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Vitiligo

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12/29/2019 Vitiligo - Wikipedia

Vitiligo
Vitiligo is a long-term skin condition characterized by
Vitiligo
patches of the skin losing their pigment.[1] The patches
of skin affected become white and usually have sharp
margins.[1] The hair from the skin may also become
white.[1] The inside of the mouth and nose may also be
involved.[2] Typically both sides of the body are
affected.[1] Often the patches begin on areas of skin that
are exposed to the sun.[2] It is more noticeable in
people with dark skin.[2] Vitiligo may result in
psychological stress and those affected may be
stigmatized.[1]

The exact cause of vitiligo is unknown.[1] It is believed Non-segmental vitiligo of the hand
to be due to genetic susceptibility that is triggered by an
Pronunciation /ˌvɪtɪˈlaɪɡoʊ/
environmental factor such that an autoimmune disease
occurs.[1][2] This results in the destruction of skin Specialty Dermatology
pigment cells.[2] Risk factors include a family history of Symptoms Patches of white skin[1]
the condition or other autoimmune diseases, such as
Duration Long term[1]
hyperthyroidism, alopecia areata, and pernicious
anemia.[2] It is not contagious.[4] Vitiligo is classified Causes Unknown[1]
into two main types: segmental and non-segmental.[1] Risk factors Family history, other
Most cases are non-segmental, meaning they affect autoimmune diseases[2]
both sides; and in these cases, the affected area of the Diagnostic Tissue biopsy[2]
skin typically expands with time.[1] About 10% of cases method
are segmental, meaning they mostly involve one side of Treatment Sunscreen, makeup, topical
the body; and in these cases, the affected area of the
corticosteroids,
skin typically does not expand with time.[1] Diagnosis
phototherapy[1][2]
can be confirmed by tissue biopsy.[2]
Frequency 1% of people[3]
There is no known cure for vitiligo.[1] For those with
light skin, sunscreen and makeup are all that is typically recommended.[1] Other treatment options
may include steroid creams or phototherapy to darken the light patches.[2] Alternatively, efforts to
lighten the unaffected skin, such as with hydroquinone, may be tried.[2] Several surgical options are
available for those who do not improve with other measures.[2] A combination of treatments generally
has better outcomes.[3] Counselling to provide emotional support may be useful.[1]

Globally about 1% of people are affected by vitiligo.[3] In some populations it affects as many as 2–
3%.[5] Males and females are equally affected.[1] About half show the disorder before age 20 and most
develop it before age 40.[1] Vitiligo has been described since ancient history.[1]

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Contents
Signs and symptoms
Causes
Immune
Autoimmune associations
Diagnosis
Classification
Differential diagnosis
Treatment
Immune mediators
Phototherapy
Skin camouflage
De-pigmenting
History
Society and culture
Research
References
External links

Signs and symptoms


The only sign of vitiligo is the presence of pale patchy areas of
depigmented skin which tend to occur on the extremities.[6][7]
Some people may experience itching before a new patch occurs.[8]
The patches are initially small, but often grow and change
shape.[6][9] When skin lesions occur, they are most prominent on
the face, hands and wrists.[6][7] The loss of skin pigmentation is
particularly noticeable around body orifices, such as the mouth,
eyes, nostrils, genitalia and umbilicus.[6][7] Some lesions have
increased skin pigment around the edges.[10] Those affected by
vitiligo who are stigmatized for their condition may experience
depression and similar mood disorders.[11]

Causes
Although multiple hypotheses have been suggested as potential Vitiligo on white skin

triggers that cause vitiligo, studies strongly imply that changes in


the immune system are responsible for the condition.[1][12]
Vitiligo has been proposed to be a multifactorial disease with genetic susceptibility and environmental
factors both thought to play a role.[1]

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The TYR gene encodes the protein tyrosinase, which is not a


component of the immune system, but is an enzyme of the
melanocyte that catalyzes melanin biosynthesis, and a major
autoantigen in generalized vitiligo.[1] The National Institutes of
Health states that some believe that sunburns can cause or
exacerbate the condition, but that this idea is not well-supported
by good evidence.[13]

Immune Vitiligo on black skin

Variations in genes that are part of the immune system or part of


melanocytes have both been associated with vitiligo.[1] It is also
thought to be caused by the immune system attacking and destroying the melanocytes of the skin.[14]
A genome wide association study found approximately 36 independent susceptibility loci for
generalized vitiligo.[15]

Autoimmune associations
Vitiligo is sometimes associated with autoimmune and inflammatory diseases such as Hashimoto's
thyroiditis, scleroderma, rheumatoid arthritis, type 1 diabetes mellitus, psoriasis, Addison's disease,
pernicious anemia, alopecia areata, systemic lupus erythematosus, and celiac disease.[1][16]

