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Vitiligo is a disorder that causes the skin to lose its color. Specific causes are unknown but studies suggest a link to immune system changes.


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. Some people may experience itching before a new patch occurs. The patches are initially small, but often grow and change shape. When skin
lesion A lesion is any damage or abnormal change in the tissue of an organism, usually caused by disease or trauma. ''Lesion'' is derived from the Latin "injury". Lesions may occur in plants as well as animals. Types There is no designated classif ...
s occur, they are most prominent on the face, hands and wrists. The loss of skin pigmentation is particularly noticeable around body orifices, such as the mouth, eyes, nostrils, genitalia and umbilicus. Some lesions have increased skin pigment around the edges. Those affected by vitiligo who are stigmatized for their condition may experience depression and similar
mood disorder A mood disorder, also known as an affective disorder, is any of a group of conditions of mental and behavioral disorder where a disturbance in the person's mood is the main underlying feature. The classification is in the '' Diagnostic and St ...
s. File:Vitiligo03.jpg, Vitiligo on lighter skin File:Vitiligo1.JPG, Non-segmental vitiligo on dark skin, hand facing up File:Eyelid vitiligo 06.jpg, Non-segmental vitiligo of the eyelids


Causes

Although multiple hypotheses have been suggested as potential triggers that cause vitiligo, studies strongly imply that changes in the
immune system The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splinte ...
are responsible for the condition. Vitiligo has been proposed to be a multifactorial disease with genetic susceptibility and environmental factors both thought to play a role. The
National Institutes of Health The National Institutes of Health, commonly referred to as NIH (with each letter pronounced individually), is the primary agency of the United States government responsible for biomedical and public health research. It was founded in the lat ...
states that some believe that
sunburn Sunburn is a form of radiation burn that affects living tissue, such as skin, that results from an overexposure to ultraviolet (UV) radiation, usually from the Sun. Common symptoms in humans and animals include: red or reddish skin that is h ...
s can cause or exacerbate the condition, but that this idea is not well-supported by good evidence.


Immune

Melanin Melanin (; from el, μέλας, melas, black, dark) is a broad term for a group of natural pigments found in most organisms. Eumelanin is produced through a multistage chemical process known as melanogenesis, where the oxidation of the amino ...
is the pigment that gives skin its color; it is produced by skin cells called
melanocytes Melanocytes are melanin-producing neural crest-derived cells located in the bottom layer (the stratum basale) of the skin's epidermis, the middle layer of the eye (the uvea), the inner ear, vaginal epithelium, meninges, bones, and hear ...
. Variations in genes that are part of the immune system or part of melanocytes have both been associated with vitiligo. It is also thought to be caused by the immune system attacking and destroying the melanocytes of the skin. A genome wide association study found approximately 36 independent susceptibility loci for generalized vitiligo. The TYR gene encodes the protein
tyrosinase Tyrosinase is an oxidase that is the rate-limiting enzyme for controlling the production of melanin. The enzyme is mainly involved in two distinct reactions of melanin synthesis otherwise known as the Raper Mason pathway. Firstly, the hydroxy ...
, 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 immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an "autoimmune disease". ...
in generalized vitiligo.


