image of silk fabric and dry skin


W. Westerhof, MD, PhD


Netherlands Institute for Pigmentary Disorders, Department of Dermatology, University of Amsterdam, The Netherlands

ABSTRACT


Vitiligo is an acquired skin disorder caused by the disappearance of pigment cells from the epidermis, and results in well defined white patches that are often symmetrically distributed. The lack of melanin pigment makes the lesional skin more sensitive to sunburn. Vitiligo can be cosmetically disfiguring and is a stigmatizing condition, leading to serious psychological problems in daily life. It occurs worldwide in about 1% of the population, mostly between the ages of 10-30 years, and as often in males as in females. The cause is unknown, but might involve genetic factors, autoimmunity, toxic metabolites, and/or a higher vulnerability of melanocytes. Some new treatments for this condition include corticosteroid + UVA treatment, UVB narrow wave band (311nm) irradiation, and transplantation of autologous pigment cells. In widespread vitiligo, residual pigment can be removed by depigmentation agents. Sunscreens, camouflage products and good guidance may help the patient to better cope with this disease.

Key Words:
vitiligo, UVA, UVB, PUVA

There is concern that many vitiligo patients are not offered treatment by dermatologists and that there is no reimbursement by insurance companies or government health services, because vitiligo is considered only a cosmetic problem. Yet quality of life studies indicate that the psychosocial problems are of the same magnitude as those found in patients with psoriasis1. When treatment is given, there seems to be a great variety in treatment choices and regimens. However, no guidelines are available based on controlled trials and evidence based medicine2. Based on a meta-analysis of the literature concerning medical and surgical treatments of vitiligo3,4 some treatment recommendations are presented here.

Past and Current Therapies for Vitiligo

The best studied and therefore the most commonly used medical treatments are corticosteroids (topical, intra-lesional and oral), oral and topical psoralens + ultraviolet A (PUVA), phenylalanine + UVA, oral and topical khellin + UVA, fluticasone propionate + UVA, narrow band UVB, and broad band UVB3,4,5,6. Because no drugs are involved, narrow band UVB at 311nm irradiation has an advantage over classical PUVA, and is therefore suitable for women during their childbearing years and for children. There is:

  • no photocontact allergenicity
  • no hyperkeratosis after long-term irradiation
  • less itching and xerosis
  • less contrast between lesional and pigmented skin
  • shorter treatment sessions and no preparation with psoralen
  • less phototoxicity
  • faster repigmentation
  • less radiation and cumulative dosages6

Methods of autologous transplantation of melanocytes have been developed to repigment lesions that are stable, as well as those that are refractory to medical therapies. Examples that are listed in order of technical feasibility and cost are: minigrafting, split skin thickness grafting, grafting of epidermal blisters, grafting of melanocyte containing cultured epidermal sheets, and grafts of pure melanocyte cultures. It is important to stress that a test grafting (two biopsies of 2 mm diameter) should be performed before the actual grafting method is chosen7,8.

Treatment typeActivity

Corticosteroid cream + UVA

6 months 1x/day cream, 2x/wk UVA

UVB 311nm

1 year, 2x/wk, 4 follow up visits

PUVA

1 year, 2x/wk, 4 follow up visits

UVB broadspectrum

1 year, 2x/wk, 4 follow up visits

Minigrafting

100cm2 (2 sessions)

Split skin grafting

100cm2 (1 session)

Depigmentation laser

1 year, 6 sessions

Depigmentation cream

12 months, 1x/d application

Table 1: Current treatment options recommended for vitiligo.

AgeClinical Type of VitiligoFirst-Choice Therapy*Alternative Therapies*

Children < 12 yrs

All

Class 3 corticosteroids (and UVA); course of treatment is 6-9 mo (patients aged <6 yrs, no UVA)

Local UVB (311nm); course of treatment is 6-12 mo Topical PUVA; course of treatment is 6-12 mo

Adults

Localized (≤ 2% depigmentation)

Class 3 corticosteroids (and UVA); Course of treatment is 6-9 mo

Local UVB (311nm); course of treatment is 6-12 mo Topical PUVA; course of treaetment is 6-12 mo

Generalized (> 2%)

UVB (311nm); course of treatment is 6-24 mo

Oral PUVA; course of treatment is 6-24 mo

Segmental or stable

Autologous transplantation, (until 100% repigmentation)

Class 3 corticosteroids (and UVA); course of treatment is 6-9 mo UVB (311nm); course of treatment is 6-24 mo

Lip-Tip

Autologous transplantation, (until 100% repigmentation)

Micropigmentation, (until 100% repigmentation)

Therapy-resistant and/or generalized (>80% depigmentation)

Depigmentation with bleaching creme
and/or laser (until 100% depigmentation)

None

Table 2: Treatment scheme for vitiligo11
* The course or treatment is expressed as a range from minimum to maximum.

