Vitiligo Basics
Aaron Lerner, M.D. and
Gisela Moellmann, Ph.D. -
Department of Dermatology, Yale University

WHAT IS VITILIGO?
WHO GETS VITILIGO?
WHAT DETERMINES SKIN COLOR?
WHAT IS THE CAUSE OF VITILIGO?
HOW DOES VITILIGO DEVELOP?
EMOTIONAL IMPACT OF VITILIGO
WHAT IS THE TREATMENT FOR VITILIGO?
WHAT IS REPIGMENTATION THERAPY?
WHO IS A GOOD CANDIDATE FOR REPIGMENTATION?
WHAT IS DEPIGMENTATION THERAPY AND WHEN IS IT WARRANTED?
CAN COSMETICS BE USED TO MAKE VITILIGO LESS NOTICEABLE?
IS VITILIGO CURABLE?
THE FUTURE
DOES ANYONE CARE?
THE NATIONAL VITILIGO FOUNDATION CARES TOO!
HOW CAN OTHERS HELP
FOUNDATION OBJECTIVES

WHAT IS VITILIGO?
Patients with Vitiligo develop white spots in 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, and in the hair follicles, mouth, eyes, and some parts of the central nervous system. In Vitiligo, pigment cells can be lost in any of these areas. Common sites of pigment loss are:

• Exposed areas: hands, face, upper part of the chest
• Around body openings: eyes, nostrils, mouth, nipples, umbilicus, 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
• Choroids of the eye

WHO GETS VITILIGO?
Vitiligo affects at least 1% of the population. 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. 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).

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WHAT DETERMINES SKIN COLOR?
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.

WHAT IS THE CAUSE OF VITILIGO?
The precise cause of Vitiligo is not known. A combination of genetic, immunologic and neurogenic factors is of major importance in most cases. Many people report pigment loss shortly after a severe sunburn. Others relate the onset of Vitiligo to emotional trauma associated with an accident, death in the family, divorce, etc. Early graying of hair is part of Vitiligo. Patients with Vitiligo appear to have normal pigment cells. An increase in something such as nitric oxide, may be toxic for pigment cells or there may be a lack of growth factors that are required for normal pigment cells to be viable.

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HOW DOES VITILIGO DEVELOP?
The beginning of Vitiligo and the severity of pigment loss differs with each patient. Light skinned people usually notice the pigment loss during the summer as the contrast between the vitiliginous skin and the suntanned skin becomes distinct. People with dark skin may observe the onset of Vitiligo at any time. Individuals who have severe cases will 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, the 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 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.

EMOTIONAL IMPACT OF VITILIGO
Most individuals with Vitiligo are upset because of the uneven skin color. It is difficult for those who do not have Vitiligo to appreciate the significance of this problem to the victim. Patients with Vitiligo benefit from talking to other patients with Vitiligo.

WHAT IS THE TREATMENT FOR VITILIGO?
Even though treatment of Vitiligo is not completely satisfactory, there are two basic methods: to try to restore the normal pigment (repigment), or to try to destroy the remaining pigment cells (depigmented).

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WHAT IS REPIGMENTATION THERAPY?
To get repigmentation, new pigment cells must be produced from existing ones. 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 would provide pigment cells that radiate to one eighth to a quarter of an inch, there could be marked repigmentation. However, if the hairs are white or if there are no hairs, repigmentation must occur from the edge.

In repigmentation therapy, a patient is given a Psoralen drug and then is exposed to ultraviolet light A (UV-A). When psoralen drugs are activated by UV-A, they stimulate repigmentation by increasing the availability of color-producing cells at the skin's surface. The response varies among patients and body sites.

The psoralen drugs used for repigmentation therapy are trimethylpsoralen and 8-methyoxypsoralen. A patient takes the prescribed dose by mouth two hours before lying in the sun or under artificial ultraviolet (UV-A) light. The ideal time for natural sunlight is between 11 AM and 1 PM when the sun is highest. Treatment every other day is recommended. Too much ultraviolet light can be harmful.

Treatment schedules can be adjusted for each patient. If the day is cloudy or if sun exposure is not possible on a scheduled treatment day, then the patient does not take any medication because the drug does not work without appropriate sunlight. In the northern part of the United States, patients usually begin therapy in May and continue until September. Moderate repigmentation should take place during this time.

Treatment is usually discontinued during the winter. This rest period is desirable so that patients do not tire of continual treatment. Artificial sources of UV-A light can be used throughout the year, but patients should consult their dermatologist to determine whether such treatments are accessible and desirable. UV-A light systems for home use are expensive and treatment with them time consuming. Ordinary sunlamps are not effective with the psoralen medications. Only UV-A light produces the desired interaction. A dermatologist's supervision is required during all aspects of repigmentation therapy.

