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Vitiligo

What is Vitiligo? What causes it? How does it spread? Who does it affect? How can it be treated? Read on to learn everything you need to better understand this skin hypopigmentation disorder.
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What is Vitiligo?

Vitiligo is a pigment disorder that affects the skin. It is primarily the result of the destruction of the melanocytes (the cells responsible for the skin pigmentation).

When vitiligo affects the skin, white patches appear on it. These white patches can also appear on mucosal membranes and the retina. Often, the hairs growing in the part of the skin affected by vitiligo also turn white.

Melanocytes are pigment cells found at the bottom of the epidermis and just before the dermis layer of the skin. They are found more abundantly in dark-skinned people than in light-skinned people.

When sunlight shines on the skin, it activates melanocytes and stimulates them to produce melanin.

However, when melanocytes self-destruct or are destroyed by autoimmune attack (it is still not clear what happens exactly), the number of these cells in some areas of the skin may drop significantly. As melanocytes die out, the skin loses the ability to produce melanin. With less melanin, the skin cannot turn dark or tan but appears gray or white.

The white spots seen with vitiligo mark the places where there are very few or no melanocytes in the epidermis.

Presentation and Progression of Vitiligo

Vitiligo can affect any part of the body but it is mostly found on the face, hands, wrists, armpits, neck and scalp.

Vitiligo lesions can also affect all the orifices of the body including the mouth, eyes, nostrils, nipples, genitals, navel and rectum.

The white patches of skin which has experienced depigmentation are called vitiligo lesions. Some of these vitiligo lesions do have hyperpigmentation marking the edges of the depigmentation patches.

These lesions can also appear symmetrically. This means that white patches of vitiligo lesions may appear on about the same areas on both arms, hands or legs.

The first sign of vitiligo are small areas of milky white patches on the skin. These may go unnoticed in white-skinned people (except during the summer months when the skin turns tan) but it becomes fairly noticeable in dark skin people.

For some people, vitiligo lesions may stop at this stage. For some, the lesions do dormant for a long while. However, some people experience rapid progression of vitiligo lesions which may spread to other parts of the body. 

When vitiligo spreads, the white lesions grow in size and change shapes.

Vitiligo is a relatively mild skin condition. It is not painful and it is not contagious; it does not itch and there are no sores. People living with vitiligo do not have any other symptoms besides the white patches on the skin. However, these patches can make the skin unsightly and cause depression in stigmatized patients.

Furthermore, since the skin patches are without melanin vitiligo lesions are more prone to sunburn than normal areas of the skin.

Who Gets Vitiligo?

Vitiligo is not a common skin disease. Overall, it affects 1 in 100 people and it affects both men and women equally. 50% of those affected are first diagnosed in their twenties and most people who get it experience it before they turn 40.

Even though vitiligo affects people of all races equally, it is most noticeable in dark-skinned people.

Vitiligo has a genetic component. In some families, it is an inheritable trait. It is estimated that 30% of people living with the skin condition have another member of their families with the same condition. 

However, this hereditary link is not too strong. For example, only 5 – 7% of children of vitiligo parents will inherit the condition.

Other Diseases linked to Vitiligo
  • Alopecia areata (baldness)
  • Hyperthyroidism e.g. Hashimoto thyroiditis and Graves disease
  • Pernicious anemia
  • Inflammatory bowel disease
  • Psoriasis
  • Diabetes mellitus
  • Adrenocortical insufficiency e.g. Addison’s disease

While vitiligo is associated with these autoimmune diseases, it is by no means a way to diagnose or suspect another autoimmune disease. This is because most people with vitiligo do not have any other autoimmune disease.

Clinical Aspects of Vitiligo

The exact cause of vitiligo is unknown. However, the most popular theory is that it is caused by autoimmune attack on the melanocytes. Some of the proposed causes of vitiligo are discussed below.

Autoimmune Theory

Clinical evidence for the autoimmune cause of vitiligo is provided by the presence of specific antibodies in vitiligo patients. Specifically, CD8+ T cells have been found in vitiligo lesions. These T cells are believed to be directly involved in the destruction of melanocytes. Antibodies specific to melanocyte have been observed in the blood.

Some experts believe vitiligo is also caused by genetic defects. These defects have been seen in the genes expressed in the immune system and the melanocytes.

For example, the gene TYR has been shown to increase the risk of vitiligo by increasing the susceptibility of the melanocytes to the immune system. However, because the melanocytes are easily affected by the immune cells, the same gene is responsible for protecting vitiligo patient against melanoma.

Besides the strong link between some autoimmune diseases and vitiligo, the skin condition has also been linked to inflammatory diseases.

Defective Melanocyte Theory

Some studies have shown that melanocytes in vitiligo patients are intrinsically defective and prone to quick cell death.

Observed abnormalities in melanocytes include inefficient cellular metabolism and ineffective regulation of receptors.

