What is vitiligo?
Vitiligo is an acquired depigmenting disorder of the skin, in which pigment cells (melanocytes) are lost. It presents with increasing numbers of well-defined milky-white patches of skin.
Who gets vitiligo?
Vitiligo affects 0.5–1% of the population, and occurs in all races. It may be more common in India, with reports of up to 8.8% of the population affected. In half of sufferers, pigment loss begins before the age of 20, and in about 80% it begins before the age of 30 years. In one fifth, other family members also have vitiligo. Males and females are equally affected.
Even though most people with vitiligo are in good general health, some of them face a greater risk of having autoimmune diseases such as diabetes, thyroid disease (in 20% of patients over 20 years with vitiligo), pernicious anaemia (B12 deficiency), Addison disease (adrenal gland disease), systemic lupus erythematosus, rheumatoid arthritis, psoriasis and alopecia areata (round patches of hair loss).
What are the clinical features of vitiligo?
Vitiligo can affect any part of the body. Common sites are exposed areas (face—especially around eyes or mouth, neck, finger tips and toes), body folds (armpits, groin), nipples, navel, and genitalia. Vitiligo also favours sites of injury (cuts, scrapes, thermal burns and sunburn)—it is one of the disorders that exhibits the Koebner phenomenon. New-onset vitiligo also sometimes follows emotional stress.
Vitiligo may occasionally start as multiple halo naevi; pigmented moles that develop a peripheral depigmented zone then gradually fade and in time, disappear.
The hair may also go grey early on the scalp, eyebrows, eyelashes and body. White hair is called ‘leukotrichia’ or ‘poliosis’. The retina at the back of the eye may also be affected. However, the colour of the iris does not change.
Complete loss of pigment can affect a single patch of skin or it may affect multiple patches. Small patches or macules a few millimetres in size are sometimes described as confetti-like. The white patches gradually enlarge over weeks to months. They may be round, oval, linear or irregular in shape. The edge of the white patch is usually the colour of unaffected skin, but sometimes it is darker (hyperpigmented) or lighter (hypopigmented) than the unaffected skin. The term trichrome vitiligo is used to describe 3 shades of skin colour. Very rarely, there are 4 shades of pigment (white, pale brown, dark brown and normal skin), called quadrichrome vitiligo. Occasionally, each patch of vitiligo has an inflamed red border.
Extension of vitiligo tends to occur fairly rapidly for a few months, then it stabilises. Some spontaneous repigmentation may occur, generally arising from hair follicles as brown spots within a white patch. At some time in the future, the vitiligo begins to extend again. Cycles of pigment loss followed by periods of stability may continue indefinitely.
The severity of vitiligo differs with each individual. Light skinned people usually notice the pigment loss during the summer as the contrast between the affected skin and sun tanned skin becomes more distinct. People with dark skin may observe the onset of vitiligo any time. Pigment has occasionally been reported to have may be lost from the entire skin surface. There is no way to predict how much pigment an individual will lose or how fast it will be lost.
How is vitiligo classified?
Classifications have identified clinical, genetic, pathobiological, epidemiological, and molecular characteristics of vitiligo.
The Vitiligo European Taskforce came to a consensus about classification of vitiligo in 2007. They decided on 4 main categories with subtypes.
- Nonsegmental vitiligo: focal, mucosal, acrofacial, generalised and universal subtypes
- Segmental vitiligo: focal, mucosal, unisegmental, bi- or multisegmental subtypes
- Mixed vitiligo (nonsegmental combined with segmental vitiligo)
- Unclassified vitiligo (early disease): focal at onset, multifocal asymmetrical non-segmental, mucosal (one site) subtypes
Nonsegmental vitiligo tends to be bilateral and symmetrical in distribution. If expanding areas or new areas of depigmentation occur, it is considered unstable. Vitiligo may also regress (improve). Vitiligo is considered stable if there is no progression or regression over a period of one to two years.
Segmental vitiligo occurs as a single white patch in 90%, but in a few, a second or rarely a third segment is affected. Each segment affects one side of the body. It arises in a young person and after the first year, generally remains stable. It is an example of cutaneous mosaicism. The segments often follow Blaschko lines, but occasionally have dermatomal pattern (following nerve distribution), phylloid pattern (leaf-like) or checkerboard pattern. The border of the white patch can be irregular or less often, smooth. White hair is often noted within segmental vitiligo.
Mixed vitiligo is rare.
