauma, like a burn, can destroy pigment cells resulting in leukoderma. Vitiligo is just one of the forms of leukoderma.
The precise cause of vitiligo is not known. A combination of genetic, immunologic and environmental factors is involved in most cases. This means that certain people are predisposed (have a greater tendency) to develop vitiligo upon exposure to the right trigger. Many people report pigment loss shortly after a severe sunburn, while others have related their onset of vitiligo to emotional trauma associated with an accident, death in the family, or divorce.
The beginning of vitiligo and the severity of pigment loss differs with each individual. Light skinned people usually notice the pigment loss during the summer as the contrast between depigmented skin and suntanned skin becomes distinct. People with dark skin may observe the onset of vitiligo at any time. Individuals who have severe cases may lose pigment over their entire body surface. There is no way to predict how much pigment an individual will lose. Illness and stress can result in more pigment loss. The degree of pigment loss can also vary within each vitiligo patch, and a border of abnormally dark skin may encircle a patch of depigmented skin.
Vitiligo frequently begins with a rapid loss of pigment, which may be followed by a lengthy period when the skin color does not change. Later, pigment loss may resume, perhaps after the individual has suffered physical trauma or stress. The loss of color may continue until, for unknown reasons, the process stops. Cycles of pigment loss followed by periods of stability may continue indefinitely. It is rare (but possible) for a patient with vitiligo to repigment or regain skin color spontaneously. Most patients who say that they no longer have vitiligo may actually have become depigmented and are no longer bothered by contrasting skin color. While such patients appear to be "cured", they really are not. People who have vitiligo all over their bodies do not look like albinos because the color of their hair may not change (or it can be dyed) and eye color does not change.
Many individuals with vitiligo are distressed because of their uneven skin color. It is difficult for people who do not have vitiligo to appreciate the significance of this problem. Individuals with vitiligo benefit from talking to, and sharing experiences with, other people with the disease.
There are two basic approaches to treatment: try to restore the normal pigment (repigmentation therapy), or try to destroy the remaining pigment cells (depigmentation therapy). Both are described below.
For skin to be repigmented, new pigment cells must be recruited from those that exist nearby. That means that new pigment cells must come from the base of hair follicles, from the edge of the lesion, or from the patch of vitiligo itself if depigmentation is not complete. The maximum amount of repigmentation that can be expected in any one spot in a year of treatment is an eighth to a quarter of an inch. If, in a given area, most of the hair follicles provide pigment cells that radiate to one eighth to a quarter of an inch, there may be substantial repigmentation. However, if the hairs are white or if there are no hairs present, repigmentation must occur from the edge of the depigmented area.
Hydrocortisone-type compounds (immunomodulators and immunosupressants), when applied to the skin, slow the process of depigmentation and sometimes enhance repigmentation. Cortisone ointments or creams sold over the counter, such as 0.5% hyrocortisone, are too weak to be effective. More potent cortisones are available by prescription. When potent cortisones are used daily for a long time, they may produce side effects such as thinning of the skin. Under the care of a dermatologist, it is usually possible to adjust the treatment with topical hydrocortisones such that side effects are minimized.
Phototherapy methods can also be used to induce repigmentation of the skin, either alone or in combination with hydrocortisone-type compounds. These forms of therapy consist of narrow-band ultraviolet B (UV-B) light, excimer laser, and PUVA (psoralen plus UV-A).
Narrow-band UVB (also referred to as narrowband and NB-UVB) is a relatively new vitiligo treatment option that helps to repigment cells with minimal to no side effects. It involves exposing the skin to a narrow-band UVB light source, which can be done in a doctor's office or using home phototherapy units. Typically treatment is done three times week for several months.
Excimer laser treatment involves exposing the skin to a beam of focused laser light. It is most commonly used for small areas of depigmentation. The majority of patients with head and neck depigmentation achieve substantial repigmentation in less than four months. Treatment must be done by a doctor; no home units are available at this time.
In PUVA, a patient is given a psoralen drug and is then exposed to UV-A light. When psoralen drugs are activated by the light, they stimulate repigmentation by increasing the movement of pigments cells into the vitiligo lesion. The response varies among patients and body sites. PUVA therapy is no longer the first choice of phototherapy given the availability and relative success of narrow-band UVB and excimer laser treatments.
