Guidelines for the Management of Vitiligo

The European Dermatology Forum Consensus

A. Taieb; A. Alomar; M. Böhm; M.L. Dell'Anna; A. De Pase; V. Eleftheriadou; K. Ezzedine; Y. Gauthier; D.J. Gawkrodger; T. Jouary; G. Leone; S. Moretti; L. Nieuweboer-Krobotova; M.J. Olsson; D. Parsad; T. Passeron; A. Tanew; W. van der Veen; N. van Geel; M. Whitton; A. Wolkerstorfer; M. Picardo

Disclosures

The British Journal of Dermatology. 2013;168(1):5-19. 

In This Article

Abstract and Introduction

Abstract

The aetiopathogenic mechanisms of vitiligo are still poorly understood, and this has held back progress in diagnosis and treatment. Up until now, treatment guidelines have existed at national levels, but no common European viewpoint has emerged. This guideline for the treatment of segmental and nonsegmental vitiligo has been developed by the members of the Vitiligo European Task Force and other colleagues. It summarizes evidence-based and expert-based recommendations (S1 level).

Introduction

Vitiligo is an acquired depigmenting disorder affecting 0·5% of the world population, without sex or racial differences. It affects all age groups.[1,2] According to the consensus definition given to generalized/vulgaris or nonsegmental vitiligo (NSV) by the Vitiligo European Task Force (VETF)[1]'vitiligo vulgaris/NSV is an acquired chronic pigmentation disorder characterized by white patches, often symmetrical, which usually increase in size with time, corresponding to a substantial loss of functioning epidermal and sometimes hair follicle melanocytes'; however, this is not specific enough. It needs to be completed by a list of disorders (the acquired generalized hypomelanoses) which may clinically overlap with NSV, but which are clearly attributable to known aetiological factors. In cases of uncertain diagnosis, additional noninvasive and invasive procedures may be needed (Table 1).

Segmental vitiligo (SV) is defined descriptively as for NSV except for a unilateral distribution (asymmetric vitiligo) that may totally or partially match a cutaneous segment (e.g. dermatomal-like). Some specific features of SV are rapid onset and involvement of the hair follicle pigmentary system. One unique segment is involved in most patients (Table 2).

Concerning therapy and NSV topographic subtypes, acral lesions show the worst response rate.[3] Distinction between SV and NSV may affect prognosis in terms of resistance to repigmentation. Other forms of vitiligo (such as mucosal or eyelid vitiligo) may necessitate specific approaches not detailed in depth in this guideline.

In terms of therapy, the immune-mediated inflammatory phase of vitiligo needs to be better defined in order to develop specific approaches targeted to this important phase of the disease pathogenesis. This phase is mostly silent and only revealed when skin biopsies are taken at the margins of progressing lesions. Stable vitiligo needs a treatment to regenerate the melanocytes from hair follicle or interfollicular precursors.

In the assessment steps it is important to consider age, pre-existing diseases, in particular autoimmune disorders, previous medications, and objective and subjective parameters (Table 3).[4–6] As the care often extends over a long period of time, patients are frequently frustrated by the failure of previous treatments. Psychological stress is common. The treatment plan should be discussed with the patient to obtain a high level of compliance. It must be remembered that some therapies are not licensed for vitiligo and can only be prescribed 'off-label'.[6]

If the patient's history or routine laboratory parameters suggest additional autoimmune disorders, further investigations and specialist advice (e.g. in the case of autoimmune polyglandular syndrome) are strongly suggested (Table 1).

This guideline for the treatment of SV and NSV vitiligo has been developed by the members of the VETF and other colleagues. It summarizes evidence-based and expert-based recommendations (S1 level).

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