Autoimmune associations

Vitiligo is sometimes associated with autoimmune and inflammatory diseases such as Hashimoto's thyroiditis, scleroderma,
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are inv ...
,
type 1 diabetes mellitus Type 1 diabetes (T1D), formerly known as juvenile diabetes, is an autoimmune disease that originates when cells that make insulin (beta cells) are destroyed by the immune system. Insulin is a hormone required for the cells to use blood sugar f ...
,
psoriasis Psoriasis is a long-lasting, noncontagious autoimmune disease characterized by raised areas of abnormal skin. These areas are red, pink, or purple, dry, itchy, and scaly. Psoriasis varies in severity from small, localized patches to comple ...
,
Addison's disease Addison's disease, also known as primary adrenal insufficiency, is a rare long-term endocrine disorder characterized by inadequate production of the steroid hormones cortisol and aldosterone by the two outer layers of the cells of the adrena ...
,
pernicious anemia Pernicious anemia is a type of vitamin B12 deficiency anemia, a disease in which not enough red blood cells are produced due to the malabsorption of vitamin B12. Malabsorption in pernicious anemia results from the lack or loss of intrinsic ...
, alopecia areata,
systemic lupus erythematosus Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Commo ...
, and celiac disease. Among the inflammatory products of NALP1 are
caspase 1 Caspase-1/Interleukin-1 converting enzyme (ICE) is an evolutionarily conserved enzyme that proteolytically cleaves other proteins, such as the precursors of the inflammatory cytokines interleukin 1β and interleukin 18 as well as the pyroptos ...
and caspase 7, which activate the inflammatory
cytokine Cytokines are a broad and loose category of small proteins (~5–25 kDa) important in cell signaling. Cytokines are peptides and cannot cross the lipid bilayer of cells to enter the cytoplasm. Cytokines have been shown to be involved in au ...
interleukin-1β. Interleukin-1β and interleukin-18 are expressed at high levels in people with vitiligo. In one of the mutations, the
amino acid Amino acids are organic compounds that contain both amino and carboxylic acid functional groups. Although hundreds of amino acids exist in nature, by far the most important are the alpha-amino acids, which comprise proteins. Only 22 alpha ...
leucine in the NALP1 protein was replaced by
histidine Histidine (symbol His or H) is an essential amino acid that is used in the biosynthesis of proteins. It contains an α-amino group (which is in the protonated –NH3+ form under biological conditions), a carboxylic acid group (which is in the ...
(Leu155 → His). The original protein and sequence is highly conserved in evolution, and is found in humans,
chimpanzee The chimpanzee (''Pan troglodytes''), also known as simply the chimp, is a species of great ape native to the forest and savannah of tropical Africa. It has four confirmed subspecies and a fifth proposed subspecies. When its close relative t ...
,
rhesus monkey The rhesus macaque (''Macaca mulatta''), colloquially rhesus monkey, is a species of Old World monkey. There are between six and nine recognised subspecies that are split between two groups, the Chinese-derived and the Indian-derived. Generally ...
, and the bush baby.
Addison's disease Addison's disease, also known as primary adrenal insufficiency, is a rare long-term endocrine disorder characterized by inadequate production of the steroid hormones cortisol and aldosterone by the two outer layers of the cells of the adrena ...
(typically an autoimmune destruction of the
adrenal gland The adrenal glands (also known as suprarenal glands) are endocrine glands that produce a variety of hormones including adrenaline and the steroids aldosterone and cortisol. They are found above the kidneys. Each gland has an outer cortex ...
s) may also be seen in individuals with vitiligo.


Diagnosis

An
ultraviolet light Ultraviolet (UV) is a form of electromagnetic radiation with wavelength from 10 nm (with a corresponding frequency around 30  PHz) to 400 nm (750  THz), shorter than that of visible light, but longer than X-rays. UV radiatio ...
can be used in the early phase of this disease for identification and to determine the effectiveness of treatment. Using a
Wood's light A blacklight, also called a UV-A light, Wood's lamp, or ultraviolet light, is a lamp that emits long-wave (UV-A) ultraviolet light and very little visible light. One type of lamp has a violet filter material, either on the bulb or in a separa ...
, skin will change colour ( fluoresce) when it is affected by certain bacteria, fungi, and changes to pigmentation of the skin.


Classification

Classification attempts to quantify vitiligo have been analyzed as being somewhat inconsistent, 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.


Non-segmental

In non-segmental vitiligo (NSV), there is usually some form of
symmetry Symmetry (from grc, συμμετρία "agreement in dimensions, due proportion, arrangement") in everyday language refers to a sense of harmonious and beautiful proportion and balance. In mathematics, "symmetry" has a more precise definiti ...
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). Classes of non-segmental vitiligo include the following: * Generalized vitiligo: the most common pattern, wide and randomly distributed areas of depigmentation * Universal vitiligo: depigmentation encompasses most of the body * Focal vitiligo: one or a few scattered macules in one area, most common in children * Acrofacial vitiligo: fingers and periorificial areas * Mucosal vitiligo: depigmentation of only the mucous membranes


Segmental

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 The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the sp ...
and is most often unilateral. It is much more stable/static in course and its association with autoimmune diseases appears to be weaker than that of generalized vitiligo. SV does not improve with topical therapies or UV light, however surgical treatments such as cellular grafting can be effective.


