Medically reviewed by Drugs. Last updated on Mar 14, Vitiligo consists of white patches of skin that are caused by the loss of melanin, the pigment that gives skin its color. Melanin is produced by special cells called melanocytes, which are destroyed in people who have vitiligo. Experts are still working out the details to understand why this disease occurs, but evidence strongly suggests that vitiligo is an autoimmune disorder, in which the body's immune system mistakenly targets and injures these specific cells within your own body.
Vitiligo can cause minor changes or extensive changes in the skin. In some people, it may be hardly noticeable, while in others it is obvious. In dark-skinned people the vitiligo patches are obvious since they contrast with normal skin. Light-skinned people may have fewer cosmetic concerns, but patches without pigment can become obvious in the summer because unaffected skin tans but vitiligo skin does not tan.
Vitiligo occurs in about 1 percent to 2 percent of the population. Approximately 30 percent of people with vitiligo have a family history of the condition. About half of people with vitiligo start showing symptoms before age People with vitiligo have an increased risk of developing certain diseases, such as hypothyroidism an underactive thyroid , hyperthyroidism an overactive thyroid , type 1 diabetes, Addison's disease a disease that causes a decrease in the function of the adrenal gland and pernicious anemia vitamin B 12 deficiency.
Also, people with these conditions have an increased risk of developing vitiligo. These medical conditions are all problems that involve the immune system attacking cells in the body. Vitiligo causes patches of white skin that are often symmetrical even , with dark or red borders. The patches can occur anywhere, but the areas most commonly affected are the backs of the hands, the face, and areas that have skin folds, such as the armpits and genitals.
Body openings, such as the lips, eyes, nipples and anus are also common areas for vitiligo, as are areas that have been sunburned. Vitiligo can occur in bursts, so that sizeable areas of skin may rapidly lose their pigment during the beginning stages of the condition, yet then these whitened skin patches may abruptly stop expanding for months or years. Vitiligo causes a pattern of skin changes that usually can be recognized easily by a doctor.
If the skin changes are in a pattern that suggests other conditions, your doctor might recommend a biopsy of the skin to be certain about your diagnosis. In a biopsy, a small piece of skin is removed and examined in a laboratory. A biopsy usually is not needed to diagnose vitiligo. In 1 out of every 5 to 10 people, some or all of the pigment eventually returns on its own and the white patches disappear. For most people, however, the whitened skin patches last and grow larger if vitiligo is not treated.
Vitiligo is a lifelong condition. Vitiligo is difficult to treat, and responses vary. The most important treatment is to protect areas of vitiligo from the sun. It is very easy for areas without pigment to become sun-burned. This increases the risk of skin cancer. Other treatments can be attempted if vitiligo causes emotional or social distress.
The goals of treatment are to minimize the contrast in color between your normal skin and skin patches that have lost pigment. If you are light-skinned, part of your treatment may be to protect your normal skin from tanning by using sunscreens with a sun protection factor SPF of at least Topical treatments are can be helpful in some people. These are applied directly to the skin.
Steroid creams or ointments are applied once a day for up to several months. These medications are not always effective, and they can thin the skin with continued use. Other medicines that may be helpful include tacrolimus Protopic and pimecrolimus Elidel , but these medicines are used with caution because of a possible link between these drugs and skin cancer or lymphoma.
Ultraviolet B light treatment can be effective for treating vitiligo in many patients.
- My Blog - Saint Albans, VT Dermatologist.
- Why an international consensus conference on vitiligo?.
- Baba Yaga - p. I (Italian Edition).
- Exegese zu Mk 3,1-6 (German Edition).
Ultraviolet light can be provided by a hand-held light box for smaller areas of skin. People who have many areas of skin involved can be treated by putting on goggles and standing inside a closet-sized light box for several minutes. The treatment must be repeated often, usually for three times a week and for at least six months.
Side effects, which should be discussed thoroughly with your dermatologist, include itching, pain and sunburn as well as increased risk of skin cancers. Psoralen plus ultraviolet A light treatment commonly called PUVA causes slightly more pronounced side effects than ultraviolet B light therapy, but it is another effective way to treat vitiligo. Psoralens are drugs that cause skin to darken when they react with ultraviolet A light.
