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All you need to know about ... vitiligo

Trish Garibaldinos
RGN
Clinical Nurse Specialist

Jane Smith
RGN
Deputy Unit Manager

St John's Institute of Dermatology
Guy's and St Thomas' Foundation Trust
London

Vitiligo is the most common acquired depigmenting skin disorder where the pigment-producing cells, melanocytes, are gradually destroyed within the epidermis, leading to sharply defined milky white patches on the skin (see Figure 1). Records of this condition go back thousands of years - it is mentioned in both the Koran and the Bible. The incidence of vitiligo is about 1% of the population world-wide with no discrimination between races, sex or age.

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Healthy skin contains melanin, a yellow, brown or black pigment produced by melanocytes in the skin. These pigments determine your skin, hair and eye colour.  Vitiligo is classified according to the extent and distribution of the disease. Vitiligo is not infectious or contagious, does not affect people physically but can cause major psychological and emotional problems and have adverse effects on quality of life.(1)

What is the cause?
The exact cause of vitiligo remains unclear although advances in the understanding of this disease have led to four possible theories:(2)

  • Autoimmune theory: This theory suggests that an autoimmune response occurs where antibodies are produced to the body's own melanocytes and destroys them.
  • Neuronal theory: This theory suggests that certain chemical mediators are released from nerve endings into the epidermis causing destruction of the melanocytes.
  • Self-destruction theory of Lerner: This theory suggests that melanocytes are destroyed because there is a failure of or reduction in the elimination of toxic metabolites produced during melanin synthesis.
  • Composite theory: This theory brings together elements of the above three theories.

Who is most at risk?
There is a recognised association between vitiligo and other autoimmune diseases (see Box 1). It is thought that vitiligo may also be triggered by certain events, such as severe sunburn, injury or emotional stress.

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There is thought to be a genetic factor, as about 30-40% of patients have another family member with the disease.
Vitiligo affects people of all ethnic origins, but it is much more noticeable in people with darker skin.

What are the signs and symptoms?
Vitiligo can start at any age, but about 50% of cases develop before the age of 20. The course of the disease is hard to predict but most people experience a slow progression with gradual pigmentation loss as melanocytes deplete. Sometimes pigment is lost very rapidly over several months then stabilises and remains quiescent for a number of years. Vitiligo is usually first noticed on sun-exposed areas of the skin, such as the face and the dorsa of the hands, which become very prone to sunburn as the melanocytes disappear. Darker areas of skin in the axillae, groins and genitals may also be affected. Areas of skin subjected to repeated friction or trauma, such as the dorsa of the hands and feet, elbows, knees and ankles are also affected. Injury to the skin following a scratch or a burn often results in loss of pigmentation - this is known as the Koebner phenomenon. Rarely the pigment in the eyes may be affected.(3)
In fairer skin individuals vitiligo may be hardly noticeable apart from when the uninvolved skin becomes tanned and makes the white area more pronounced. 
Scalp hair, eyebrows and eyelashes that grow in areas affected by vitiligo may also turn white when the patches are present for some time. Mucous membranes that line the inside of the mouth, nose, genital and rectal areas can also be affected. The patches of vitiligo feel the same as the rest of the skin and usually do not itch.
The distribution of the patches is usually symmetrical and may be localised or generalised. Complete loss of all skin pigment is very rare.

There are two main types of vitiligo:

  • Generalised vitiligo: the patchy white areas are often symmetrical with patches on one side of the body roughly mirroring those on the other side. The lesions often have definite borders, sometimes with the margins becoming hyperpigmented and increasing irregularly in size. It may affect or spread to any part of the body (see Figure 1).
  • Segmental vitiligo: the white patches usually stay on one side of the body, and they do not spread after the first six months. It may follow a dermatomal distribution (see Figure 1). 

