| Vitiligo |
| Classification and external resources |
|
Non-segmental vitiligo of the hand.
|
| ICD-10 |
L80 |
| ICD-9 |
709.01 |
| OMIM |
193200 |
| DiseasesDB |
13965 |
| MedlinePlus |
000831 |
| eMedicine |
derm/453 |
| MeSH |
D014820 |
Vitiligo // is a condition that causes depigmentation of parts of the skin. It occurs when melanocytes, the cells responsible for skin pigmentation, die or are unable to function. The cause of vitiligo is unknown, but research suggests that it may arise from autoimmune, genetic, oxidative stress, neural, or viral causes.[1] The incidence worldwide is less than 1%,[2] with some populations averaging between 2-3% and as high as 16%.[3] There are two main types of vitiligo: idiopathic and chemical.[4]
Contents
- 1 Signs and symptoms
- 2 Suggested causes
- 2.1 Immune system studies
- 2.2 Inflammatory and Thyroid associations
- 3 Vitiligo in rabbinic literature
- 4 Classification
- 4.1 Non-segmental
- 4.2 Segmental
- 5 Differential diagnosis
- 6 Treatment
- 6.1 Phototherapy
- 6.2 Immune mediators
- 6.3 Skin camouflage
- 6.4 De-pigmenting
- 6.5 Transplanting melanocytes
- 7 Notable cases
- 8 See also
- 9 References
- 10 External links
Signs and symptoms
The most notable symptom of vitiligo is patchy areas of skin that have lost their pigment which tends to occur on the extremities.[5][6] Although patches are initially small, they often grow and change shape.[1][5] When skin lesions occur, they are most prominent on the face, hands and wrists.[5][6] The loss of skin pigmentation is particularly noticeable around body orifices, such as the mouth, eyes, nostrils, genitalia and umbilicus.[5][6] Some lesions have increased skin pigment around the edges.[7] Patients who are stigmatized for their condition may experience depression and similar mood disorders.[8] A black light (also referred to as a UVA light, Wood's lamp, or simply ultraviolet light) can be used in the early phase of this disease for identification and to determine effectiveness of treatment. Skin with vitiligo, when exposed to a black light, will glow yellow, green or blue. In contrast, healthy skin will have no reaction.
Suggested causes
Although multiple theories have been suggested as potential triggers that cause vitiligo to occur, medical studies implicating changes in the immune system have strongly advocated a correlation between the two, while a cure based on this study is being developed.
Immune system studies
Variations in genes that are part of the immune system or part of melanocytes have both been associated with vitiligo.[medical citation needed]
In one case, the gene TYR, which makes the melanocyte more susceptible to the immune system in vitiligo, also makes the melanocyte more susceptible to the immune system in the skin cancer malignant melanoma. Therefore, people with vitiligo caused by the TYR gene are less likely to have malignant melanoma.[medical citation needed]
A genomewide association study found 10 independent susceptibility loci for generalized vitiligo, responsible for 7.4% of the genetic risk. Some patients had vitiligo alone; others had generalized vitiligo with other autoimmune diseases. Most loci were associated with both forms. (The exception was PTPN22, which was only associated with generalized vitiligo.) In the major histocompatibility complex (MHC) region, which controls the immune system, major association signals were identified in the class I gene region (between HLA-A and HLA-HGC9) and class II gene region (between HLA-DRB1 and HLA-DQA1). Outside the MHC region, association signals were identified near RERE, PTPN22, LPP, IL2RA, GZMB, UBASH3A and C1QTNF6 genes, which are associated with other autoimmune diseases. TYR encodes 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. The major alleles of TYR are associated with vitiligo, and the minor alleles are associated with malignant melanoma. Vitiligo-associated 402R tyrosinase may be more efficiently presented to the immune system. Melanoma-associated 402Q may fail to be identified by the immune system.[9]
The transcriptional profile of melanocytes from vitiligo patients have been studied. Oligonucleotide microarrays containing approximately 16,000 unique genes were used to analyse mRNA expression in melanocytes from vitiligo patients and age-matched healthy controls. In total, 859 genes were identified as differentially expressed.[10]
Inflammatory and Thyroid associations
Vitiligo is sometimes associated with autoimmune and inflammatory diseases,[11] commonly thyroid overexpression and underexpression. A study comparing 656 people with and without vitiligo in 114 families found several mutations (single-nucleotide polymorphisms) in the NALP1 gene. The NALP1 gene, which is on chromosome 17 located at 17p13, is on a cascade that regulates inflammation and cell death, including myeloid and lymphoid cells, which are white cells that are part of the immune response. NALP1 is expressed at high levels in T cells and Langerhan cells, white blood cells that are involved in skin autoimmunity. Polymorphisms of CD4 were shown to be associated with the vitiligo and other autoimmune diseases like type I Diabetes Mellitus.[12]
Among the inflammatory products of NALP1 are caspase 1 and caspase 7, which activate the inflammatory cytokine interleukin-1β. Interleukin-1β is expressed at high levels in patients with vitiligo. Some compounds inhibit caspase and interleukin-1β, and so might be useful drugs for vitiligo and associated autoimmune diseases. 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 found in humans, chimpanzee, rhesus monkey, and bush baby, which means that it is an important protein and an alteration is likely to be harmful. Addison's disease (typically an autoimmune destruction of the adrenal glands) may cause vitiligo.[13][14]
Vitiligo in rabbinic literature
Main article: Tzaraath
Main article: rabbinic literature
According to some Rabbinic literature, vitiligo is one and the same with the ancient biblical Tzaraath. It has been suggested by those sources that factors involving the dietary and sexual habits of parents prior and during conception of the vitiligo patient may be a precursor to vitiligo development in later stages of the lifecycle of the offspring.[15]
Classification
Classification attempts to quantify vitiligo have been analyzed as being somewhat inconsistent,[16] while recent consensus have agreed to a system of segmental vitiligo (SV) and non-segmental vitiligo (NSV).