They can be applied as a cream or taken as pills. After the psoralen medicine is used, you are exposed to ultraviolet light. PUVA treatment is not for pregnant women, women who are breastfeeding or children younger than There is also an increased risk of skin cancers. Oral medicines that suppress your immune system can sometimes allow normal pigment to return. Non-segmental vitiligo typically evolves over time, in both distribution and extension patterns. For NSV, the disease may be initially classified as acrofacial but will later progress to be better classified as generalized or universal.
Conversely, some cases of NSV may spare the extremities generalized non-acrofacial vitiligo. Some cases of NSV exhibit a flexural distribution, and others a predilection for extensor aspects, suggesting different triggers or etiologies. More recently, mixed vitiligo MV has been defined as the combination of initial SV followed by the occurrence of bilateral NSV patches several months or, more rarely, years later Ezzedine et al.
Clinically, NSV is characterized by depigmented macules that vary in size from a few to several centimeters in diameter, often involving both sides of the body with tendency toward symmetrical distribution. Transient erythema in depigmented skin may be observed following ultraviolet UV irradiation, which can be misleading in a clinical context.
During the course of the disease, hyperpigmented lesional borders may sometimes be observed, especially in dark-skinned individuals after UV exposure. Involvement of the scalp and other hair-bearing areas may manifest with localized patches of gray or white hairs. Contrary to SV, in NSV, body hairs are usually spared and remain pigmented, although hair depigmentation may occur with disease progression. The diagnosis of focal vitiligo should be considered only after having ruled out all other diagnoses, and a biopsy may be helpful to exclude other causes of focal hypopigmentation.
Table 1. Focal vitiligo refers to an acquired, small, isolated hypopigmented lesion that does not fit a typical segmental distribution, and which has not evolved into NSV after a period of 1—2 yr. When presenting in isolation, especially for genital involvement, a differential diagnosis of lichen sclerosus should be addressed by biopsy. Concomitant occurrence of genital lichen sclerosus and vitiligo has been reported Ortonne, , suggesting the possibility of a causal link between the two conditions. In fair-skinned individuals, the diagnosis of oral mucosa vitiligo is rarely made; it is unclear whether this is attributable to low incidence or low diagnostic accuracy.
Universal vitiligo corresponds to complete or nearly complete depigmentation of the skin. Scalp hair involvement is also common. However, vitiligo may spare the scalp, pubic, and axillary areas early in the course of disease. Small perifollicular, discrete, or coalescent pigmentation may persist in sun-exposed areas. Complete skin depigmentation in NSV patients who have undergone therapeutic depigmentation should be excluded from the diagnosis of VU.
Several conditions that may fit into the general clinical spectrum of vitiligo are difficult to classify into the two classical forms NSV and SV. These forms are illustrated in a glossary Appendix 2. When these lesions coexist with classical vitiligo macules, it is best classified as NSV. Other distinct conditions that may be difficult to distinguish from vitiligo clinically include idiopathic guttate melanosis and progressive macular hypomelanosis. Recent clinical reports of mixed SV and NSV have challenged the concept that these two forms of vitiligo are distinct Ezzedine et al.
Furthermore, evidence of inflammation has been demonstrated in some cases of early SV, suggesting that NSV and SV may both involve inflammatory or immune-related etiology van Geel et al. In addition to its limited, segmental distribution, SV has other distinguishing characteristics as compared to NSV.
Segmental vitiligo typically has a rapidly progressive but limited course, depigmentation spreads within the segment over a period of 6—24 months and then stop; further extension is rare.
In addition, melanocyte autografts typically yield good results in SV patients, with stable repigmentation. The pathophysiology of the segmental distribution remains highly controversial van Geel et al. This association may be viewed as an example of a superimposed segmental manifestation of a generalized polygenic disorder, in which segmental involvement precedes disease generalization and is more resistant to therapy Happle, , The presence of halo nevi and leukotrichia at onset may be risk factors for developing MV in patients with SV Ezzedine et al.
