What is Vitiligo?

Vitiligo is a disease in which the pigment cells of the skin, melanocytes, are destroyed in certain areas.

Vitiligo results in depigmented, or white, patches of skin in any location on the body.

Vitiligo can be focal and localized to one area, or it may affect several different areas on the body.

The exact cause of vitiligo is unknown, although most experts believe that it is an autoimmune condition in which the body’s immune system mistakenly attacks and destroys certain cells within the body.

Most people who have vitiligo will develop the condition prior to age 40; about half develop it before age 20.

Vitiligo tends to run in families.

Vitiligo is sometimes associated with other medical conditions, including thyroid dysfunction.

Vitiligo is not painful and does not have significant health consequences; however, it can have emotional and psychological consequences.

Some medical treatments can reduce the severity of the condition, but it can be difficult to cure.

What is vitiligo, and what causes it?

Vitiligo (pronounced vit-ill-EYE-go) is a pigmentation disorder in which melanocytes (the cells that make pigment) in the skin are destroyed. As a result, white patches appear on the skin in different parts of the body. Similar patches also appear on both the mucous membranes (tissues that line the inside of the mouth and nose) and the retina (inner layer of the eyeball). The hair that grows on areas affected by vitiligo sometimes turns white.

The cause of vitiligo is not known, but doctors and researchers have several different theories. There is strong evidence that people with vitiligo inherit a group of three genes that make them susceptible to depigmentation. The most widely accepted view is that the depigmentation occurs because vitiligo is an autoimmune disease – a disease in which a person’s immune system reacts against the body’s own tissues. People’s bodies produce proteins called cytokines that, in vitiligo, alter their pigment-producing cells and cause these cells to die. 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 as causes of vitiligo.

Who is affected by vitiligo?

About 0.5 to 1 percent of the world’s population, or as many as 65 million people, have vitiligo. In the United States, 1 to 2 million people have the disorder. Half the people who have vitiligo develop it before age 20; most develop it before their 40th birthday. The disorder affects both sexes and all races equally; however, it is more noticeable in people with dark skin.

Vitiligo seems to be somewhat more common in people with certain autoimmune diseases, including 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 anaemia (a low level of red blood cells caused by the 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. In fact, 30 percent of people with vitiligo have a family member with the disease. However, only 5 to 7 percent of children will 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 commonly found on sun-exposed areas of the body, 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, genitals, and rectum.

Vitiligo generally appears in one of three patterns:

  • focal pattern — depigmentation limited to one or only a few areas
  • segmental pattern — depigmented patches that develop on one side of the body
  • generalized pattern — the most common pattern. Depigmentation occurs symmetrically on both sides of the body.
  • In addition to white patches on the skin, people with vitiligo may have premature greying of the scalp hair, eyelashes, eyebrows, and beard. People with dark skin may notice a loss of colour inside their mouths.

Will the depigmented patches spread?

Focal pattern vitiligo and segmental vitiligo remain localized to one part of the body and do not spread. There is no way to predict if generalized 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?

The diagnosis of vitiligo is made based on a physical examination, medical history, and laboratory tests. A doctor will likely suspect vitiligo if you report (or the physical examination reveals) white patches of skin on the body-particularly on sun-exposed areas, including the hands, feet, arms, face, and lips. If vitiligo is suspected, the doctor will ask about your medical history. Important factors in the diagnosis include a family history of vitiligo; a rash, sunburn, or other skin trauma that occurred at the site of vitiligo 2 to 3 months before depigmentation started; stress or physical illness; and premature greying of the hair (before age 35). In addition, the doctor will ask whether you or anyone in your family has had any autoimmune diseases and whether you are very sensitive to the sun.

To help confirm the diagnosis, the doctor may take a small sample (biopsy) of the affected skin to examine under a microscope. In vitiligo, the skin sample will usually show a complete absence of pigment-producing melanocytes. On the other hand, the presence of inflamed cells in the sample may suggest that another condition is responsible for the loss of pigmentation.

Because vitiligo may be associated with pernicious anaemia (a condition in which an insufficient amount of vitamin B12 is absorbed from the gastrointestinal tract) or hyperthyroidism (an overactive thyroid gland), the doctor 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), which sometimes occurs with vitiligo. 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.

What treatment options are available?

The main goal of treating vitiligo is to improve 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; their location, sizes, and how widespread they are; and what you prefer in terms of treatment. Each patient responds differently to therapy, and a particular treatment may not work for everyone. Current treatment options for vitiligo include medication, surgery, and adjunctive therapies (used along with surgical or medical treatments).

  • Medical therapies

A number of medical therapies, most of which are applied topically, can reduce the appearance of vitiligo. These are some of the most commonly used:

o   Topical steroid therapy. Steroid creams may be helpful in repigmenting (returning the colour to) white patches, particularly if they are applied in the initial stages of the disease. Corticosteroids are a group of drugs similar to hormones such as cortisone, which are produced by the adrenal glands. Doctors often prescribe a mild topical corticosteroid cream for children under 10 years old and a stronger one for adults. You must apply the cream to the white patches on the skin for at least 3 months before seeing any results. Corticosteroid creams are the simplest and safest treatment for vitiligo, but are not as effective as psoralen photo chemotherapy (see below). Yet, as with any medication, these creams can cause side effects. For this reason, the doctor will monitor you closely for skin thinning and skin striae (streaks or lines on the skin). These side effects are more likely to occur in areas where the skin is already thin, such as on the face and armpits, or in the genital region. They can be minimized by using weaker formulations of steroid creams in these areas.