Non-segmental
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. Vitiligo where little pigmented skin remains is referred to as vitiligo universalis. NSV can come about at any age (unlike segmental vitiligo, which is far more prevalent in teenage years).[7]
Classes of non-segmental vitiligo include:
- Generalized Vitiligo: the most common pattern, wide and randomly distributed areas of depigmentation[17]
- Universal Vitiligo: depigmentation encompasses most of the body[17]
- Focal Vitiligo: one or a few scattered macules in one area, most common in children[17]
- Acrofacial Vitiligo: fingers and periorificial areas[17]
- Mucosal Vitiligo: depigmentation of only the mucous membranes[17]
Segmental
Segmental vitiligo (SV) differs in appearance, etiology and prevalence from 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 spine and is most often unilateral. It spreads much more rapidly than NSV and, without treatment, it is much more stable/ static in course and is not associated with auto-immune diseases. It is a very treatable condition that responds to topical treatment.[7]
Differential diagnosis
Conditions with similar symptoms include the following:
- Pityriasis alba
- Tuberculoid leprosy
- Postinflammatory hypopigmentation
- Tinea versicolor[17]
- Albinism
- Piebaldism[17]
- Idiopathic guttate hypomelanosis[17]
- Progressive macular hypomelanosis[17]
- Primary adrenal insufficiency
Treatment
There are a number of treatments for vitiligo with the best evidence for applied steroids and the combination of ultraviolet light in combination with creams.[18] Due to the higher risks of skin cancer, the NHS suggests phototherapy only be used if primary treatments are ineffective.[19]
Phototherapy
Exposing the skin to UVB light from UVB lamps is the most common treatment for vitiligo. The treatments can be done at home with a domestic UVB lamp or in a clinic. It is important to control the exposure time so that the skin does not burn from 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 more than 3 years, it can take a few months. In a clinic the treatments are done 2–3 times a week, and at home every day, which makes the home treatments more effective. Spots on a large area of the body may require full body treatment in a clinic or hospital. Both UVB broadband and UVB narrowband lamps can be used.[20][21] There is no treatment that totally repigments the skin. Adding a psoralen, a photosensitizer, or an immunomodulant[22] that increases the effect of the UV light can aid in partial repigmentation.
A 1997 report suggests that combining vitamin B12 and folic acid supplements with sun exposure caused repigmentation in 52% of cases.[23]
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.[19]
Narrowband ultraviolet B (UVB) phototherapy is now used more commonly than PUVA as it is less damaging to the skin. 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.[19]
Immune mediators
Tentative evidence supports a role for tacrolimus.[24] There is tentative short term evidence for pimecrolimus but long term data is missing.[25]
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 affected skin.[17]
De-pigmenting
Most vitiligo is idiopathic; however, in cases where it is triggered by skin bleaching or other substances, it is said to be chemical after being treated with bleaching agents. 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 colour. The removal of all the skin pigment with monobenzone is permanent and vigorous. Sun-safety must be adhered to for life to avoid severe sun burn and melanomas. Depigmentation takes about a year to complete.[19]
Transplanting melanocytes
In October 1992, a scientific report was published of successfully transplanting melanocytes to vitiligo affected areas, effectively repigmenting the region.[26] The procedure involved taking a thin layer of pigmented skin from the patient'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 patients experienced nearly complete repigmentation of their skin. The longevity of the repigmentation differed from person to person.[27]
Notable cases
Michael Jackson said that he was diagnosed with vitiligo universalis.