How is the severity of vitiligo assessed?
In most cases the severity of vitiligo is not formally assessed. However, clinical photographs may be taken to monitor the condition.
At least 2 scoring systems have been devised for vitiligo and are used in clinical trials.
- Vitiligo Area Scoring Index (VASI)
- Vitiligo European Task Force (VETF) system
VASI is based on the PASI scoring system for psoriasis. It measures the extent and degree of depigmentation in 6 sites: hands, upper extremities, trunk, lower extremities and feet, head/neck.
VETF is based on SCORAD scoring system for atopic dermatitis. The VETF assesses extent, staging and spreading/progression in 5 sites: head/neck, trunk, arms, legs and hands/feet. It grades from 0 (normal pigmentation) to 4 (complete hair whitening). Spreading is assessed using the following scores: 0 (stable disease), -1 (regressive disease) and +1 (progressive disease).
VETF includes a clinical assessment form to record the sex, age, duration of disease, age of onset, episodes of repigmentation, impact of vitiligo on quality of life, family history, additional medical conditions and the Fitzpatrick skin type of the patients.
What is the cause of vitiligo?
Vitiligo is due to loss or destruction of melanocytes, which are the cells that produce melanin, the pigment that determines the colour of skin, hair, and eyes. If melanocytes cannot form melanin or if their number decreases, skin colour becomes progressively lighter.
The exact cause of vitiligo is unknown. It is thought to be a systemic autoimmune disorder, associated with deregulated innate immune response, although this has been disputed for segmental vitiligo. There is a genetic susceptibility and vitiligo is a component of some rare syndromes. The gene encoding the melanocyte enzyme tyrosinase, TYR, is likely involved.
There are three theories on the cause of vitiligo:
- The pigment cells are injured by abnormally functioning nerve cells.
- There may be an autoimmune reaction against the pigment cells (the body may destroy its own tissue, which it perceives as foreign).
- Autotoxic theory – the pigment cells self-destruct.
Current investigations are evaluating the pattern of cytokines (messenger proteins) in affected skin and potential proteins within the melanocyte that are susceptible to attack. The role of the hair follicle in repigmentation is also being studied. Inactive melanocytes and melanocyte stem cells are found in the outer root sheath of hair follicles and can be activated by treatment to mature and migrate to the skin surface.
How is vitiligo diagnosed?
Vitiligo is normally a clinical diagnosis, and no tests are necessary to make the diagnosis. The white patches may be seen more easily under Wood lamp examination (black light).
Occasionally skin biopsy may be recommended, particularly in early or inflammatory vitiligo, when a lymphocytic infiltration may be observed. Melanocytes and epidermal pigment are absent in established vitiligo patches.
Blood tests to assess other potential autoimmune diseases or polyglandular syndromes may be arranged, such as thyroid function, B12 levels and autoantibody screen.
Clinical photographs are useful to document the extent of vitiligo for monitoring. Serial digital images may be arranged on follow-up. The extent of vitiligo may be scored according to the body surface area affected by depigmentation.
Precautions that should be taken by people with vitiligo
Minimise skin injury
Those prone to vitiligo should be careful to minimise skin injury, as it is common for healing to result in a new white patch at the site. The injury might be a cut, a graze, a scratch, or an area prone to rubbing. New depigmentation often develops with a linear shape.
Protect against sun exposure
The white skin needs careful sun protection because it can only burn on exposure to ultraviolet radiation (UVR); it cannot tan. The normal skin also needs protecting to prevent sunburn (which could cause spreading of the vitiligo), and to reduce the contrast in colour between the normal skin and the vitiligo.
- Wear sun protective clothing
- Stay out of the sun at peak periods (the middle of the day during the summer months, when ultraviolet index >3).
- Apply SPF 50+sunscreen daily to exposed skin and reapply every 2 hours when outdoors.
|Images supplied by Dr Shahbaz A. Janjua|
What treatment is available for vitiligo?
Treatment of vitiligo is currently unsatisfactory. Best results are obtained in vitiligo that is recent in onset and when it affects face and trunk without poliosis. Repigmentation occurs when melanocyte stem cells in the bulb at the base of the hair follicle are activated and migrate to the skin surface.
A trial of the following topical treatments may be recommended. They are most effective if applied to the affected areas for a few weeks in new white patches while an inflammatory process is present.