o   Psoralen photo chemotherapy. Also known as psoralen and ultraviolet UVA therapy (PUVA) therapy, this is probably the most effective 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 expensive, and care must be taken to avoid side effects, which can sometimes be severe. Psoralen is a drug that contains 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 sunlight or to ultraviolet A (UVA) light that comes from a special lamp. Typically, you will receive treatments in your doctor’s office so you can be carefully watched for any side effects. You must minimize exposure to sunlight at other times. Both oral and topical psoralen photo chemotherapy are described below.

o   Topical psoralen photo chemotherapy. Often it is used for people with a small number of depigmented patches affecting a limited part of the body and for children age 2 and older who have localized patches of vitiligo. Treatments are done in a doctor’s office under artificial UVA light two or three times a week. The doctor or nurse applies a thin coat of psoralen to your white patches about 30 minutes before exposing you to enough UVA light to turn 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 colour appears. After each treatment, you wash your skin with soap and water and apply 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 (hyperpigmentation) of the treated patches or the normal skin surrounding the vitiligo.

You can minimize your chances of sunburn if you avoid exposure to direct sunlight after each treatment. Usually, hyperpigmentation is a temporary problem that eventually disappears when treatment is stopped.

o   Oral psoralen photo chemotherapy. Used for people with extensive vitiligo (affecting more 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 it increases the risk of damage to the eyes if they are not adequately protected. For oral PUVA therapy, you take a prescribed dose of psoralen by mouth about 2 hours before exposure to artificial UVA light or sunlight. If artificial light is used, the doctor adjusts the dose of light until the skin in the areas being treated becomes 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 facility to receive PUVA therapy, the doctor may prescribe psoralen that can be used with natural sunlight exposure. The doctor will give you careful instructions on carrying out treatment at home and monitor you during scheduled check-ups. Known side effects of oral psoralen include sunburn, nausea and vomiting, itching, abnormal hair growth, and hyperpigmentation. Oral psoralen photo chemotherapy may also increase the risk of skin cancer. If you are undergoing oral PUVA therapy, you should apply sunscreen and avoid direct sunlight for 24 to 48 hours after each treatment to avoid sunburn and reduce the risk of skin cancer. To avoid eye damage, particularly cataracts, you should also wear protective UVA sunglasses for 18 to 24 hours after each treatment.

o   Depigmentation. This treatment involves fading the rest of the skin on the body to match the areas that are already white. For people who have vitiligo on more than 50 percent of their bodies, depigmentation may be the best treatment option. Patients apply the drug monobenzyl ether of hydroquinone (monobenzone) twice a day to pigmented areas until they match the already-depigmented areas. You must avoid direct skin-to-skin contact with other people for at least 2 hours after applying the drug, as transfer of the drug may cause depigmentation of the other person’s skin. The major side effect of depigmentation therapy is inflammation (redness and swelling) of the skin. You may experience itching or dry skin. Depigmentation tends to be permanent and is not easily reversed. In addition, a person who undergoes depigmentation will always be unusually sensitive to sunlight.

  • Surgical therapies

All surgical therapies must be considered only after proper medical therapy has failed. Surgical techniques are time-consuming and expensive and usually not paid for by insurance carriers.

They are appropriate only for carefully selected patients who have vitiligo that has been stable for at least 3 years:

o   Autologous skin grafts. The doctor removes skin from one area of your 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 many people find it neither acceptable nor affordable.

o   Skin grafts using blisters. In this procedure, the doctor creates blisters on your 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 scarring and lack of repigmentation. However, there is less risk of scarring with this procedure than with other types of grafting.

o   Micropigmentation (tattooing). This procedure involves implanting pigment into the skin with a special surgical instrument. It works best for the lip area, particularly in people with dark skin. However, it is difficult for the doctor to match perfectly the colour of the skin of the surrounding area. The tattooed area will not change in color when exposed to sun, although the surrounding normal skin will. So even if the tattooed area matches the surrounding skin perfectly at first, it may not later on. 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.

o   Autologous melanocyte transplants. In this procedure, the doctor takes a sample of your 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 your depigmented skin patches. This procedure is currently experimental and is impractical for the routine care of people with vitiligo. It is also very expensive, and its side effects are not known.

  • Additional therapies

In addition to medical and surgical therapies, there are many things you can do on your own to protect your skin, minimize the appearance of white patches, and cope with the emotional aspects of vitiligo:

o   Sunscreens. People, who have vitiligo, particularly those with fair skin, should minimize sun exposure and use a sunscreen that provides protection from both UVA and UVB light. Tanning makes the contrast between normal and depigmented skin more noticeable. Sunscreen helps protect the skin from sunburn and long-term damage.

o   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. Many cosmetic companies offer makeup or dyes that you may find helpful for covering up depigmented patches. Self-tanning lotions have an advantage over makeup in that the colour will last for several days and will not come off with washing.

o   Counselling and support groups. Many people with vitiligo find it helpful to get counselling from a mental health professional. People often find they can talk to their counsellor about issues that are difficult to discuss with anyone else. A mental health counsellor can also offer support and help in coping with vitiligo. In addition, it may be helpful to attend a vitiligo support group.