About 75% of the patients who undergo PUVA therapy respond to some extent. Even for these individuals, complete repigmentation rarely occurs. After the initial two to three weeks of exposure to sunlight, patients will look worse since the contrast between their light and tanned skin increases. With time repigmentation will begin, and the appearance of the skin will improve. If therapy in stopped in winter, most people will retain at least half of the color they achieved during the summer months.
People with vitiligo should always protect their skin against excessive sun exposure by wearing protective clothing, staying out of the sun at peak periods (except during doctor-approved treatment times), and applying sunscreen lotions and creams. Sun protection products are numbered according to the sun protection factor (SPF), with the higher numbers giving more protection. Patients with vitiligo should use a sunscreen with an SPF of 15 or higher, except during times of treatment. During treatment, an SPF of 8 to 10 protects against sunburn but does not block the needed UV-A light. Sunscreens should be reapplied after swimming or perspiring, unless the sunscreen is formulated to resist wash-off. To prevent potential damage to the eyes, special sunglasses with protective lenses should be worn during sunlight exposure and for the remainder of any day on which psoralen drugs were taken.
Not all individuals with vitiligo are good candidates for repigmentation therapies. Good candidates generally meet the following requirements:
- Pigment loss of less than five years duration for patients over 20 years of age. In general, children and young adults respond better than older people.
- Patients should be at least 10 years old. Treatment is safe for younger children, but because treatments may be tedious to them, better results may be achieved when the child is interested in treatment. An individual must be committed to the treatment because it is a long process that requires a great deal of patience.
- Patients should be healthy. Any associated illness, such as hyperthyroidism, should be treated at the same time as the vitiligo.
- For PUVA therapy only, a pregnant woman should not be treated because of the potential harmful effects of the drug on her developing baby.
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If a person has vitiligo over more than half of the exposed areas of their body, he or she is not a good candidate for repigmentation. Rather, such a person may want to try depigmentation of the remaining pigmented skin. The drug for depigmentation is monobenzylether of hydroquinone (Benoquin). Many patients with vitiligo are at first apprehensive about the idea of depigmentation and are reluctant to go ahead. However, patients who achieve complete depigmentation are usually satisfied with the end results. Some people become allergic to the medication and must discontinue therapy. Moreover, depigmentation is not always permanent. Some patchy pigmentation may return. It can be re-treated under the advisement of a dermatologist.
Most patients, even those who are responding well to therapy, may want to make their vitiligo less obvious. Many find that a combination of cosmetics effectively conceals their skin disorder. Patients who are interested in dyes and stains should consult a dermatologist for the names of suitable commercial products.
At present, the answer is no. Vitiligo results from a variety of factors (genetic and environmental) interacting in unknown ways. While research has advanced our understanding of the physical and psycho-social causes underlying vitiligo, a cure for this disease does not currently exist. More research is required to fully understand the factors involved and to find ways to reverse or block them.
Research on vitiligo, defects of pigment cells, autoimmune response, and the associated genes has increased significantly in the past decade. Therapeutically, attempts to stop the progression of vitiligo by the use of topical immunosuppressants/immunomodulators and new phototherapy procedures appear promising. Skin transplantation procedures, where a dermatologist removes a patch of normally pigmented skin and grafts it onto a depigmented area, are progressing well. In addition, melanocyte transplant therapy, where a dermatologist takes pigment cells from an unexposed normally-pigmented patch of skin, grows them in culture to large numbers, and returns them into a white patch of skin, is also being developed.
There is much concern for the vitiligo patient, but there is relatively little emphasis on funding clinical and basic science research on this disease because it is not lethal and is often considered cosmetic in nature. The National Institutes of Health (NIH) awards about $500,000 annually toward pigment research programs. However, this pales in comparison to money awarded to research on cancer, AIDS, cardiovascular disease, etc. Despite this, research does continue in the United States and around the world to various degrees. Universities such as Denver, Loyola, Cincinnati, Howard, and Boston have active programs in vitiligo research (we maintain a research roadmap that identifies these and other research centers). But, for the reasons mentioned above, funds are sparse. It is because of this that your contributions to NVFI are so crucial for enabling support and research on better therapies and, ultimately, a cure.
The simplest way you can help is by joining our community and/or making a donation to the Foundation. If you are interested in being more actively involved, please contact us to discuss various volunteer opportunities.