- Topical corticosteroids can be used on patches of vitiligo on trunk and limbs once daily for 15 days per month for up to 6 months. Potent steroids should be avoided on thin skinned areas of face (especially eyelids), neck, armpits and groin.
- Calcineurin inhibitors (pimecrolimus cream and tacrolimus ointment are recommended for vitiligo affecting eyelids, face, neck, armpits and groin.
Phototherapy refers to treatment with ultraviolet (UV) radiation. There are several options. Which is used depends on the patient's skin type, location and severity of vitiligo patches, and the availability of the equipment. Treatment is usually given twice weekly for a trial period of 3–4 months. If repigmentation is observed, treatment is continued until repigmentation is complete or for a maximum of 1–2 years. Topical therapy is usually continued. Treatment times are generally quite brief, depending on the specific treatment provided. The aim is to cause the treated skin to appear very slightly pink the following day. It is important to avoid burning (red, blistered, peeling, itchy or painful skin), as this could cause the vitiligo to get worse.
- Whole-body or hand-foot narrowband (311 nm) UVB phototherapy
- Targeted UVB phototherapy for small areas of vitiligo. Home treatment has been advocated.
- Excimer laser UVB (308 nm) for small areas of vitiligo
- Photochemotherapy (PUVA). This form of light treatment requires the patient to take a psoralen medicine orally, or apply a psoralen cream/paint to affected areas, prior to exposure to longwave ultraviolet light (UVA) for a few minutes. Hands and feet respond poorly, face and trunk do better. PUVA is usually prescribed twice weekly, and is continued for up to two years. PUVA is unsuitable for children or very fair skinned people.
- In some countries, psoralen is followed by sunlight exposure, but this is uncontrolled and overexposure to the sun can result in serious sunburn.
Phototherapy probably works in vitiligo by 2 mechanisms.
- Immune suppression—preventing destruction of the melanocytes
- Stimulation of cytokines (growth factors)
Repigmentation occurs from the melanocytes in the hair follicle or on the edge of the depigmented areas.
Minocycline is an anti-inflammatory antibiotic in the tetracycline family. It has been shown to stabilise vitiligo in some patients.
Oral steroids are sometimes used as "minipulses" for 3 to 6 months.
Afamelanotide, a potent analogue of naturally occurring αmelanocyte stimulating hormone given by subcutaneous implantation, is under investigation for the treatment of vitiligo alone and in combination with narrowband UVB phototherapy.
In the future, it is anticipated that monoclonal antibody biologic treatments will be developed to treat vitiligo. Oral tofacitinib, a Janus kinase inhibitor approved for the treatment of severe rheumatoid arthritis, was reported to result in significant repigmentation in a patient with generalised vitiligo after 5 months of treatment.
Surgical treatment of stable vitiligo
Some centres are treating stable and segmental vitiligo by surgery. Small sites can be treated using topical or local anaesthetic injections, but if large areas are treated, general anaesthetic is necessary.
In non-cultured melanocyte-keratinocyte cell transplantation, the donor epidermis is separated from the dermis. Epidermal pieces are made into a cell suspension. The suspension is applied to the wounded vitiligo site and covered with a dressing. This method can be used on any site and large areas can be treated. However pain and risk of infection becomes greater when areas more than 250 sq cm are treated.
In punch grafting, tiny punches of normal skin are taken from a pigmented donor site and placed into the vitiligo skin. Cosmetic results are not as good as with cell transplantation, because scarring and cobblestone appearance may occur.
Blister grafts result in good cosmetic results and there is no scarring of the donor site. Blisters are formed by suction or cryotherapy. Blister grafting is time consuming, and the grafts are tricky to handle. It is therefore only suitable for small lesions.
Split skin grafting is quick to do, as the grafts can be applied directly onto dermabraded recipient area. It can lead to good results on small solitary patches of vitiligo.
Some researchers have used the patient's own melanocytes grown in tissue culture. This technique is restricted to research centers, and is expensive. Good results are reported, especially if the vitiligo affects a small area.
Adverse reactions to surgical treatments of vitiligo may include pain, wound infection hyperpigmentation, and post-treatment hypopigmentation (sometimes in a ring around the treated site).
Unfortunately, even when treatment has resulted in improvement, vitiligo may recur in treated and untreated sites.
If a dark skinned person has vitiligo affecting a large part of the exposed areas, he or she may wish to undergo depigmentation therapy. A cream containing monobenzyl ether of hydroquinone, also called p-(benzyloxy)phenol, is applied to the skin. This can cause all the skin to lose its pigment. Its effect is usually permanent, but depigmentation may recur. It is not always effective.