However, the precise definition of CV is unclear. Indeed, although the cutaneous depigmentation may be limited to the areas exposed to chemicals, it may extend progressively from the initial site of chemical contact to the whole body, leading to typical NSV Cummings and Nordlund, Thus, such chemical agents may serve as uncommon environmental triggers or haptens for the induction of what in fact is typical vitiligo. The opinion of the VGICC participants was that CV requires clearer definition by both case studies and epidemiological investigation in at-risk populations exposed to these causative chemicals, investigation of potential predisposing factors, time between exposure and onset of depigmentation of exposed areas, and time between first depigmentation and onset of generalized vitiligo.
As such, at this time, CV should not be included in the classification of vitiligo as a separate entity. For SV, prediction of the final theoretical size of the involved segment is an important clinical problem. For NSV, the importance of the remaining melanocyte reservoir should be evaluated in addition to the descriptive classification.
Separating SV from other types of vitiligo is the most important consensus position, principally because of its prognostic implications. A definition based on lesion size to differentiate SV from focal vitiligo was not judged appropriate. Pure mucosal and long-lasting focal vitiligo may remain in this category. For VU, no consensus was achieved on the minimal percentage of body involvement that should define the condition.
Nevertheless, precise recording of extent of skin and hair involvement, as well as possible non-cutaneous melanocyte involvement, is recommended to assess clinical evolution. A glossary with photographs is provided Appendix 2. Segmental vitiligo refers to a clinically unambiguous segmental distribution of depigmented lesions, typically associated with rapid onset and with leukotrichia. There is no consensus concerning the mechanism underlying lesion distribution in SV.
Focal vitiligo, a term that applies to localized macules characterized by loss of melanocytes, is assigned to the category UnV until more definitive classification can be made on clinical grounds generally after 1—2 yr of follow-up. Establishing the disease stability is important for making therapeutic decisions, but the notion of stable disease is subject to interpretation.
Moellmann et al. Falabella et al. However, these criteria may be challenged by clinical observations in which KP and minigraft testing are discordant. One of the striking features of vitiligo, compared to other chronic skin conditions, is the absence of clinical symptoms and overt signs of inflammation. However, histological studies indicate that an inflammatory response can be detected at the progressing edge of depigmented lesions Attili and Attili, ; Hann et al.
Skin biopsy in vitiligo is currently not recommended to make therapeutic decisions. Digitized photographic assessment is useful for individual lesions, especially when surgery is considered. For systemic treatments, reliable histological markers of disease extent and stability are important, whereas the utility of biomarkers such as enumeration of T cells or other markers has not been established and requires additional evaluation. When assessed carefully, it is not rare to document a variable course of individual lesions in a patient, with different lesions regressing, being stable, or progressing simultaneously.
When available, serial digital imaging should be employed. Ideally, stability should be assessed using the combined criteria of a clinical scoring system VASI or VETF , patient self-reporting, and serial digital imaging of specific lesions over at least 12 months. The need to perform biopsies to assess stability was not deemed mandatory until more reliable markers of disease progression are validated and readily available. Except for SV, stability is difficult to predict.
More uniform tools for assessment of stability are needed. There is no current agreement on the reliability of a skin biopsy for assessing lesion stability. In vitiligo, KP is responsible for the onset of so-called isomorphic depigmented lesions. It has long been known that vitiligo is more prominent in skin areas exposed to physical trauma compared with adjacent, more protected areas, for example, metacarpophalangeal joints versus between the joints , the nose tip and alae versus nasal sulci , axilla and elbows.
Such stimuli are termed chronic koebnerization factors, and these may be responsible for the chronicity of vitiligo. Nevertheless, scientific evidence to support this hypothesis remains lacking. The occurrence of KP in SV is controversial; in SV patients, whether KP is present or not inside or even outside the related segment is still a matter of debate. Koebner phenomenon can be assessed by history, by clinical examination, or by assessment of experimentally induced KP. Njoo et al. The VETF has recently published a position paper on KP in vitiligo, including the classification into three types of KP, that is, by history, by clinical examination, and by experimental induction van Geel et al.
