Vitiligo overview

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Vitiligo from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

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History and Symptoms

Physical Examination

Laboratory Findings

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

## Overview

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Vitiligo (IPA Template:IPA) or leukoderma is a chronic skin condition that causes loss of pigment, resulting in irregular pale patches of skin. The cause of vitiligo is complex, may be multifactorial, and is not fully understood. There is some evidence suggesting it is caused by a combination of auto-immune, genetic, and environmental factors. Vitiligo is the most common depigmenting disease with a worldwide incidence of 1%.

## Historical Perspective

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Human pigmentation diseases, such as vitiligo, have been described for over 3,000 years by different cultures around the world. Some famous texts, as the Eber Papyrus, Atharva Veda or even the Bible, provide a description of "white spotted-diseases" that could include cases of vitiligo. Celsus was the first to use the word "vitiligo" in the first century A.C. and Moriz Kaposi was one of the first to describe the histopathologic features of vitiligo.

## Classification

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Vitiligo can be classified in two clinical subtypes. One is segmented vitiligo, which affects only 1 segment of the body (face, arm or leg); and non-segmented vitiligo, involving more than 1 segment, such as both knees or both hands.

Characteristics of the Subtypes of Vitiligo | Non-segmental Vitiligo | Segmental Vitiligo

|
|

Definition  | Involves both sides of the body, most common type  | Involves 1 segment of the body (face, arm or leg)
Age  | Later onset is more common than in childhood  | More common in childhood
Onset and progression  | Progressive, with acute episodes  | Rapid and stabilizes
Hair involvement  | In later stages  | Early
Association with other autoimmune conditions  | Yes  | No
Common location  | Areas prone to pressure or friction  | Face
Response to autologous grafting  | Good response  | Relapses
Table adapted from N Engl J Med 2009;360:160-9[1], EDF Vitiligo Guidelines, A. Taieb et al. [2] and J Am Acad Dermatol Mazereeuw-Hautier et al [3]

## Pathophysiology

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Vitiligo is caused by a loss of skin melanocytes. Although the exact mechanism is not known, at least in some cases, an autoimmune process may play a role. [4][5] The fact that vitiligo is more prevalent in patients with certain autoimmune disorders, such as Addison's disease, hyperthyroidism, alopecia areata and pernicious anemia supports this hypothesis,[6][7][8] but it should also be recognized that the majority of patients with vitiligo do not have any autoimmune disorder.

## Causes

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Vitiligo is caused by a loss of skin melanocytes. Although the exact mechanism is not known, at least in some cases, an autoimmune process may play a role. [4][5]

## Differentiating Vitiligo from other Diseases

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There are numerous conditions that cause hypopigmentation from which vitiligo must be differentiated, and the most common are pityriasis alba, postinflammatory hypopigmentation, tinea versicolor, halo nevus, tuberous sclerosis and albinism.

## Epidemiology and Demographics

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Vitiligo is the most common human pigmentation disorder, with a prevalence of 1,000/100,000 (1%) of the population. Males and females are equally affected. Half of patients are diagnosed before the age of 20.

## Risk Factors

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Autoimmune diseases and a family history of vitiligo are considered risk factors for developing this condition. A patient that has a relative with vitiligo has an 18 fold increased risk of developing the disease and having an earlier onset of the disease.

## Natural History, Complications and Prognosis

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The natural history of vitiligo is variable. Depigmentation may be stable or progressive and can cause even a total body depigmentation or remit spontaneously, although spontaneous remission is uncommon.

## Diagnosis

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### History and Symptoms

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Vitiligo is an asymptomatic disease that commonly presents during the second decade of life, with a gradual depigmentation over time.[9][10]

### Physical Examination

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Vitiligo is a chronic skin condition that causes loss of pigment, resulting in irregular pale patches of skin that may be distributed according to different patterns.

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

* Vitiligo. Adapted from Dermatology Atlas[11]

### Laboratory Findings

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There are no laboratory abnormalities in vitiligo disease. Consideration should be given to ordering laboratory studies to exclude the presence of other associated conditions such as pernicious anemia, Addison's disease and thyroid disease.

### Other Diagnostic Studies

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Although not performed routinely, since the diagnosis of vitiligo is often reached by a thorough history assessment and physical examination, a biopsy of the lesion may show microscopical changes undergoing on the hypopigmented region.

## Treatment

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### Medical Therapy

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Potent topical corticosteroids (mometasone) and topical calcineurin inhibitors are first-line therapy to achieve repigmentation of vitiligo lesions. Phototherapy has been proven effective for the treatment of generalized vitiligo. Combined treatment with both topical calcineurin inhibitors plus phototherapy have proven more effective in achieving repigmentation in a shorter period of time than single treatments.

## Support organizations

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Support groups and organizations are available to help individuals learn more about vitiligo, understand treatment options, and find support from other individuals with vitiligo.

* Vitiligo Support International is the largest vitiligo organization in the world. The nonprofit organization provides free access to online message boards, chat rooms, frequently asked questions, information and articles, as well as a patient-referred doctor search. The group advocates on behalf of patients, conducts patient conferences and has local support groups.