Among the inflammatory products of NALP1 are caspase 1 and caspase 7, which activate the
inflammatory cytokine interleukin-1β. Interleukin-1β and interleukin-18 are expressed at high levels
in people with vitiligo.[17] In one of the mutations, the amino acid leucine in the NALP1 protein was
replaced by histidine (Leu155 → His). The original protein and sequence is highly conserved in
evolution, and is found in humans, chimpanzee, rhesus monkey, and the bush baby. Addison's disease
(typically an autoimmune destruction of the adrenal glands) may also be seen in individuals with
vitiligo.[18][19]

Diagnosis
An ultraviolet light can be used in the early phase of this disease
for identification and to determine the effectiveness of
treatment.[20] Using a Wood's light, skin will change colour
(fluoresce) when it is affected by certain bacteria, fungi, and
changes to pigmentation of the skin.[21]

Classification
Classification attempts to quantify vitiligo have been analyzed as
being somewhat inconsistent,[22] while recent consensus have
agreed to a system of segmental vitiligo (SV) and non-segmental
vitiligo (NSV). NSV is the most common type of vitiligo.[1] UV photograph of a hand with
vitiligo

Non-segmental

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In non-segmental vitiligo (NSV), there is usually some form of


symmetry in the location of the patches of depigmentation. New
patches also appear over time and can be generalized over large
portions of the body or localized to a particular area. Extreme
cases of vitiligo, to the extent that little pigmented skin remains,
are referred to as vitiligo universalis. NSV can come about at any
age (unlike segmental vitiligo, which is far more prevalent in
teenage years).[10]

Classes of non-segmental vitiligo include the following:

Generalized vitiligo: the most common pattern, wide and


randomly distributed areas of depigmentation[23]
Universal vitiligo: depigmentation encompasses most of the
body[23]
Focal vitiligo: one or a few scattered macules in one area,
most common in children[23]
Acrofacial vitiligo: fingers and periorificial areas[23]
Mucosal vitiligo: depigmentation of only the mucous
membranes[23]

Segmental UV photograph of a foot with vitiligo


Segmental vitiligo (SV) differs in appearance, cause, and
frequency of associated illnesses. Its treatment is different from
that of NSV. It tends to affect areas of skin that are associated
with dorsal roots from the spinal cord and is most often
unilateral.[1][24] It is much more stable/static in course and its
association with autoimmune diseases appears to be weaker than
that of generalized vitiligo.[24] SV does not improve with topical
therapies or UV light, however surgical treatments such as
cellular grafting can be effective.[10]

Eyelid vitiligo
Differential diagnosis
Chemical leukoderma is a similar condition due to multiple
exposures to chemicals.[25] Vitiligo however is a risk factor.[25] Triggers may include inflammatory
skin conditions, burns, intralesional steroid injections and abrasions.[26]

Other conditions with similar symptoms include the following:

Pityriasis alba
Tuberculoid leprosy
Postinflammatory hypopigmentation
Tinea versicolor[23]
Albinism
Piebaldism[23]
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Idiopathic guttate hypomelanosis[23]


Progressive macular hypomelanosis[23]
Primary adrenal insufficiency

Treatment
There is no cure for vitiligo but several treatment options are available.[1] The best evidence is for
applied steroids and the combination of ultraviolet light in combination with creams.[27] Due to the
higher risks of skin cancer, the United Kingdom's National Health Service suggests phototherapy be
used only if primary treatments are ineffective.[28] Lesions located on the hands, feet, and joints are
the most difficult to repigment; those on the face are easiest to return to the natural skin color as the
skin is thinner in nature.[1]

Immune mediators
Topical preparations of immune suppressing medications including glucocorticoids (such as 0.05%
clobetasol or 0.10% betamethasone) and calcineurin inhibitors (such as tacrolimus or pimecrolimus)
are considered to be first-line vitiligo treatments.[1]

Phototherapy
Phototherapy is considered a second-line treatment for vitiligo.[1] Exposing the skin to light from UVB
lamps is the most common treatment for vitiligo. The treatments can be done at home with an UVB
lamp or in a clinic. The exposure time is managed so that the skin does not suffer overexposure.
Treatment can take a few weeks if the spots are on the neck and face and if they existed not more than
3 years. If the spots are on the hands and legs and have been there for more than 3 years, it can take a
few months. Phototherapy sessions are done 2–3 times a week. Spots on a large area of the body may
require full body treatment in a clinic or hospital. UVB broadband and narrowband lamps can be
used,[29][30] but narrowband ultraviolet picked around 311 nm is the choice. It has been constitutively
reported that a combination of UVB phototherapy with other topical treatments improves re-
pigmentation. However, some people with vitiligo may not see any changes to skin or re-pigmentation
occurring. A serious potential side effect involves the risk of developing skin cancer, the same risk as
an overexposure to natural sunlight.

