Elsevier

The Lancet

Volume 386, Issue 9988, 4–10 July 2015, Pages 74-84
The Lancet

Seminar
Vitiligo

https://doi.org/10.1016/S0140-6736(14)60763-7Get rights and content

Summary

Vitiligo, an acquired pigmentary disorder of unknown origin, is the most frequent cause of depigmentation worldwide, with an estimated prevalence of 1%. The disorder can be psychologically devastating and stigmatising, especially in dark skinned individuals. Vitiligo is clinically characterised by the development of white macules due to the loss of functioning melanocytes in the skin or hair, or both. Two forms of the disease are well recognised: segmental and non-segmental vitiligo (the commonest form). To distinguish between these two forms is of prime importance because therapeutic options and prognosis are quite different. The importance of early treatment and understanding of the profound psychosocial effect of vitiligo will be emphasised throughout this Seminar.

Introduction

Vitiligo is an acquired chronic depigmenting disorder of the skin resulting from selective destruction of melanocytes. Celsus1 was the first to use the term vitiligo in his Latin medical classic De Medicina during the second century BCE.1, 2 The name is believed to derive from the Latin vitium, meaning defect or blemish,3 rather than vitellus, meaning calf.4 Typical vitiligo lesions can be defined as milky white, non-scaly macules with distinct margins. According to a recent international consensus conference,5 vitiligo can be classified into two major forms—namely, non-segmental vitiligo, also known as vitiligo, and segmental vitiligo. Non-segmental vitiligo, the commonest form of this unpredictable disease, is characterised by symmetrical and bilateral white patches. Different clinical subtypes have been described, including generalised, acrofacial, and universalis types, all with a bilateral distribution. Segmental vitiligo is less common than non-segmental vitiligo and usually has a unilateral distribution. Overall, progressive patchy loss of pigmentation from skin, overlying hair, and sometimes mucosa remains the basis of diagnosis of vitiligo.

Section snippets

Epidemiology

Vitiligo is the most common depigmenting disorder. The largest epidemiological study6 was done in 1977 on the island of Bornholm in Denmark, where vitiligo was reported to affect 0·38% of the population. The prevalence of vitiligo is often referred to as 0·5–1% of the world's population,5 although the exact prevalence is difficult to estimate, with rates as high as 8·8% in India.7 This high value could be due to the inclusion of cases with chemically induced depigmentation,8 or because these

Classification

Segmental vitiligo lesions are characterised by their unilateral and segmental or band-shaped distribution, (figure 1) early involvement of the follicular melanocyte reservoir, early age of onset, and rapid stabilisation,22 whereas non-segmental vitiligo lesions are typically bilaterally distributed in an acrofacial pattern, or scattered symmetrically over the entire body, evolving over time. Non-segmental vitiligo can initially have an acrofacial distribution, but can later progress to the

Pathophysiology

Histological examination and immunohistochemical studies with a large panel of antibodies generally show an absence of melanocytes in lesional skin, although sometimes an occasional melanocyte can be seen.28 However, the presence of a lymphocytic infiltrate has been described when biopsy specimens are taken from perilesional skin of actively spreading or inflammatory vitiligo (figure 3), in which there is a raised erythematous border. Various theories have been suggested for the cause of

Management

Before management is discussed with the patient, the extent of the disease should be assessed with natural and Wood's lamp examination. The Vitiligo European Task Force23 has developed an assessment form summarising the results of the personal and family history of the patient and clinical examination items. Skin phototype and disease duration, extent, and activity are important elements that will help to guide therapeutic management. Additionally, the patient's psychological profile and way of

Effects on people

So far, there is no cure for vitiligo. Current treatment results vary between individuals and are often unsatisfactory. Best results are generally reported for the face, whereas acral lesions respond poorly. Moreover, treatment is more efficient in recently developed lesions compared with older lesions, which argues for early therapeutic intervention.93 Patients with a fair complexion should be advised, after discussion, that no treatment can be offered because of an expected poor response and

Future directions

Whitton and colleagues80 pointed out that there is no consensus about the classification and definition of the disorder or about methods of assessment and outcome measures used in trials, and noted heterogeneity of interventions used to treat vitiligo.106, 107 These issues have been recognised by international experts worldwide as a priority for research. Efforts have been made to resolve these issues by the creation of consensus,5 identification and definition of priorities for research,108

Search strategy and selection criteria

We searched PubMed with the terms “vitiligo”, “autoimmunity”, and “leucoderma”. Our search covered articles published in English between Jan 1, 2000, and Jan 31, 2014. We identified additional reports that were judged the most relevant and recently published from the reference lists of selected articles. Some important older publications are cited either directly or indirectly through review articles.

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