The presence or absence of KP is useful for guiding therapeutic intervention, especially surgery Falabella, From a pathophysiological viewpoint, KP may result from melanocyte damage via different pathways, as in psoriasis and lichen planus Gregorio et al. So-called photosensitive vitiligo may reflect Koebnerization linked to sunburn rather than an underlying lupus diathesis. There is a general impression that KP and disease stability are related, but objective data are lacking. Report of worsening of vitiligo following phototherapy or sun exposure was mentioned by one expert at the Seoul meeting.
It was noted in the discussion that the aggravation of vitiligo during phototherapy could be multifactorial, the most frequent being just absence of control of disease with phototherapy alone, koebnerization in case of too large increments of UV radiation, and possible absence of photoadaptation Hamzavi et al.
The association of true vitiligo with systemic lupus erythematosus is considered as a rare event by experts. In fact, it appears that vitiligo may always be, at least in part, a systemic autoimmune disorder Le Poole and Luiten, ; Le Poole et al. However, for SV, evidence of a general autoimmune context is less convincing. A recent study of SV associated with halo nevi reported an increased number of T cells that recognize melanocyte antigens in SV lesional skin compared with non-lesional skin and blood van Geel et al. Similar studies of MV might shed additional light on the role of inflammation and autoimmunity in the pathogenesis of both SV and vitiligo Ezzedine et al.
Despite the considerable congruent data on the topic, opinions regarding consistent autoimmune involvement were not yet fully in favor of a consensus prior to the Bordeaux meeting Table 4. However, there was consensus on the need for additional research, especially by analyzing skin samples in cases of progressive disease.
In particular, when performing a biopsy of a vitiligo lesion, it would be worthwhile to include adjacent perilesional skin to assess disease progression in the biopsied area as immune infiltrates are most often present in a defined, narrow margin of actively depigmenting vitiligo skin Wang et al. At present, these are thought to be less often present in SV; though, this may largely reflect a relative lack of data on this topic.
The VGICC stressed the importance of recognizing vitiligo as an autoimmune disease, as this directly impacts optimal treatment strategies, which almost certainly will require controlling the autoimmune process to facilitate melanocyte proliferation and migration to achieve skin repigmentation. Nevertheless, understanding the role of immune mechanisms in progressive depigmentation does not negate the importance of identifying precipitating environmental triggers of melanocyte-directed autoimmunity, as these may be exploited to permit both disease prevention and improved treatment of existing disease.
Finally, vitiligo patients are at elevated risk of developing other concomitant autoimmune diseases, particularly autoimmune thyroid disease, and should be screened for these other diseases on a periodic basis. At present, it remains unclear what environmental factors may trigger vitiligo, and how these can be modified to either treat or prevent disease.
Vitiligo: What Causes It, Treatment, and More | Everyday Health
The VGICC nomenclature and recommendations regarding related issues is an important first step, but highlighted several still-unmet needs. The consensus on classification, definition of stability, and KP should facilitate clinical research and patient management worldwide. There is an obvious need for a pathophysiology-based classification, which may lead to better treatment approaches, but more work is needed to achieve this level of knowledge. At present, improved clinical definitions may allow investigators to better define patient subgroups for scientific investigation e.
There is also a need for a prognosis-based classification. For this purpose, case registries with careful documentation of disease presentation, progression, and treatment responses should be established. Approaches to assess the remaining melanocyte reservoir would be valuable, both to improve descriptive classification and to ascertain most appropriate treatment modality. There is a need for more homogenous reporting in vitiligo research. This most common form of vitiligo is characterized by asymptomatic, well-circumscribed, milky-white macules involving multiple parts of the body, usually a symmetrical pattern.
The disease can start at any site of the body but the fingers, hands, and face are frequently the initial sites. In acrofacial vitiligo, the involved sites are usually limited to face, head, hands, and feet. A distinctive feature is depigmentation of the distal fingers and facial orifices.