How is it diagnosed?
The diagnosis of vitiligo is made by skin assessment and by taking a history of both the patient and their family.  Vitiligo usually presents as progressive very white patches in typical sites, usually in a symmetrical pattern on both sides of the body. Often a family member has either vitiligo or an autoimmune disease, such as diabetes or thyroid disease. The age of onset and hyperpigmented border will usually point to a diagnosis of vitiligo. However, in the early stages of the disease, when the patches present with reduced pigmentation rather than complete loss of pigment, diagnosis can be difficult. It is important to exclude other dermatological conditions that can present with reduced pigmentation, known as postinflammatory hypopigmentation such as psoriasis and eczema. Careful examination of the whole skin and the texture of the lesions are very important to confirm the diagnosis. A special ultraviolet lamp called a Wood's light can help to outline the patches and show the extent of loss of pigmentation. In rare cases vitiligo can be confused with other skin disorders with pigment loss such as piebaldism, albinism, pityriasis alba and pityriasis versicolor. 
Blood tests may be done to rule out other auto-immune diseases (see Box 2).

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What are the current best treatments?
The treatments available are often unsatisfactory, lengthy and do not offer a permanent cure. Some newer treatments are not available on the NHS and are very expensive for patients to access privately.
The choice of treatment depends on the number of white patches, how widespread the disease is, the patient's age and the patient's preference.

Sunscreens
Although sunscreens are not a treatment they are important in protecting vitiligo skin from sunburn. Because melanin is either absent or inactive in the white patches there is no protection from the effects of ultraviolet light and sunburn can occur very easily, even on cloudy days. There is concern that the vitiligo may spread due to sunburn.
A high-protection, broad-spectrum sunscreen providing the maximum UVB and UVA sun protection factor should be used and reapplied regularly on sun-exposed skin even on cloudy days. Sunscreens can also help to minimise tanning to the pigmented skin and make the contrast less noticeable. They will also protect from the long-term possible effects of photoaging and skin cancer.  Appropriate clothing such as tightly woven materials, hats and sunglasses should be worn especially during the summer months to provide protection
(see Box 3).

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Corticosteroids
Topical steroid therapy may be helpful when used on patches of vitiligo of recent onset using either potent or very potent types in order to maximise effectiveness. As there is greater absorption of steroids in the skin on the face and genitals less potent steroids, mild to moderate, are prescribed. Also young children are usually treated with mild-to-moderate steroids. Topical steroids need to be applied one to two times per day for at least three months to assess effectiveness. There is often concern with the use of topical steroids because of the potential adverse effects such as thinning of the skin and stretch marks, but these are rarely seen when used correctly over the short term in the treatment of vitiligo.
 
Calcineurin inhibitors
More recently topical calcineurin inhibitors such as tacrolimus and pimecrolimus have been used in vitiligo to try and stop the inflammatory process. Their effectiveness has yet to be determined.
 
Camouflage
For patients who want to cover their vitiligo, cosmetic camouflage can be very effective, but is best used on the face and neck as it is rubbed off by clothing and friction. The British Red Cross offer a free service with around 250 camouflage clinics in many NHS hospitals across the UK. The volunteers are highly trained and are very skilled in mixing colour tones to achieve a very close match to the normal surrounding skin. Patients are taught how to mix and apply the make-up themselves. Most of the make-up used can be obtained on prescription from the GP. 

Fake tans
Unlike camouflage make-up that sits on top of the skin, fake-tanning products actually penetrate into the skin and bind to the stratum corneum. They contain a chemical called dihydroxyacetone which produces a brown colour that builds up over two to three days and lasts a few more days if application stops. It is never a perfect match, particularly for dark brown or black skin, but has the advantage of being waterproof and does not rub off on clothing, and is therefore suitable for use on arms and legs.
 