Mechanism of Action

The pigment cells are thought to have an inherent defect, making them vulnerable to mechanical, thermal or chemical trauma. Initiated by above mechanisms or by other unknown factors, it is also possible that autoimmune destruction of pigment cells takes place. The result is the partial or complete loss of pigment cells from the epidermis, hair shafts and roots. The treatments are aimed at stopping the pigment cell destruction (steroids, ultra violet irradiation) and stimulating pigment cell division and outgrowth (ultraviolet irradiation, grafting of pigment cells). In universal vitiligo, remaining normal pigment cells are destroyed by toxic phenolic substances such as topical 4-methoxyphenol9 or monobenzyl ether of hydroquinone12, or laser10.

TreatmentAdverse effects

UV radiation modalities

Sunburn, solar elastosis, carcinogenesis?

Corticosteroid

Systemic: Adrenal suppression

Topical: atrophy, striae, acne

Minigrafting

Infection, cobblestoning, pitted scars (donorsite)

Split skin grafting

Split skin grafting

Monobenzone

Irritation

Table 3: Adverse effects to the various treatment schemes used to treat vitiligo3,4,9.

Conclusion

Vitiligo is still not acknowledged by most dermatologists as a true skin disease, and active treatment is usually not prescribed. In cases where treatment is undertaken there is no consistency in treatment choices and regimens. A systematic review of the literature shows that the treatments listed here are the most effective and safest vitiligo therapies (evidence-based medicine). The patient’s quality of life should be the main measure of the treatment outcome.

References

  1. Porter J, Hill-Beuf A, Lerner A, Nordlund J. Response to cosmetic disfigurement: patients with vitiligo. Cutis 39(6):493-4 (1987 Jun).
  2. Evidence-based Medicine. A new approach to teaching the practice of medicine. Evidence-Based Medicine Working Group. JAMA 268(17):2420-5 (1992 Nov).
  3. Njoo MD, Spuls PI, Bos JD, Westerhof W, Bossuyt PM. Nonsurgical repigmentation therapies in vitiligo. Meta-analysis of the literature. Arch Dermatol 134(12):1532-40 (1998 Dec).
  4. Njoo MD, Westerhof W, Bos JD, Bossuyt PM. A systematic review of autologous transplantation methods in vitiligo. Arch Dermatol 134(12):1543-9 (1998 Dec).
  5. Westerhof W, Nieuweboer-Krobotova L, Mulder PG, Glazenburg EJ. Left-right comparison study of the combination of fluticasone propionate and UV-A vs. either fluticasone propionate or UV-A alone for the long-term treatment of vitiligo. Arch Dermatol 135(9):1061-6 (1999 Sep).
  6. Westerhof W, Nieuweboer-Krobotova L. Treatment of vitiligo with UV-B radiation vs topical psoralen plus UV-A. Arch Dermatol 133(12):1525-8 (1997 Dec).
  7. Falabella R, Arrunategui A, Barona MI, Alzate A. The minigrafting test for vitiligo: detection of stable lesions for melanocyte transplantation. J Am Acad Dermatol 32(2 Pt 1):228-32 (1995 Feb).
  8. Westerhof W, Boersma B. The minigrafting test for vitiligo: detection of stable lesions for melanocyte transplantation. J Am Acad Dermatol 33(6):1061-2 (1995 Dec).
  9. Njoo MD, Bos JD, Westerhof W. Depigmentation therapy in vitiligo universalis using topical 4-methoxyphenol and the Q-switched ruby laser. J Am Acad Dermatol. In press 2000.
  10. Thissen M, Westerhof W. Laser treatment for further depigmentation in vitiligo. Int J Dermatol 36(5):386-8 (1997 May).
  11. Njoo MD, Westerhof W, Bos JD, Bossuyt PM. The development of guidelines for the treatment of vitiligo. Arch Dermatol. In press 1999.
  12. Ellison JM, Griffiths WAD. Vitiligo treated with monobenzyl ether of hydroquinone. Retinoids & Lipid-Soluble Vitamins in Clinical Practice 16(1):10-2 (2000).