It may later include other body sites, resulting in typical generalized vitiligo. Vitiligo universalis is the most extensive form of the disease and generally occurs in adulthood. Hairs may also be partially spared. Whereas this diagnosis is facile in dark-skinned individuals, it may be difficult in very fair-skinned individuals.
Usually, VU is preceded by generalized vitiligo that gradually evolves to complete or near complete depigmentation of the skin and hair. Criteria proposed for mixed vitiligo Ezzedine et al. Mixed vitiligo in a 9-yr-old patient with patient A segmental and B non-segmental involvement. Lesions present as sharply demarcated depigmented punctiform 1- to 1.
Vitiligo punctata, patient with multiple pea-sized depigmented macules of the trunk, punctate vitiligo. The disease seems to be limited to dark-skinned individuals. The relation to true vitiligo comes from pathology and coexistence with conventional vitiligo macules. The differential diagnosis from early stage cutaneous lymphoma is of primary importance, and repeated biopsies with molecular studies of clonality may be needed. This refers to a form of generalized vitiligo seen in a young black patient that primary involved the follicular reservoir with limited skin involvement, contrasting with marked generalized hair whitening, and melanocyte loss in hair follicles Figure 4 Article in revision, PCMR.
Additional cases are required to further assess whether follicular vitiligo constitutes a separate form of vitiligo.
- Message sent successfully!
- What Is It?.
- Pictorial Websters: A Visual Dictionary of Curiosities?
Follicular vitiligo, complete whitening of the body hair A associated with depigmented macules of the face and eyelashes whitening B in a yr-old boy. Mono-segmental vitiligo is the most common form of SV, referring to the presence of one or more white de-pigmented macules distributed on one side of the body Figure 5 , usually respecting the midline although some lesions may partly cross the midline , early follicular involvement leukotrichia , and rapid development over a few weeks or months, and overall protracted course.
Monosegmental vitiligo, depigmented macules unilaterally distributed with sharp midline demarcation.
Rarely, segmental vitiligo may refer to multiple segmental lesions distributed either unilaterally or bilaterally Figure 6. The onset may be simultaneous or not. Bisegmental vitiligo, bilaterally distributed, depigmented macules of each lesion do not cross the midline. National Center for Biotechnology Information , U. Pigment Cell Melanoma Res. Author manuscript; available in PMC May 1. Ezzedine , 1, 2 H. Suzuki , 4 I. Katayama , 5 I. Hamzavi , 3 C.
Anbar , 8 C.
Understanding the symptoms of vitiligo
Silva de Castro , 9 A. Lee , 10 D. Parsad , 11 N. Le Poole , 13 N. Oiso , 14 L. Benzekri , 15 R.
Spritz , 16 Y. Gauthier , 1, 2 S. Hann , 17 M. Picardo , 18 and A. Silva de Castro. Le Poole. Author information Copyright and License information Disclaimer. Ezzedine, rf. Copyright notice. The publisher's final edited version of this article is available at Pigment Cell Melanoma Res. See other articles in PMC that cite the published article. Keywords: vitiligo, consensus conference. Why an international consensus conference on vitiligo?
The Vitiligo Global Issues Consensus Conference The VETF, which includes medical experts, scientists, and representatives of patient support groups, proposed to hold an international consensus conference on vitiligo at the International Pigment Cell Conference IPCC , attended by many physicians and scientists in the vitiligo field.
Table 1 Differential diagnosis of vitiligo. Open in a separate window. Table 2 Classification Taieb and Picardo, Focal vitiligo The diagnosis of focal vitiligo should be considered only after having ruled out all other diagnoses, and a biopsy may be helpful to exclude other causes of focal hypopigmentation. Universal vitiligo Universal vitiligo corresponds to complete or nearly complete depigmentation of the skin. Other unclassified or poorly classified generalized vitiligoid conditions Several conditions that may fit into the general clinical spectrum of vitiligo are difficult to classify into the two classical forms NSV and SV.