Vitiligo is a common disease that affects about 1% of the population. Vitiligo is characterised by patches of depigmentation of the skin. It affects all races, although it is more visible in dark-skinned individuals.

The exact cause of vitiligo is unknown. Melanocytes are absent in vitiliginous areas; however, the cause of melanocyte destruction is not understood. Autoimmune and neurogenic mechanisms have been postulated.

At present, there is most evidence for an autoimmune process mediated by T-cells.

Vitiligo has been classified into focal, segmental and generalised types, which are differentiated by extent of involvement and course.

Generalised vitiligo is the most common type and is characterised by multiple areas of depigmentation, often occurring in a symmetrical distribution.

The course of vitiligo is unpredictable, and the extent varies from an isolated patch to complete depigmentation of the entire body.

Onset may occur at any age. Commonly affected regions include the eyes, mouth, digits, elbows, knees and trunk. Hair, mucous membranes and genitalia may also be involved. New patches may develop at sites of trauma (Koebner phenomenon).

Vitiligo is a disfiguring disease with an unpredictable course. The impact on patients’ quality of life can be significant.

Figure 1. Generalised diffuse vitiligo is relatively
unknown and is characterised by widespread
depigmentation.

DIFFERENTIAL DIAGNOSIS
There are numerous other causes of leukoderma that need to be excluded.

Piebaldism
Piebaldism is a rare autosomal dominant disorder of melanocyte development.

Clinically there may be a white forelock of hair, as well as symmetrical areas of hypopigmentation and depigmentation on the skin that are present from birth.

Pityriasis versicolor
This is distinguished by the presence of fine scales. It is caused by superficial overgrowth of Pityrosporum ovale.

It occurs mainly on the trunk, and looks pale on tanned skin types. It responds to topical antifungal therapy.

Post-inflammatory leukoderma
This is temporary and may occur following multiple inflammatory conditions, including eczema and psoriasis.

The hypopigmentation is caused by focally decreased melanin, but melanocytes are normal.

Idiopathic guttate hypomelanosis
This is a common benign condition characterised by multiple hypopigmented macules on sun-exposed areas of the arms and legs that onsets in adulthood.

Leprosy
Leprosy could be suspected in patients from endemic areas. Tuberculoid leprosy presents with well-defined hypopigmented anaesthetic macules.

Figure 2. Depigmentation in vitiligo often
occurs in a symmetrical distribution.

INVESTIGATIONS
Investigations are rarely necessary to make the diagnosis of vitiligo.

Other causes of leukoderma can generally be differentiated by history and examination. If there is any doubt, a specialist opinion is preferable.

Investigations are advised to exclude associated autoimmune diseases, particularly thyroid disease, diabetes and pernicious anaemia.

TREATMENT
The most important aspect of treatment in vitiligo is a realistic but positive attitude.

Although the impact of vitiligo is largely cosmetic and treatment is difficult, a dismissive or nihilistic approach is not helpful.

The principles of management of vitiligo are sun protection, cosmetic camouflage, repigmentation and depigmentation.

Sun protection is important to protect vitiliginous skin from sunburn, especially if active repigmentation is not pursued.

Cosmetic camouflage includes makeup, fake tan and camouflage tattooing. There are a number of commercially available products designed for this application.

Camouflage is particularly useful for visible areas, such as the face or hands.

The ideal treatment for vitiligo in most patients’ minds is repigmentation; that is, the restoration of normal skin colour.

There are medical and surgical repigmentation strategies available.

Medical repigmentation is the first-line active treatment modality, and includes phototherapy, topical corticosteroids and topical calcineurin inhibitors.

These treatments can be quite effective, but long-term compliance is needed, and response is unpredictable.

Surgical repigmentation is indicated in patients refractory to medical management with stable disease. Numerous techniques involving traditional skin grafts and cellular suspensions have been used.

As this involves invasive treatment, patients need to be highly motivated and well informed.

Depigmentation is indicated in patients with extensive disease who wish to attain a uniform skin colour.

Depigmentation is achieved using the monobenzyl ether of hydroquinone. As this form of depigmentation is permanent, patients need to be clearly informed.

As a highly visible disease, vitiligo can be very distressing to patients and lead to social stigmatisation.

Emotional support, combined with practical coping strategies, is an important part of management.

By Dr Linda Martin, MBBS (UNSW) Hons 1, Research Fellow, Department of Dermatology, St George Hospital, Sydney, NSW; Associate Professor Dedee Murrell, FAAD, Head of Dermatology, St George Hospital; Dr Richard Wittal, FACD, and Dr John Le Guay, FACD, both from the Vitiligo Clinic, Skin and Cancer Foundation, Darlinghurst, Sydney, NSW.