Ultraviolet light (UVA) treatments are normally carried out in a hospital clinic. Psoralen and
ultraviolet A light (PUVA) treatment involves taking a drug that increases the skin's sensitivity to
ultraviolet light, then exposing the skin to high doses of UVA light. Treatment is required twice a week
for 6–12 months or longer. Because of the high doses of UVA and psoralen, PUVA may cause side
effects such as sunburn-type reactions or skin freckling.[28]

Narrowband ultraviolet B (NBUVB) phototherapy lacks the side-effects caused by psoralens and is as
effective as PUVA.[1] As with PUVA, treatment is carried out twice weekly in a clinic or every day at
home, and there is no need to use psoralen.[28] Longer treatment is often recommended, and at least
6 months may be required for effects to phototherapy.[31] NBUVB phototherapy appears better than
PUVA therapy with the most effective response on the face and neck.[31]

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With respect to improved repigmentation: topical calcineurin inhibitors plus phototherapy are better
than phototherapy alone,[32] hydrocortisone plus laser light is better than laser light alone, gingko
biloba is better than placebo, and oral mini-pulse of prednisolone (OMP) plus NB-UVB is better than
OMP alone.[8]

Skin camouflage
In mild cases, vitiligo patches can be hidden with makeup or other cosmetic camouflage solutions. If
the affected person is pale-skinned, the patches can be made less visible by avoiding tanning of
unaffected skin.[23]

De-pigmenting
In cases of extensive vitiligo the option to de-pigment the unaffected skin with topical drugs like
monobenzone, mequinol, or hydroquinone may be considered to render the skin an even color. The
removal of all the skin pigment with monobenzone is permanent and vigorous. Sun-safety must be
adhered to for life to avoid severe sunburn and melanomas. Depigmentation takes about a year to
complete.[28]

History
Descriptions of a disease believed to be vitiligo date back to a passage in the medical text Ebers
Papyrus c. 1500 BCE in ancient Egypt. Mentions of whitening of the skin were also present circa 1400
BCE in sacred Indian texts such as Atharvaveda as well as Shinto prayers in East Asia c. 1200 BCE.
The Hebrew word "Tzaraath" from the Old Testament book of Leviticus[33] dating to 1280 BCE[34] (or
1312 BCE[35]) described a group of skin diseases associated with white spots, and a subsequent
translation to Greek led to continued conflation of those with vitiligo with leprosy and spiritual
uncleanliness.[33]

Medical sources in the ancient world such as Hippocrates often did not differentiate between vitiligo
and leprosy, often grouping these diseases together. In Arabic literature, the word "alabras" has been
associated with vitiligo, with this word found in the Quran. The name "vitiligo" was first used by the
Roman physician Aulus Cornelius Celsus in his classic medical text De Medicina.[33]

The etymology of the term "vitiligo" is believed to be derived from "vitium", meaning "defect" or
"blemish".[33]

Society and culture


The change in appearance caused by vitiligo can affect a person's emotional and psychological well-
being and may create difficulty in becoming or remaining employed. People can experience emotional
stress, particularly if vitiligo develops on visible areas of the body, such as the face, hands, or arms.
Participating in a vitiligo support group may improve social coping skills and emotional resilience.[36]
Notable cases include that of Michael Jackson.[37]

Research
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Afamelanotide is in phase II and III clinical trials for vitiligo and other skin diseases.[38]

A medication for rheumatoid arthritis, tofacitinib, has been tested for the treatment of vitiligo.[39]

In October 1992, a scientific report was published of successfully transplanting melanocytes to vitiligo
affected areas, effectively re-pigmenting the region.[40] The procedure involved taking a thin layer of
pigmented skin from the person's gluteal region. Melanocytes were then separated out to a cellular
suspension that was expanded in culture. The area to be treated was then denuded with a
dermabrader and the melanocytes graft applied. Between 70 and 85 percent of people with vitiligo
experienced nearly complete repigmentation of their skin. The longevity of the repigmentation
differed from person to person.[41]

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External links
Vitiligo (https://curlie.org/Health/Conditions_and_Diseases/Skin Classification ICD-10: L80 (htt D
_Disorders/Vitiligo/) at Curlie p://apps.who.int/cla
Questions and Answers about Vitiligo (http://niams.nih.gov/Heal ssifications/icd10/br
th_Info/Vitiligo/default.asp) – US National Institute of Arthritis
owse/2016/en#/L8
and Musculoskeletal and Skin Diseases
0) · ICD-9-CM:
709.01 (http://www.i
cd9data.com/getIC
D9Code.ashx?icd9
=709.01) · OMIM:
193200 (https://omi
m.org/entry/19320
0) · MeSH:
D014820 (https://w
ww.nlm.nih.gov/cgi/
mesh/2015/MB_cg
i?field=uid&term=D
014820) ·
DiseasesDB:
13965 (http://www.d
iseasesdatabase.co
m/ddb13965.htm)
External MedlinePlus:
resources 000831 (https://ww
w.nlm.nih.gov/medli
neplus/ency/article/
000831.htm) ·

https://en.wikipedia.org/wiki/Vitiligo 10/11
12/29/2019 Vitiligo - Wikipedia

eMedicine:
derm/453 (https://e
medicine.medscap
e.com/derm/453-ov
erview) · Patient
UK: Vitiligo (https://
patient.info/doctor/vi
tiligo-pro)

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