Oxidative Stress Theory

The accumulation of free radicals can also increase the rate at which melanocytes die off. This oxidative stress is attributed to hydrogen peroxide which leads to the accumulation of oxidized pteridines.

Two of such oxidized pteridines are responsible for diagnostic yellowish green or bluish fluorescence observed when vitiligo patches are examined under certain lights.

In addition, the oxidative stress is also responsible for the high levels of the antioxidant enzyme superoxide dismutase and the low levels of the enzyme, catalase, in vitiligo patients when compared to control subjects.

Neural Theory

There have been cases of patients who developed vitiligo after nerve injury. This is attributed to the loss of melanin due to the loss of neurochemicals released from active nerve endings.

However, in some cases melanocytes can still survive for years and long enough to be revived when those nerves are re-stimulated.

Other neural activities have been reported in depigmented patches of vitiligo. These include increased sweating and constriction of the blood vessels. Also in vitiligo patients, there is increased excretion of metabolites of neurochemicals such as homovanillic acid and vanilmandelic acid.

Classification of Vitiligo

There are 3 systems of classifying vitiligo. These are explained below.

Classification by Color

Trichrome Vitiligo: For this type of vitiligo, the patches have 3 colors: brown, tan and white which correspond to the 3 different zones including unaffected skin at the edges, hypochromia (with very few melanocytes) and achromia (with no melanocyte). The tan zone then slowly turns to white.

Quadrichrome and Pentachrome Vitiligo: These are variations of trichome vitiligo but with additional colors (dark brown etc.) observed around the hair follicles and attributed to repigmentation of some of the tan or white areas.

Blue Vitiligo: This is characterized by the blue color of vitiligo patches. It is seen in patients who just had skin inflammation which was deeply pigmented before the onset of vitiligo.

Other variations of the above include marginal inflammatory vitiligo which is differentiated by the red, inflamed borders of the color zones and mild itching. Another variation is Koebner phenomenon which occurs when vitiligo only affects areas of the skin that recently experienced some injuries such as irritation, cuts or burns.

Classification by Location

Localized Vitiligo

There are 3 subtypes in this class:

Focal: This is characterized by macules found only in one location on the skin. The most common location for this type of vitiligo is along the nerves of the face and jaws.

Segmental: This type of vitiligo mostly affects children and it is not caused by autoimmune or hormonal (thyroid hormone) disorders. Half of the patients in this class have patches of white hairs.

Mucosal: This type of vitiligo only affects the mucosal membranes.

Generalized Vitiligo

There are also 3 subtypes in this class:

Acrofacial: The loss of melanocytes and white patches occur on the fingers and body orifices.

Vulgaris: The white patches for this type of vitiligo are scattered and distributed randomly.

Mixed: This is a combination of acrofacial and vulgaris or a combination of segmental and generalized vitiligo.

Universal Vitiligo: This is a full or almost full depigmentation that affects the whole body. It is caused by multiple hormonal factors.

Classification by Progression

Segmental Vitiligo: This type of vitiligo starts early in life and progresses rapidly. The white patches may also last a lifetime.

Non-segmental Vitiligo: This refers to all kinds of vitiligo that do not fall under the definition for segmental vitiligo.

Non-segmental vitiligo is more likely to be caused by autoimmune disorders and inflammation than segmental vitiligo. It is also more likely to be inheritable.

Vitiligo Treatment

Vitiligo has no cure but its progression can be considerably slowed down and even its appearance on the skin can be improved. No one treatment works for everyone and the treatment of choice will depend on how far the skin disorder has progressed.

If the white patches are only found in body parts always kept covered up, and if the vitiligo remains local to that area, it may be left untreated. For other cases, treatment may be necessary.

There are 4 basic ways of treating Vitiligo.

  • Covering up the white patches or camouflaging the skin
  • Reversing the changes in the skin (applicable when there are still some melanocytes left to be activated)
  • Completely depigmenting the skin to produce an even skin tone
  • Protecting the depigmented, pale skin from sun damage

Skin Camouflage

Covering up vitiligo patches is only effective for mild cases of the skin disorder.

The aim of camouflaging is to match the skin color of the unaffected areas of the skin. Since the unaffected areas are always darker than the vitiligo lesions, special cosmetic camouflage creams are used to disguise the areas of the skin affected.

However, finding the perfect color to match the rest of the skin can be near impossible. Therefore, skin camouflage is most effective when large areas of the skin need to be covered.

Self-tanning lotions which are sold without prescriptions may also help cover vitiligo patches. These solutions can only provide a rough color match for the skin and they last for only a few days.

To make skin camouflage solutions more effective, users most avoid prolong exposure to sunlight which can tan the unaffected areas of the skin even darker.

Repigmentation of the Skin

There are vitiligo treatment options that directly aim to reverse the color change on the skin by reactivating melanocytes and boosting the production of melanin. These solutions have to be used early on in the progression of the disease.