REFERENCES
Njoo MD, Spuls PI, Bos JD. Westerhof W. Bossuyt PM. Nonsurgical repigmentation therapies in vitiligo. Meta-analysis of the literature. Archives of Dermatology 1998;134(12):1532-40.
Njoo MD, Westerhof W, Bos JD, Bossuyt PM. A systematic review of autologous transplantation methods in vitiligo. Archives of Dermatology 1998;134(12):1543-49.
Whitton ME, Ashcroft DM, Barrett C W, Gonzalez U. Interventions for vitiligo. The Cochrane Database of Systematic Reviews 2006, Issue 1. Art. No.: CD003263. DOI: 10.1002/14651858.CD003263.

A depigmenting rash.A 21-year-old woman presents with a 12-month history of widespread depigmentation.

It initially began on her right hand, and she is depressed about her skin condition.

Clinically, she has asymptomatic white areas with well-defined edges. The hairs are white within these areas.

There is no cure for vitiligo and its cosmetic
disfigurement can have profound psychological effects.

PROVISIONAL DIAGNOSIS
Vitiligo is a common acquired loss of pigmentation of the skin, affecting 1%-2% of the population.

Destruction of melanocytes occurs and, although the precise cause is unknown, genetic factors (10% have a family history), autoimmune factors, trauma to the skin, and anxiety/stress can be associated.

The most frequently involved sites are the face (around the eyes and mouth), hands, axillae, umbilicus, nipples, inguinal and anogenital regions. It can also develop at sites of injury (Koebner phenomenon).

Lesions can be localised or generalised. Ocular disease can be associated. Vitiligo can remain localised and stable indefinitely, or it may progress slowly or rapidly. Postulated factors precipitating progression include emotional distress, physical illness, severe sunburn and pregnancy.

The cosmetic disfigurement can have profound psychological effects. It is therefore important to offer treatment to these individuals.

While most vitiligo patients are healthy, endocrinopathies occur in some patients. These can include thyroid dysfunction, diabetes mellitus, pernicious anaemia and Addison’s disease.

DIFFERENTIAL DIAGNOSIS

• Pityriasis versicolor
• Pityriasis alba
• Post-inflammatory hypopigmentation
• Idiopathic guttate hypomelanosis
• Lichen sclerosus
• Leprosy
• Hypopigmented mycosis fungoides
• Piebaldism
• Naevus depigmentosus.

INVESTIGATIONS
Diagnosis of vitiligo is based on clinical examination. Wood’s lamp examination confirms the depigmentation.

Autoimmune screen – thyroid antibodies, gastric parietal cell and intrinsic factor antibodies.

TREATMENT
There are four options.

1. No treatment

A good option if the patient is very fair. Strict sun protection is important.

2. Camouflage

Use of makeup and topical dyes, including self-tanning lotions. Micro-tattooing can be useful for small stable areas of vitiligo.

3. Active repigmentation

a) Medical: topical cortico­steroids, light therapy with photosensitising psoralen drugs applied topically or given systemically in conjunction with sunlight exposure or UV phototherapy (PUVA), narrowband UVB phototherapy and other topical agents – calcipotriol, pimecrolimus and tacrolimus.

b) Surgical: should only be considered when medical therapies fail. These procedures should only be performed on patients with stable, non-progressive vitiligo, ideally localised or segmental. They can be used in conjunction with medical therapies.

They involve transfer of a patient’s own melanocytes from unaffected skin into vitiligo-affected skin (autologous melanocyte transplantation).

Techniques include mini-grafting using punch grafts, non-cultured epidermal suspension, cultured epidermal suspension and suction blister grafting.

c) Lasers: e.g. the 308 nm xenon chloride excimer laser.

4. Depigmentation

Treatment with monobenzyl ether of hydroquinone can be used in patients with extensive (>80%) vitiligo. This process may take six months to two years. Strict sun protection is required after depigmentation. Pigment-removing lasers can also be used.

There is no cure for vitiligo. The treatment is prolonged and progress is slow. Patients require motivation, encouragement and empathy, and must be reassured that repigmentation may be achieved.

Some patients need help with the emotional and psychological aspects and referral to a psychologist or psychiatrist should be considered. Vitiligo support groups can also offer support.

This patient was given a course of narrowband UVB phototherapy, and she is developing follicular repigmentation. She is also receiving counselling. Her self-esteem is improving and she is more positive about her disease.

Dr Richard Wittal is a dermatologist who runs a tertiary referral vitiligo clinic at the Skin and Cancer Foundation (Darlinghurst, NSW). He is also in private practice in Beecroft, NSW.

Dr Stephen Shumack is the Honorary Secretary of the Australasian College of Dermatologists.

THE presence of a white halo around a naevus is not an uncommon finding in young children, but when you see multiple halo naevi develop suddenly in an adult, be aware this can be the first sign of developing vitiligo.

In vitiligo, areas of pigment loss will occur in a symmetrical fashion, often beginning in the groin, axillae or the dorsa of the hands.

Multiple halo naevi are caused by an autoimmune attack on the naevus cells, which can spill over into the surrounding melanocytes and also melanocytes elsewhere. The involved naevi will be completely removed by this process and the skin involved can slowly repigment normally.

For further discussion of this case, visit www.skinconsult.com.au.