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Vitiligo

Orpha number ORPHA3435
Synonym(s) -
Prevalence -
Inheritance Multigenic/multifactorial
Age of onset Childhood
Adolescent
Adult
ICD-10
  • L80
ICD-O -
OMIM
UMLS
  • C0042900
MeSH
  • D014820
MedDRA
  • 10047642
SNOMED CT
  • 56727007

Summary

Vitiligo is an acquired and progressive skin disorder characterized by circumscribed hypomelanosis of the skin and hair.

It affects 0.5-1% of the world's population, irrespective of gender and race. Thus it is not a rare disease.

Onset of vitiligo is commonly in childhood or young adulthood but it can develop at any age. Patients present with one or several amelanotic macules surrounded by a normal or hyperpigmented border. White patches may have, in rare cases, a red inflammatory border. First lesions usually appear on the hands, forearms, feet and face. The lesional skin is more sensitive to sunburns. Three main forms of vitiligo can be distinguished: segmental vitiligo (SV), characterized by localized lesions and overall stable course, non-segmental vitiligo (NSV) which is associated with Koebner phenomenon (scars depigmentation), markers of autoimmune disease and lifelong evolution, and mixed vitiligo, presenting characteristics of SV with secondary onset of bilateral vitiligo patches.

Vitiligo is due to a disappearance of functioning melanocytes at the dermal-epidermal junction and subsequent loss of melanin in the epidermis. Etiology is unknown and the several pathogenetic hypotheses do not account for the entire spectrum of the disease. Vitiligo is a multifactorial disorder with genetic and non-genetic factors of which immune-mediated inflammatory component, as is suggested by skin biopsies taken at the edge of progressing lesions. Solid association with the disease have been demonstrated for the FBXO11 (2p16.3) and NLRP1 (17p13) genes, and other genetic factor may influence the phenotype, various susceptibility genes being identified (HLA, PTPN22, CTLA4 and TYR).

Diagnosis is based on clinical examination and can be facilitated in fair skinned individuals with the use of a Wood's lamp.

Differential diagnosis includes, among other diseases, Vogt-Koyanagi-Harada disease, mycosis fungoides, piebaldism, tuberous sclerosis, Waardenburg syndrome, lichen sclerosus (see these terms), Alezzandrini syndrome, nevus depigmentosus, postinflammatory depigmentation, and chemical leukoderma.

Although no full therapeutic solution for vitiligo is available, many options may lead to acceptable results in most patients, according to disease stage (stable/progressive) including medical and surgical interventions. Major treatments consist of topical corticosteroids, topical calcineurin inhibitors and narrowband ultraviolet. Surgical treatment, consisting of autologous transplantation methods, is generally recommended for stable/focal vitiligo, after medical therapy has failed. Finally, for patients with extensive areas of vitiligo, depigmentation of the residual melanin can be considered. Natural photoadaptation of vitiligo lesions occurs and limits the systematic use of sunblockers, except for limiting contrast with surrounding pigmented skin and during hours of intense insolation.

Rare cases of spontaneous repigmentation have been observed and use cosmetic products for camouflage of vitiligo lesions significantly improve patients' quality of life.

Expert reviewer(s)

  • Pr Alain TAIEB

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Detailed information

Summary information
Review article
  • EN (2009)Patient Inform
Article for general public
  • FR (2006,pdf)
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