- Michael Jackson announced publicly in a 90-minute interview with Oprah Winfrey in February 1993 that he had vitiligo. This was confirmed by the autopsy report following his death in 2009.[28]
- Jon Hamm reported developing stress-induced vitiligo while working on the series Mad Men.[29]
- UFC fighter Scott Jorgensen suffers from a particularly aggressive form of the disease and has received the unofficial nickname 'Spotty' as a result of the appearance it causes his skin to have. In 2012 Jorgensen claimed to have allowed the disease to "pretty much take over" and as a result give his skin a uniform colour.[30]
- The Canadian model Chantelle Brown-Young has a prominent form of vitiligo. Her participation in the America's Next Top Model contest led to her being called a "vitiligo spokesmodel".[31]
- American rapper Krizz Kaliko is diagnosed with the condition, and titled his debut album Vitiligo.
- Lee Thomas, broadcast reporter, speaker, and best-selling author of Turning White: A Memoir of Change, which covered his experience of living with vitiligo
See also
- Albinism, uniform absence of melanin
- Alphos, a non-contagious leprosy formerly referred to as "vitiligo"
- Amelanism, lack of pigmentation
- Birth mark
- Chimera (genetics)
- Coloboma
- Erythrism, excessive red pigmentation
- Heterochromia iridum, another condition characterized by pigment variations
- Leucism, reduced pigmentation
- Leukoderma
- List of cutaneous conditions
- Melanising agents
- Melanism, uniform saturation of melanin
- Nevus depigmentosus
- Piebaldism
- Pityriasis alba
- Quadrichrome vitiligo, characterized by patches of reduced but not absent pigment
- Selective ultraviolet phototherapy
- Uvb lamps
- Xanthochromism
References
- ^ a b Halder, RM; Chappell, JL (2009). "Vitiligo update". Seminars in cutaneous medicine and surgery 28 (2): 86–92. doi:10.1016/j.sder.2009.04.008. PMID 19608058.
- ^ Nath SK, Majumder PP, Nordlund JJ (1994). "Genetic epidemiology of vitiligo: multilocus recessivity cross-validated". American Journal of Human Genetics 55 (5): 981–90. PMC 1918341. PMID 7977362.
- ^ Based on a study conducted by Dr. C. Krüger, and Dr. K.U. Schallreuter, as posted by the National Center for Biotechnology Information (http://www.ncbi.nlm.nih.gov/pubmed/22458952)
- ^ Fisher's Contact Dermatitis by Alexander Fisher, pg 470
- ^ a b c d National Institute of Arthritis and Musculoskeletal and Skin Diseases (March 2007). "What Is Vitiligo? Fast Facts: An Easy-to-Read Series of Publications for the Public Additional". Retrieved 2010-07-18.
- ^ a b c Halder RM, et al. (2007). "72. Vitiligo". In Wolff K, Freedberg IM, Fitzpatrick TB (eds). Fitzpatrick's dermatology in general medicine (7th ed.). New York: McGraw-Hill Professional. ISBN 978-0-07-146690-5. OCLC 154751587.
- ^ a b c Huggins RH, Schwartz RA, Janniger CK (2005). "Vitiligo". Acta Dermatovenerologica Alpina, Panonica, et Adriatica 14 (4): 137–42, 144–5. PMID 16435042.
- ^ Picardi A, Pasquini P, Cattaruzza MS, Gaetano P, Melchi CF, Baliva G, Camaioni D, Tiago A, Abeni D, Biondi M (2003). "Stressful life events, social support, attachment security and alexithymia in vitiligo. A case-control study". Psychotherapy and Psychosomatics 72 (3): 150–8. doi:10.1159/000069731. PMID 12707482.
- ^ Jin Y, Birlea SA, Fain PR, et al. (2010). "Variant of TYR and Autoimmunity Susceptibility Loci in Generalized Vitiligo". N. Engl. J. Med. 362 (18): 1686–97. doi:10.1056/NEJMoa0908547. PMC 2891985. PMID 20410501.
- ^ Strömberg S, Björklund MG, Asplund A, et al. (2008). "Transcriptional profiling of melanocytes from patients with vitiligo vulgaris". Pigment Cell & Melanoma Research 21 (2): 162–71. doi:10.1111/j.1755-148X.2007.00429.x. PMID 18426409.