Patients 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 treatment time, and/or 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 1 5 or higher, except during the hours of treatment. During treatment, an SPF of 8 to I 0 protects against sunburn but does not block the UV-A needed for treatment. Sunscreens should be reapplied after swimming or perspiring. To prevent potential damage to the eyes, special sunglasses with protective lenses should be worn during sunlight exposure and for the remainder of the day on which the psoralen drug was taken. Another method of psoralen treatment, used occasionally for patients with small, scattered Vitiligo patches, involves the application of a solution of the drug directly to t he affected skin area. This is then exposed to sunlight. Such topical treatment makes a person very susceptible to severe burn and blisters following too much sun exposure.

Hydrocortisone-type compounds when applied to the skin slow the process of depigmentation and sometimes even enhance repigmentation. The weak cortisones that are sold over the counter, such as 0.5% hyrocortisone, are too weak to help. The very potent cortisones when used daily for a longtime 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 so that side effects are at a minimum.

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WHO IS A GOOD CANDIDATE FOR REPIGMENTATION?
All patients are not good candidates for re-pigmentation. The ideal person for this therapy should meet these 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 the method is tedious, better results are 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. No one with a sensitivity or allergy to sunlight can be treated. Any associated illness, such as hyperthyroidism, should be treated at the same time as the Vitiligo.
  
• The patient should have the opportunity to be exposed to sunlight for one or two hours three times a week during the summer months for a period of two to five years.
  
• A pregnant woman should not be treated because of the potential harmful effects of the drug on her developing baby. About 75% of the patients who undergo psoralen - UV-A light therapy respond to some extent. Even for these, complete repigmentation rarely occurs. After the initial two to three weeks of exposure to sunlight, patients will look worse since the contrast between light and tanned skin increases. With time, repigmentation will begin, and the appearance of the skin improves. If patients stop the therapy in winter, most will retain at least half of the color they achieved during the summer months.

WHAT IS DEPIGMENTATION THERAPY AND WHEN IS IT WARRANTED?
If a person has Vitiligo over more than half of the exposed areas of the body, he or she is not a 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. Many patients with Vitiligo are at first apprehensive about the idea of depigmentation and 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.

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CAN COSMETICS BE USED TO MAKE VITILIGO LESS NOTICEABLE?
Most patients, even those who are responding well to therapy, would like to make the Vitiligo less obvious. Many find that a combination of cosmetics effectively deemphasizes their skin disorder. Patients who are interested in dyes and stains should consult a dermatologist for the names of suitable commercial products.

IS VITILIGO CURABLE?
The precise answer at this time is no. Vitiligo is probably caused by a variety of factors interacting in specific ways. Research has advanced the understanding of the physical and psychosocial aspects of Vitiligo, but the cause and cure for this disease are unknown.

THE FUTURE
Research on pigment cells as related to Vitiligo has increased greatly in the past ten years. Attempts to stop Vitiligo by the use of hydrocortisone compounds applied to the skin appear promising. Melanocyte transplants, where the 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, is also promising.

DOES ANYONE CARE?
Yes, there is much concern for the patient with Vitiligo. The National Institute of Health grants approximately $500,000 annually toward pigment research programs. In addition, research continues in the United States and around the world to various degrees. Many Universities such as Cincinnati, Harvard, Howard and Yale, have active programs in Vitiligo research. But, as is often the case, funds are sparse.

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THE NATIONAL VITILIGO FOUNDATION CARES TOO!
Organized and initially funded in Tyler, Texas in September of 1985, the Foundation has begun to grow and reach out across the country with chapters now in many cities across the USA. Each chapter is helping by forming support groups of patients, families and physicians to inform, direct and counsel the patient. In addition, the chapters are actively trying to raise funds for research to hopefully find a cure for Vitiligo and a prevention of its occurrence.

HOW CAN OTHERS HELP
Anyone can help with their time and their money. Contact the National Vitiligo Foundation and volunteer. Send your contribution to further the work of the Foundation.

FOUNDATION OBJECTIVES

• To locate, inform and counsel Vitiligo patients and their families.
  
• To increase public awareness and concern for the Vitiligo patient.
  
• To broaden the concern for the patient within the medical community.
  
• To encourage, promote and fund increased scientific and clinical research on the cause, treatment and ultimate cure of Vitiligo.
  
• To establish a central Vitiligo center with local treatment facilities around the country.

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