Differential diagnosis

Chemical leukoderma is a similar condition due to multiple exposures to chemicals. Vitiligo however is a risk factor. Triggers may include inflammatory skin conditions, burns, intralesional steroid injections and abrasions. Other conditions with similar symptoms include the following: * Albinism * Halo nevus * Idiopathic guttate hypomelanosis (white sunspots) * Piebaldism *
Pityriasis alba Pityriasis alba is a skin condition, a type of dermatitis, commonly seen in children and young adults as dry, fine-scaled, pale patches on the face. It is self-limiting and usually only requires use of moisturizer creams. The condition is so name ...
* Postinflammatory hypopigmentation * Primary adrenal insufficiency * Progressive macular hypomelanosis * Tinea versicolor *
Tuberculoid leprosy Tuberculoid leprosy is a form of leprosy characterized by solitary skin lesions that are asymmetrically distributed with few lesions and well demarcated edges. There is also early and marked nerve damage. It tends to heal spontaneously. Tuberculoid ...


Treatment

There is no cure for vitiligo but several treatment options are available. The best evidence is for applied
steroid A steroid is a biologically active organic compound with four rings arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes that alter membrane fluidity; and ...
s and the combination of
ultraviolet light Ultraviolet (UV) is a form of electromagnetic radiation with wavelength from 10 nm (with a corresponding frequency around 30  PHz) to 400 nm (750  THz), shorter than that of visible light, but longer than X-rays. UV radiatio ...
in combination with creams. Due to the higher risks of skin cancer, the United Kingdom's
National Health Service The National Health Service (NHS) is the umbrella term for the publicly funded healthcare systems of the United Kingdom (UK). Since 1948, they have been funded out of general taxation. There are three systems which are referred to using the " ...
suggests phototherapy be used only if primary treatments are ineffective. 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.


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. In July 2022, ruxolitinib cream (sold under the brand name Opzelura) was approved for medical use in the United States for the treatment of vitiligo.


Phototherapy

Phototherapy is considered a second-line treatment for vitiligo. 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, but narrowband ultraviolet peaked 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 Psoralen (also called psoralene) is the parent compound in a family of naturally occurring organic compounds known as the linear furanocoumarins. It is structurally related to coumarin by the addition of a fused furan ring, and may be considered ...
and ultraviolet A light (
PUVA PUVA (psoralen and UVA) is an ultraviolet light therapy treatment for skin diseases: eczema, psoriasis, graft-versus-host disease, vitiligo, mycosis fungoides, large plaque parapsoriasis, and cutaneous T-cell lymphoma, using the sensitizing e ...
) 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. Narrowband ultraviolet B (NBUVB) phototherapy lacks the side-effects caused by psoralens and is as effective as PUVA. 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. Longer treatment is often recommended, and at least 6 months may be required for effects to phototherapy. NBUVB phototherapy appears better than PUVA therapy with the most effective response on the face and neck. With respect to improved repigmentation: topical calcineurin inhibitors plus phototherapy are better than phototherapy alone, hydrocortisone plus laser light is better than laser light alone, gingko biloba is better than
placebo A placebo ( ) is a substance or treatment which is designed to have no therapeutic value. Common placebos include inert tablets (like sugar pills), inert injections (like Saline (medicine), saline), sham surgery, and other procedures. In general ...
, and oral mini-pulse of
prednisolone Prednisolone is a steroid medication used to treat certain types of allergies, inflammatory conditions, autoimmune disorders, and cancers. Some of these conditions include adrenocortical insufficiency, high blood calcium, rheumatoid arth ...
(OMP) plus NB-UVB is better than OMP alone.


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.


De-pigmenting

In cases of extensive vitiligo the option to de-pigment the unaffected skin with topical drugs like monobenzone, mequinol, or
hydroquinone Hydroquinone, also known as benzene-1,4-diol or quinol, is an aromatic organic compound that is a type of phenol, a derivative of benzene, having the chemical formula C6H4(OH)2. It has two hydroxyl groups bonded to a benzene ring in a ''pa ...
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 Sunburn is a form of radiation burn that affects living tissue, such as skin, that results from an overexposure to ultraviolet (UV) radiation, usually from the Sun. Common symptoms in humans and animals include: red or reddish skin that is h ...
and melanomas. Depigmentation takes about a year to complete.