Photochemotherapy (PUVA)
PUVA has been used to treat vitiligo for many years. It combines a photosensitising drug (psoralen) taken orally or applied topically, with long-wave ultraviolet A. The psoralen sensitises the skin to the UVA and can sometimes stimulate inactive melanocytes to migrate from around the hair follicles and repigment the vitiligo skin. The psoralen is absorbed by the eye and patients need to wear glasses that block long-wave UV for up to 12 hours after ingestion. This treatment is given in secondary care and requires the patient to attend hospital twice weekly for many months; it is therefore often not practical for the patient due to work and family commitments.
PUVA is rarely completely successful or permanent with about 30% achieving good repigmentation and 75% of this group relapsing within two to three years. Patients need to be carefully screened and informed of the long-term side-effects including premature aging and skin cancer. There is a lifetime limit on the number of PUVA treatments because of the risk of nonmelanoma skin cancers 

Narrowband UVB (TLO-1) phototherapy
Narrowband UVB has been used in recent years to treat vitiligo and evidence suggests that it is as effective as and probably safer than PUVA in terms of skin cancer risk. It has the advantage of not requiring UV sensitising tablets or wearing special glasses for 12 hours. The repigmentation with narrowband UVB is usually a better match to the patient's normal skin than PUVA repigmentation, which can be much darker. The patient needs to attend hospital twice weekly for treatments with a review at four months to determine whether or not the treatment is working. Again the treatment can go on for many months and so demands a great commitment from the patient.(3)

Depigmentation
Where there is widespread depigmentation a bleaching agent such as monobenzyl ether of hydroquinone can be used to remove the remaining areas of pigment. It can cause skin irritation, contact dermatitis and in rare cases ochronosis, a yellowish degeneration of the dermal connective tissue. This is applied twice daily to the remaining pigmented areas of skin over a number of months until pigment fades and then disappears. This agent is usually prescribed in secondary care by an experienced dermatologist and requires careful pretreatment counselling and supervision as the treatment may have cultural consequences for patients with brown or black skin. Repigmentation is sometimes possible when treatment stops although it may be irreversible. The removal of pigmentation will make the patient extremely sensitive to light.

Skin grafting
This is a specialised treatment and is only available in a few centres. It involves taking punch biopsies from normal pigmented skin and grafting small sections grafted into small incisions in the vitiligo skin. They are then secured with tape and left in situ for a week. Multiple grafts are needed and are more suitable for small areas of stable vitiligo. Melanocytes may then grow out from the grafts into the vitiligo skin.

Support and counselling
The change in appearance caused by vitiligo can have a major impact on a person psychologically and emotionally. Some people find it very difficult to cope with normal daily living such as work and social activities. Vitiligo can affect confidence, self-esteem and relationships and the constant anxiety of changes in a person's appearance as more white patches appear is difficult to live with. Many people admit to feeling self-conscious and embarrassed especially when vitiligo is on skin exposed such as face and hands, and they have to deal with stares and comments from other people. Children and adolescents also face particular problems with teasing and bullying from their peers at school.(4)
Counselling and support from family, friends and professional groups is essential to help people cope with this disease. Organisations such as The Vitiligo Society and Changing Faces provide invaluable help, support and information for people with vitiligo (see Resources). It is important that healthcare professionals are supportive and well informed of the treatment options and support available.(1)

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References

  1. Vitiligo Society. Vitiligo - understanding the loss of skin colour. 2nd ed. London: Vitiligo Society; 2002.
  2. Bleehan SS. Disorders of skin colour. Rook/Wilkinson/Ebling: Textbook of dermatology. In: Champion RH, Burton JL, Burns T, Breathnach SM, editors. Oxford: Blackwell Science; 1998.
  3. Gawkrodger DJ. Diagnosing and treating vitiligo. Independent Nurse 2007;Jan/Feb:22.
  4. Bor R, Papadopoulos L. Psychological approaches to dermatology. Oxford: Blackwell Publishing; 1999.

Resources
British Association of Skin Camouflage
PO Box 202
Macclesfield
Cheshire SK11 6FP
W: www.skin
camouflage.net

British Red Cross
(contact the Therapeutic Beauty Care officer at local branch)
W: www.redcross.org.uk/skincamouflage

Changing Faces
The Squire Centre
33-37 University Street London WC1E 6JN
W: www.changingfaces.org.uk
The Vitiligo Society 125 Kennington Road London SE11 6SF
T: 0800 018 2631
W: www.vitiligosociety.org.uk