- ^ Hedstrand H, Ekwall O, Olsson MJ, et al. (2001). "The transcription factors SOX9 and SOX10 are vitiligo autoantigens in autoimmune polyendocrine syndrome type I". The Journal of Biological Chemistry 276 (38): 35390–5. doi:10.1074/jbc.M102391200. PMID 11423552.
- ^ Mashaghi A et al. (2010). "Possible association of the CD4 gene polymorphism with vitiligo". Clin Exp Dermatol 35 (5): 521–4. doi:10.1111/j.1365-2230.2009.03667.x. PMID 19843086.
- ^ Gregersen PK (2007). "Modern genetics, ancient defenses, and potential therapies". The New England Journal of Medicine 356 (12): 1263–6. doi:10.1056/NEJMe078017. PMID 17377166.
- ^ Jin Y, Mailloux CM, Gowan K, et al. (2007). "NALP1 in vitiligo-associated multiple autoimmune disease". The New England Journal of Medicine 356 (12): 1216–25. doi:10.1056/NEJMoa061592. PMID 17377159.
- ^ Rabbi Peretz Rivkin (2014). "6. Why Jews do not get vitiligo". In Yosef Rivkin. The Vitiligo Theory (1st ed.). New York. ISBN 978-1-50-045337-4.
- ^ Vitiligo by Mauro Picardo and Alain Taïeb (Dec 17, 2009), Introduction section.
- ^ a b c d e f g h i j Halder RM, et al. Vitiligo. In: Wolff K, et al. Fitzpatrick's Dermatology in General Medicine. 7th ed. New York, N.Y.: McGraw-Hill Professional; 2007
- ^ Whitton, ME; Ashcroft, DM; González, U (Oct 2008). "Therapeutic interventions for vitiligo.". Journal of the American Academy of Dermatology 59 (4): 713–7. doi:10.1016/j.jaad.2008.06.023. PMID 18793940.
- ^ a b c d Anon. "Vitiligo -Treatment". Patient UK. NHS. Retrieved 2013-06-03.
- ^ Scherschun, L; Kim, JJ; Lim, HW (2001). "Narrow-band ultraviolet B is a useful and well-tolerated treatment for vitiligo". Journal of the American Academy of Dermatology 44 (6): 999–1003. doi:10.1067/mjd.2001.114752. PMID 11369913.
- ^ Don, Philip; Iuga, Aurel; Dacko, Anne; Hardick, Kathleen (2006). "Treatment of vitiligo with broadband ultraviolet B and vitamins". International Journal of Dermatology 45 (1): 63–5. doi:10.1111/j.1365-4632.2005.02447.x. PMID 16426381.
- ^ Nisticò S, Chiricozzi A, Saraceno R, Schipani C, Chimenti S (Jan 2012). "Vitiligo treatment with monochromatic excimer light and tacrolimus: results of an open randomized controlled study". Photomed Laser Surg. 30 (1): 26–30. doi:10.1089/pho.2011.3029. PMID 22054204.
- ^ Juhlin L, Olsson MJ (1997). "Improvement of vitiligo after oral treatment with vitamin B12 and folic acid and the importance of sun exposure". Acta Derm. Venereol. 77 (6): 460–2. PMID 9394983.
- ^ Tjioe, M; Vissers, WH; Gerritsen, MJ (2006). "Topical macrolide immunomodulators: a role in the treatment of vitiligo?". American journal of clinical dermatology 7 (1): 7–12. doi:10.2165/00128071-200607010-00002. PMID 16489839.
- ^ Boone, B; Ongenae, K; Van Geel, N; Vernijns, S; De Keyser, S; Naeyaert, JM (Jan–Feb 2007). "Topical pimecrolimus in the treatment of vitiligo.". European journal of dermatology : EJD 17 (1): 55–61. doi:10.1684/ejd.2007.0093. PMID 17324829.
- ^ Olsson MJ, Juhlin L (1992). "Melanocyte transplantation in vitiligo". Lancet 340 (8825): 981. doi:10.1016/0140-6736(92)92875-G. PMID 1357390.
- ^ Olsson MJ, Juhlin L (2002). "Long-term follow-up of leucoderma patients treated with transplants of autologous cultured melanocytes, ultrathin epidermal sheets and basal cell layer suspension". The British Journal of Dermatology 147 (5): 893–904. doi:10.1046/j.1365-2133.2002.04837.x. PMID 12410698.
- ^ Duke, Alan (7 May 2013). "Autopsy reveals Michael Jackson's secrets". CNN Entertainment (CNN). Retrieved 7 May 2013. "The autopsy confirmed what Jackson told people who questioned why his skin tone became lighter in the 1980s. Jackson had 'vitiligo, a skin pigmentation disease,' [LA coroner Dr. Christopher] Rogers said. 'So, some areas of the skin appear light and others appear dark.'"