History

Descriptions of a disease believed to be vitiligo date back to a passage in the medical text Ebers Papyrus in ancient
Egypt Egypt ( ar, مصر , ), officially the Arab Republic of Egypt, is a List of transcontinental countries, transcontinental country spanning the North Africa, northeast corner of Africa and Western Asia, southwest corner of Asia via a land bridg ...
. Also, the
Hebrew Hebrew (; ; ) is a Northwest Semitic language of the Afroasiatic language family. Historically, it is one of the spoken languages of the Israelites and their longest-surviving descendants, the Jews and Samaritans. It was largely preserved ...
word "
Tzaraath ''Tzaraath'' (Hebrew צָרַעַת ''ṣāraʿaṯ''), variously transcribed into English and frequently mistranslated as leprosy, describes various ritually unclean disfigurative conditions of the skin, hair of the beard and head, clothing mad ...
" from the
Old Testament The Old Testament (often abbreviated OT) is the first division of the Christian biblical canon, which is based primarily upon the 24 books of the Hebrew Bible or Tanakh, a collection of ancient religious Hebrew writings by the Israelites. The ...
book of Leviticus dating to 1280 BC (or 1312 BCHistory Crash Course #36: Timeline: From Abraham to Destruction of the Temple
, by Rabbi Ken Spiro, Aish.com. Retrieved 2010-08-19.
) 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 Leprosy, also known as Hansen's disease (HD), is a long-term infection by the bacteria '' Mycobacterium leprae'' or '' Mycobacterium lepromatosis''. Infection can lead to damage of the nerves, respiratory tract, skin, and eyes. This nerve d ...
and spiritual uncleanliness. Medical sources in the ancient world such as
Hippocrates Hippocrates of Kos (; grc-gre, Ἱπποκράτης ὁ Κῷος, Hippokrátēs ho Kôios; ), also known as Hippocrates II, was a Greek physician of the classical period who is considered one of the most outstanding figures in the history o ...
often did not differentiate between vitiligo and leprosy, often grouping these diseases together. The name "vitiligo" was first used by the Roman physician Aulus Cornelius Celsus in his classic medical text ''
De Medicina ''De Medicina'' is a 1st-century medical treatise by Aulus Cornelius Celsus, a Roman encyclopedist and possibly (but not likely) a practicing physician. It is the only surviving section of a much larger encyclopedia; only small parts still survi ...
''. The etymology of the term "vitiligo" is believed to be derived from "vitium", meaning "defect" or "blemish".


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, 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. Notable cases include US pop singer
Michael Jackson Michael Joseph Jackson (August 29, 1958 – June 25, 2009) was an American singer, songwriter, dancer, and philanthropist. Dubbed the " King of Pop", he is regarded as one of the most significant cultural figures of the 20th century. Over ...
, Canadian fashion model Winnie Harlow, New Zealand singer-songwriter Kimbra, US actor David Dastmalchian and Argentine musician
Charly García Charly García (born Carlos Alberto García, October 23, 1951) is an Argentine singer-songwriter, musician and record producer. He formed and headlined two of the most popular bands in Argentina's rock history: Sui Generis in the 1970s and Se ...
. French actor Michaël Youn is also affected, as is former French Prime Minister Édouard Philippe, the TV host, model and former Miss Colombia 2007, Taliana Vargas


Research

, afamelanotide is in phase II and III clinical trials for vitiligo and other skin diseases. A medication for rheumatoid arthritis, tofacitinib, has been tested for the treatment of vitiligo. In October 1992, a scientific report was published of successfully transplanting
melanocyte Melanocytes are melanin-producing neural crest-derived cells located in the bottom layer (the stratum basale) of the skin's epidermis, the middle layer of the eye (the uvea), the inner ear, vaginal epithelium, meninges, bones, and hear ...
s to vitiligo-affected areas, effectively re-pigmenting the region. 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.


References


External links

*
Questions and Answers about Vitiligo
nbsp;– US National Institute of Arthritis and Musculoskeletal and Skin Diseases {{Authority control Autoimmune diseases Disturbances of human pigmentation Genetic disorders by system Wikipedia neurology articles ready to translate Wikipedia medicine articles ready to translate