* The National Vitiligo Foundation (NVF) is a 501(c)(3) nonprofit organization that provides access to online resources, physician listings, frequently asked questions (etc); funds research through grants and sponsors local support groups and workshop style conferences.

* The American Vitiligo Research Foundation Inc. (AVRF) is a non-profit, tax-exempt charity that aims to increase public awareness about vitiligo and to help those affected by vitiligo, focusing specifically on children and their families. It supports finding a cure through alternatives to animal testing.

* VITFriends, LLC is a support group in the North East USA. Formed in 2004, VITFriends is still growing and touching the world. They are a web-community offering words of encouragement and sharing hope to individuals dealing with Vitiligo. The goal is to raise public awareness about vitiligo.[2]

## References

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1. ↑ Taïeb, Alain; Picardo, Mauro (2009). "Vitiligo". New England Journal of Medicine. 360 (2): 160–169. doi:10.1056/NEJMcp0804388. ISSN 0028-4793.
2. ↑ Taieb, A.; Alomar, A.; Böhm, M.; Dell’Anna, M.L.; De Pase, A.; Eleftheriadou, V.; Ezzedine, K.; Gauthier, Y.; Gawkrodger, D.J.; Jouary, T.; Leone, G.; Moretti, S.; Nieuweboer-Krobotova, L.; Olsson, M.J.; Parsad, D.; Passeron, T.; Tanew, A.; van der Veen, W.; van Geel, N.; Whitton, M.; Wolkerstorfer, A.; Picardo, M. (2013). "Guidelines for the management of vitiligo: the European Dermatology Forum consensus". British Journal of Dermatology. 168 (1): 5–19. doi:10.1111/j.1365-2133.2012.11197.x. ISSN 0007-0963.
3. ↑ Juliette Mazereeuw-Hautier, Sophie Bezio, Emmanuel Mahe, Christine Bodemer, Catherine Eschard, Valerie Viseux, Christine Labreze, Patrice Plantin, Sebastien Barbarot, Pierre Vabres, Ludovic Martin, Carle Paul & Jean-Philippe Lacour (2010). "Segmental and nonsegmental childhood vitiligo has distinct clinical characteristics: a prospective observational study". Journal of the American Academy of Dermatology. 62 (6): 945–949. doi:10.1016/j.jaad.2009.06.081. PMID 20466172. Unknown parameter `|month=` ignored (help)CS1 maint: Multiple names: authors list (link)
4. ↑ 4.0 4.1 Gauthier Y, Cario Andre M, Taïeb A (2003). "A critical appraisal of vitiligo etiologic theories. Is melanocyte loss a melanocytorrhagy?". Pigment Cell Res. 16 (4): 322–32. PMID 12859615.CS1 maint: Multiple names: authors list (link)
5. ↑ 5.0 5.1 Dell'anna ML, Picardo M (2006). "A review and a new hypothesis for non-immunological pathogenetic mechanisms in vitiligo". Pigment Cell Res. 19 (5): 406–11. doi:10.1111/j.1600-0749.2006.00333.x. PMID 16965269.
6. ↑ Shahla Babaee Nejad, Hamideh Herizchi Qadim, Leila Nazeman, Roohollah Fadaii & Mohamad Goldust (2013). "Frequency of autoimmune diseases in those suffering from vitiligo in comparison with normal population". Pakistan journal of biological sciences: PJBS. 16 (12): 570–574. PMID 24494526. Unknown parameter `|month=` ignored (help)CS1 maint: Multiple names: authors list (link)
7. ↑ Daniel Holthausen Nunes & Ligia Maria Hademann Esser (2011). "Vitiligo epidemiological profile and the association with thyroid disease". Anais brasileiros de dermatologia. 86 (2): 241–248. PMID 21603806. Unknown parameter `|month=` ignored (help)
8. ↑ Kirsty J. MacLean & Michael J. Tidman (2013). "Alopecia areata: more than skin deep". The Practitioner. 257 (1764): 29–32. PMID 24383154. Unknown parameter `|month=` ignored (help)
9. ↑ Soutor, Carol (2013). Clinical dermatology. New York: McGraw-Hill Education/Lange Medical Books. ISBN 978-0-07-177296-9.
10. ↑ Taïeb, Alain; Picardo, Mauro (2007). "The definition and assessment of vitiligo: a consensus report of the Vitiligo European Task Force". Pigment Cell Research. 20 (1): 27–35. doi:10.1111/j.1600-0749.2006.00355.x. ISSN 0893-5785.
11. ↑ 11.00 11.01 11.02 11.03 11.04 11.05 11.06 11.07 11.08 11.09 11.10 11.11 11.12 11.13 11.14 11.15 11.16 11.17 11.18 11.19 11.20 11.21 11.22 11.23 11.24 11.25 11.26 11.27 11.28 11.29 11.30 11.31 11.32 11.33 11.34 11.35 11.36 11.37 11.38 11.39 "Dermatology Atlas".

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