- ^ "Mad Men star Jon Hamm blames skin disease on stress | Don Draper". Theage.com.au. Retrieved 2014-02-11.
- ^ "Scott Jorgensen's extreme Vitiligo solution". mixedmartialarts.com. Retrieved 2014-04-07.
- ^ Taylor, Victoria (8 May 2014). "‘America’s Next Top Model’ contestant hasn't let rare skin condition hold her back". New York Daily News. Retrieved 9 May 2014.
External links
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Wikimedia Commons has media related to Vitiligo. |
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Pigmentation disorders/Dyschromia (L80–L81, 709.0)
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Hypo-/
leucism |
| Loss of melanocytes |
| vitiligo: |
- Quadrichrome vitiligo
- Vitiligo ponctué
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| syndromic |
- Alezzandrini syndrome
- Vogt–Koyanagi–Harada syndrome
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| melanocyte development: |
- Piebaldism
- Waardenburg syndrome
- Tietz syndrome
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Loss of melanin/
amelanism |
| albinism: |
- Oculocutaneous albinism
- Ocular albinism
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| melanosome transfer: |
- Hermansky–Pudlak syndrome
- Chédiak–Higashi syndrome
- Griscelli syndrome
- Elejalde syndrome
- Griscelli syndrome type 2
- Griscelli syndrome type 3
|
|
| other: |
- Cross syndrome
- ABCD syndrome
- Albinism–deafness syndrome
- Idiopathic guttate hypomelanosis
- Phylloid hypomelanosis
- Progressive macular hypomelanosis
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|
|
Leukoderma w/o
hypomelanosis |
- Vasospastic macule
- Woronoff's ring
- Nevus anemicus
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|
| Ungrouped |
- Nevus depigmentosus
- Postinflammatory hypopigmentation
- Pityriasis alba
- Vagabond's leukomelanoderma
- Yemenite deaf-blind hypopigmentation syndrome
- Wende–Bauckus syndrome
|
|
|
| Hyper- |
Melanin/
Melanosis/
Melanism |
| Reticulated |
- Dermatopathia pigmentosa reticularis
- Pigmentatio reticularis faciei et colli
- Reticulate acropigmentation of Kitamura
- Reticular pigmented anomaly of the flexures
- Naegeli–Franceschetti–Jadassohn syndrome
- Dyskeratosis congenita
- X-linked reticulate pigmentary disorder
- Galli–Galli disease
- Revesz syndrome
|
|
Diffuse/
circumscribed |
- Lentigo/Lentiginosis: Lentigo simplex
- Liver spot
- Centrofacial lentiginosis
- Generalized lentiginosis
- Inherited patterned lentiginosis in black persons
- Ink spot lentigo
- Lentigo maligna
- Mucosal lentigines
- Partial unilateral lentiginosis
- PUVA lentigines
- Melasma
- Erythema dyschromicum perstans
- Lichen planus pigmentosus
- Café au lait spot
- Poikiloderma (Poikiloderma of Civatte
- Poikiloderma vasculare atrophicans)
- Riehl melanosis
|
|
| Linear |
- Incontinentia pigmenti
- Scratch dermatitis
- Shiitake mushroom dermatitis
|
|
| Other/ungrouped |
- Acanthosis nigricans
- Freckle
- Familial progressive hyperpigmentation
- Pallister–Killian syndrome
- Periorbital hyperpigmentation
- Photoleukomelanodermatitis of Kobori
- Postinflammatory hyperpigmentation
- Transient neonatal pustular melanosis
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|
|
Other
pigments |
| iron: |
- Hemochromatosis
- Iron metallic discoloration
- Pigmented purpuric dermatosis
- Schamberg disease
- Majocchi's disease
- Gougerot–Blum syndrome
- Doucas and Kapetanakis pigmented purpura/Eczematid-like purpura of Doucas and Kapetanakis
- Lichen aureus
- Angioma serpiginosum
- Hemosiderin hyperpigmentation
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| other metals: |
- Argyria
- Chrysiasis
- Arsenic poisoning
- Lead poisoning
- Titanium metallic discoloration
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| other: |
- Carotenosis
- Tattoo
- Tar melanosis
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| Dyschromatoses |
- Dyschromatosis symmetrica hereditaria
- Dyschromatosis universalis hereditaria
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noco (i/b/d/q/u/r/p/m/k/v/f)/cong/tumr (n/e/d), sysi/epon
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proc, drug (D2/3/4/5/8/11)
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