Topical Therapy

Topical steroids are very effective for arresting the spread and even reversing the white patches of vitiligo.

Steroid creams must be applied early on and they should be used for at least 3 months before judging their efficacies. Mild steroid creams are prescribed for children and also for use in specific areas of the body where the skin is thin. These areas include the face, armpits and genitalia.

Steroids work by suppressing the immune system and saving melanocytes from autoimmune attack.

Even though steroid creams are simple to use and sometimes effective, their long-term use is discouraged because of the extensive side effects of steroids. On the skin, steroids can cause stretch marks and it can also thin the skin.

Alternatively, tacrolimus (0.03 – 0.1%) ointment is used instead of steroid cream. It works the same way as steroid creams (by suppressing cells of the immune system). Tacrolimus is especially prescribed for children and for use on the face.

A related drug, pimecrolimus 1% cream, is also used for treating vitiligo in children. It works better when combined with microdermabrasion.

Psoralen Photochemotherapy or PUVA

PUVA is also called psoralen and ultraviolet A therapy. The basic principle involves stimulating skin pigmentation by first taking a drug (psoralen) and then exposing the skin to ultraviolet A light from a special lamp.

Psoralen is a photosensitizing drug that is activated by this spectrum of ultraviolet light.

Once activated, psoralen darkens the skin. PUVA therapy can take 6 – 12 months to finish and it requires repeated visits to the doctor’s office.

Topical Psoralen Photochemotherapy:  This is used where there are only a few, localized vitiligo patches and the depigmentation is not extensive. Topical PUVA is not used in children under 2 years.

It requires once or twice weekly visits to the doctor’s office. First, a thin coat of psoralen is applied on the white patches and allowed to stay for 30 minutes before the skin is irradiated with ultraviolet light. Initially, the white patches turn pink but the pink fades with subsequent treatments and then turn into normal skin color.

Topical PUVA therapy increases the risk of sunburn. Therefore, patients are advised to avoid direct sunlight and use sunscreens.

Oral Psoralen Photochemotherapy: Oral PUVA is used when topical PUVA fails or when vitiligo affects more than 20% of the body (in which case ultraviolet irradiation is impractical). However, oral PUVA is not recommended for children under 10 years because of the increased risk of damage to the eyes.

In this therapy, an oral dose of psoralen is taken 2 hours before the light therapy.

Light therapy can either be by ultraviolet A rays or sunlight. When UVA light is used, at least a day must be allowed between each visit to the doctor. Patients are advised to avoid sunlight and use sunscreen and UVA sunglasses for 24 – 48 hours after each treatment of oral psoralen.

Side effects of oral psoralen include itching, sunburn, hyperpigmentation, abnormal hair growth, nausea and vomiting.

Narrowband UVB Phototherapy

Besides UVA, UVB lamps can also be used especially for spot treatment.

UVB is now more commonly used than UVA irradiation. It does not need photosensitizing drugs like psoralen and it can be done at home. UVB causes lesser damage than UVA and its treatment sessions are shorter.

Photochemotherapy works better especially when combined with vitamin supplements such as folic acid and vitamin B12.

Surgery

Surgery is the last resort for treating vitiligo. It is usually recommended for those who have treated vitiligo for more than 3 years. It is time-consuming and expensive.

Below are the main types of surgical treatment for vitiligo.

Autologous Skin Graft: This involves removing some skin from pigmented areas and transferring them to depigmented areas. It is only useful if the vitiligo covers small and few patches of the skin.

Skin Graft with Blisters: This involves creating blisters on pigmented areas of the skin with heat, cold or suction. The blisters are then cut and transferred to the depigmented areas.

Micropigmentation or Tattoing: This involves surgically implanting pigments in the skin. However, exactly matching graft pigments to normal skin pigments can be difficult. It is usually done on the lips and for dark-skinned people. These implanted pigments fade over time and may leave marked discoloration on the skin.

Autologous Melanocyte Transplant: This involves taking a cut of normal pigmented skin and growing it under laboratory conditions. The multiplied melanocytes are then transferred back to the depigmented areas of the skin. This is only an experimental procedure and very costly too.

Depigmentation of the Skin

Where repigmentation does not produce acceptable results, depigmentation may be considered. It is considered the best treatment option for patients with more than half of their skins covered by vitiligo patches.

However, patients must understand that the procedure is permanent and that their skins will turn white.

To depigment the skin, monobenzone is applied to the areas of the skin still pigmented two times daily. Following this, skin-to-skin contact should be avoided for 2 hours after application.

Side effects of depigmentation include itching, dry skin and increased sensitivity to sunlight.

Sources


http://emedicine.medscape.com/article/1068962-overview#showall

http://www.medicinenet.com/vitiligo/article.htm

http://www.medicinenet.com/vitiligo/page4.htm

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