Key Points

  • Vitiligo is a common disorder of depigmentation.
  • Vitiligo may occur in patterns: localized, segmental, generalized, or a variant with acral predilection and periorificial involvement.
  • Vitiligo may be associated with autoimmune disease.
  • The duration of vitiligo is lifelong and its disease course may be difficult to predict.
  • The mainstay of treatment includes topical corticosteroid and calcineurin therapies and narrow-band ultraviolet phototherapy; combined therapy may be most effective.

Introduction

Vitiligo is a common disorder of depigmentation that affects approximately 0.5 to 1% of the population and affects males and females equally. It can be distinguished from disorders of hypopigmentation by its porcelain-white appearance, its predilection for axial, acral, and periorificial areas, and the absence of any other skin changes. Vitiligo presents in several different patterns, including a generalized form, localized or segmental forms (affecting one relatively localized area of the body), or it may present in an acral form accompanied with periorificial and genital involvement. In general, the clinical pattern in an individual patient is consistent throughout the course of disease. It may also appear in areas of trauma (koebnerization phenomenon). Important associations include autoimmune disease.

The natural history of vitiligo is difficult to predict. Patients may experience intermittent episodes of flaring and remission (repigmentation).

Most treatments for vitiligo only partially repigment affected areas, and 75% repigmentation is considered to be an excellent response to therapy. The facial area responds best to all forms of treatment, and the acral extremities are refractory to treatments. Phototherapy can be effective, but many treatment sessions may be required (more than 50 sessions) for a substantial benefit. Topical corticosteroids and/or calcineurin inhibitors may also be used in localized areas of depigmentation associated with vitiligo with variable success. Relapse is common. Addressing the emotional impact of vitiligo is paramount.

Initial Evaluation

Vitiligo

Differential diagnosis

Tinea versicolor

Pityriasis alba

Discoid lupus

Treatment

Localized disease, <5% body surface area

First-line therapy: Topical treatment options include a mid- to superpotent topical steroid (such as betamethasone or clobetasol) applied twice daily to the skin. Evidence supports that this is the most effective monotherapy. Important alternatives include tacrolimus 0.1% or calcipotriene 0.005% twice daily topical treatment, however, these are slightly inferior in efficacy. Application of topical calcineurin inhibitors (such as tacrolimus or pimecrolimus) under occlusion may enhance efficacy.

  • Avoid friction or skin trauma, as these can exacerbate preexisting lesions or trigger new areas of depigmentation.

Extensive disease, >5% body surface area)

First-line therapy: Narrowband ultraviolet light, specifically UVB (NBUVB) therapy three times weekly can be used in patients with extensive lesions or those refractory to topical treatments. It may be effective in approximately two-thirds of patients and works best for lesions on the face and trunk. This can be combined with topical corticosteroids or calcineurin inhibitors applied twice daily for enhanced effect.

  • A mid-potency topical steroid to superpotent topical steroid (such as betamethasone or clobetasol) applied twice daily is an effective alternative. Response rates are similar to tacrolimus, but atrophy commonly complicates such prolonged use of potent topical steroids.
  • Tacrolimus ointment 0.1% (or 0.03% for the face) applied to affected areas twice daily. Once daily application is significantly less effective.

Alternative steps

  • Systemic corticosteroids (such as prednisone) may be used to halt rapidly progressive disease. However, safety and efficacy of this intervention is not well documented.
  • Cosmetic make-up, such as Covermark or Dermablend, may help to camouflage areas of depigmentation in visible areas. Tanning sprays or lotions (such as dihydroacetone) may also be highly effective in minimizing the visual impact of disease.
  • In patients with >50-70% body surface area pigment loss without clinical improvement or disease progression despite years of treatment, consideration may be given to generalized depigmentation with monobenzyl ether of hydroquinone. The topical treatment course may be prolonged, and a dermatologist’s supervision is strongly recommended. Successfully depigmented patients are usually very satisfied.

Pitfalls

  • If topical steroid therapy is undertaken, patients must be monitored for the development of cutaneous atrophy.
  • Patients with vitiligo are often highly motivated, and can be desperate, for any improvement. Prior to undertaking any form of therapy, but especially phototherapy, the patient must be carefully counseled regarding expected outcomes. The patient and physician must agree ahead of time when the treatment will be stopped if there is no response.

When to refer to a dermatologist

  • If the diagnosis of vitiligo is not clear.
  • For skin biopsy to confirm the diagnosis of vitiligo.
  • For management of extensive or rapidly progressive disease.
  • For initiation and/or monitoring of phototherapy.
  • For consideration of depigmentation (bleaching) therapy.

Clinical Cases

Case 1

  • 5-year-old boy
  • Four month history of an enlarging depigmented patch on right lower cutaneous lip and chin
  • Otherwise healthy
  • Family history notable for mother with autoimmune thyroiditis

Initial evaluation

  • Wood’s lamp examination confirms that the affected area is depigmented
  • No evidence of inflammation
  • A clinical diagnosis of vitiligo is rendered
  • No systemic evaluation is required at this time, as he is otherwise healthy
  • Recommend topical calcineurin ointment, tacrolimus 0.03%, applied twice daily
  • Follow-up in three months

Three-month follow-up

  • Approximately 50% repigmentation noted (with accentuation of repigmentation at follicular areas)
  • Recommend continued application of calcineurin ointment
  • Follow-up in six months

Six-month follow-up

  • Approximately 75% repigmentation noted
  • Recommend ongoing application of calcineurin ointment to depigmented areas only

Case 2

  • 24-year-old woman
  • Six-month history of progressive depigmentation: face, trunk, arms, legs
  • Approximately 30% body surface involvement
  • Otherwise healthy
  • No family history of vitiligo or autoimmune disease

Initial evaluation

  • Wood’s lamp examination confirms depigmented patches
  • A clinical diagnosis of vitiligo is rendered
  • Recommend NBUVB phototherapy combined with topical corticosteroid ointment applied twice daily
  • Follow-up in three months

Three-month follow-up evaluation

  • Subtle repigmentation, with areas of follicular accentuation of newly pigmented areas
  • Recommend continued NBUVB phototherapy and topical steroid treatment

Six-month follow-up evaluation

  • Approximately 60% repigmentation noted
  • Continue phototherapy and topical steroid treatment with follow-up evaluation at three-month intervals

References

Alikhan A, Felsten LM, Daly M, Petronic-Rosic V (2011) Vitiligo: a comprehensive overview, Part I, JAAD, 65(3): 473-491.

Felsten LM, Alikhan Al, Petronic-Rosic V (2011) Vitiligo: a comprehensive overview, Part II, JAAD, 65(3): 493-514.

Taieb A, Picardo M (2009) Vitiligo, NEJM, 360: 160-169.

Whitten ME, Pinart M, Batchelor J, Lushey C, Leonardi-Bee J, Gonzalez U (2010) Interventions for vitiligo, Cochrane Database of Systematic Reviews, Issue 1.