Use of cosmetics
- Carefully applied cosmetics can effectively disguise the vitiligo (cosmetic camouflage). Dyes, stains and make-ups can be applied and with specialist help the results can be very satisfactory. Some concealers are water-resistant.
- Dihydroxyacetone-containing "tan without sun" products; take care not to apply to the normally tanned skin because this will also look darker.
- Micropigmentation or tattooing may be considered if the vitiligo is stable.
Psychosocial effects of vitiligo
Vitiligo results in reduced quality of life and psychological difficulties in many patients, especially in adolescents and in females. The psychosocial effects of vitiligo tend to be more severe in some countries, cultures and religions than in others. Family support, counselling and cognitive behavioural treatment can be of benefit.
- Guidelines for the management and diagnosis of vitiligo (DJ Gawkrodger, AD Ormerod, L Shaw, I Mauri-Sole, ME Whitton, MJ Watts, AV Anstey, J Ingham and K Young). BJD, Vol. 159, No. 5, November 2008 (p1051-1076)
- Ezzedine K, Lim HW, Suzuki T, Katayama I, Hamzavi I, Lan CC, Goh BK, Anbar T, Silva de Castro C, Lee AY, Parsad D, van Geel N, Le Poole IC, Oiso N, Benzekri L, Spritz R, Gauthier Y, Hann SK, Picardo M, Taieb A; Vitiligo Global Issue Consensus Conference Panelists. Revised classification/nomenclature of vitiligo and related issues: the Vitiligo Global Issues Consensus Conference. Pigment Cell Melanoma Res. 2012 May;25(3):E1-13. doi: 10.1111/j.1755-148X.2012.00997.x. PubMed PMID: 22417114; PubMed Central PMCID: PMC3511780.
- Taïeb A, Picardo M; VETF Members. The definition and assessment of vitiligo: a consensus report of the Vitiligo European Task Force. Pigment Cell Res. 2007 Feb;20(1):27-35.
- Siadat AH, Zeinali N, Iraji F, Abtahi-Naeini B, Nilforoushzadeh MA, Jamshidi K, Khosravani P. Narrow-Band Ultraviolet B versus Oral Minocycline in Treatment of Unstable Vitiligo: A Prospective Comparative Trial. Dermatol Res Pract. 2014;2014:240856. doi: 10.1155/2014/240856. Epub 2014 Aug 21. PubMed PMID: 25221600; PubMed Central PMCID: PMC4158186.
- Lim HW, Grimes PE, Agbai O, Hamzavi I, Henderson M, Haddican M, Linkner RV, Lebwohl M. Afamelanotide and narrowband UV-B phototherapy for the treatment of vitiligo: a randomized multicenter trial. JAMA Dermatol. 2015 Jan;151(1):42-50. doi: 10.1001/jamadermatol.2014.1875. PubMed PMID: 25230094.
- Ezzedine K, Eleftheriadou V, Whitton M, van Geel N. Vitiligo. Lancet. 2015 Jan 14. pii: S0140-6736(14)60763-7. doi: 10.1016/S0140-6736(14)60763-7. [Epub ahead of print] Review. PubMed PMID: 25596811.
- Craiglow BG, King BA. Tofacitinib Citrate for the Treatment of Vitiligo: A Pathogenesis-Directed Therapy. JAMA Dermatol. 2015 Jun 24. doi: 10.1001/jamadermatol.2015.1520. [Epub ahead of print] PubMed PMID: 26107994.
On DermNet NZ:
- Pigmentation disorders
- Skin and hair colour
- Depigmentation therapy for vitiligo
- Vitiligo surgery
- Vitiligo – Medscape Reference: Drugs & Diseases
- Guidelines for the Management of Vitiligo The European Dermatology Forum Consensus – Medscape Dermatology
- Camouflage for patients with vitiligo – Review article in Indian Journal of Dermatology, Venereology and Leprology, January 2012
- Vitiligo – Medline Plus
- Vitiligo US National Library of Medicine Genetics Home Reference
- National Vitiligo Foundation Inc.
- Vitiligo Association of Australia
- Association Francaise de vitiligo
- Dr Kahn's surgical method for repigmentation
- Vitiligo – British Association of Dermatologists
- Schweizerische Psoriasis und Vitiligo Gesellschaft
- Vitiligo Society UK
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