Vitiligo

Vitiligo
The right clinical information, right where it's needed
Last updated: Dec 08, 2017

Table of Contents
Summary 3
Basics 4
Definition 4
Epidemiology 4
Aetiology 4
Pathophysiology 4
Classification 5
Prevention 7
Screening 7
Secondary prevention 7
Diagnosis 8
Case history 8
Step-by-step diagnostic approach 8
Risk factors 9
History & examination factors 10
Diagnostic tests 11
Differential diagnosis 12
Diagnostic criteria 15
Treatment 16
Step-by-step treatment approach 16
Treatment details overview 17
Treatment options 19
Emerging 25
Follow up 26
Recommendations 26
Complications 26
Prognosis 27
Guidelines 28
Diagnostic guidelines 28
Treatment guidelines 28
Online resources 29
References 30
Images 35
Disclaimer 45
Summary
◊ Common acquired multi-factorial skin disease exhibiting progressive depigmentation of the epidermis
in circumscribed areas, typically without erythema or scaling.
◊ Multiple auto-immune disorders may be associated with vitiligo, most commonly auto-immune
thyroiditis.
◊ In light-skinned people and in sun-protected areas, Wood's lamp examination helps with diagnosis
and evaluation of extent of disease.
◊ Presents typically as round, depigmented macules and patches that slowly enlarge, most often in a
symmetrical distribution, and with a particular affinity to periorificial and acral skin. Hair in affected
areas may be white, particularly in areas of long-standing disease.
◊ When repigmentation occurs, it typically occurs in a perifollicular pattern.
◊ While there are no licensed treatments to induce repigmentation, treatments such as UV-B light,
topical immunosuppressants, and combination approaches using phototherapy can provide
significant improvement in most cases.
Vitiligo Basics
Definition
Vitiligo is an acquired loss of melanocytes in circumscribed areas of the epidermis, resulting in complete
depigmentation of affected skin. The extent of the disease ranges from limited, focal disease to almost
BASICS
complete (universal) pigment loss.
Epidemiology
Vitiligo is a common disorder affecting all races and geographic areas with a prevalence of 0.5% to 2%
worldwide, and approximately 0.5% in the US.[6]
There is an increased prevalence in people with a FHx of the condition. Vitiligo may manifest at any time
in an individual's lifespan with an average age at onset of 20 years. Men and women are equally affected.
A minority of cases (10% to 15%) in adults present in the segmental form. In children, the segmental
variant is more prevalent. There is an increased occurrence with exposure to chemicals that are toxic to
melanocytes.[3] [7] Worldwide studies also indicate that there is genetic heterogeneity in different ethnic
groups in terms of their susceptibility to develop vitiligo.[1] [2] [3] [4] [7]
Aetiology
Vitiligo is caused by the T-cell-mediated destruction of melanocytes. Intrinsic abnormalities present in
melanocytes probably initiate inflammation through the activation of innate immunity. This inflammation leads
to the recruitment of T cells, including cytotoxic T cells that are melanocyte-specific, recognising antigens
produced specifically by melanocytes. These cytotoxic T cells destroy the melanocytes, therefore leading to
the loss of melanin production and pigmentation of the overlying keratinocytes.[8] [9] [10] This is manifest
clinically as white macules and patches of the skin.
Pathophysiology
Characteristic abnormalities in melanocytes from patients with vitiligo include elevated reactive oxygen
species and activation of the unfolded protein response, which generates the release of pro-inflammatory
signals.[11] [12] [13] [14] [15] [16] These cellular stress-induced signals most likely activate innate immune
populations within the skin.[17]
Dendritic cells are innate immune cells that acquire antigens and present them to T cells, thus activating
adaptive immune responses. Studies have suggested that heat shock proteins and other stress-induced
signals released by melanocytes activate nearby dendritic cells, enabling them to become more effective
activators of T-cell responses.[18] [19]
T-cell activation and recruitment correlates with the expression of interferon gamma and interferon gamma-
induced genes within the skin. Studies in animal models have implicated a role for both interferon gamma
and the interferon gamma-induced chemokine CXCL10 in both the progression and maintenance of
depigmentation in vitiligo.[20]
The majority of established treatments for vitiligo primarily act by suppressing immune responses directly
within the skin, although they may also promote melanocyte growth and migration, particularly phototherapy
4 This PDF of the BMJ Best Practice topic is based on the web version that was last updated: Dec 08, 2017.
BMJ Best Practice topics are regularly updated and the most recent version
of the topics can be found on bestpractice.bmj.com . Use of this content is
subject to our disclaimer. © BMJ Publishing Group Ltd 2017. All rights reserved.
Vitiligo Basics
through narrowband UV-B. All of the above pathways are potential targets for the development of new
treatments.[21]
Genome-wide association studies support these observations, having implicated genetic risk variants that
BASICS
include members of the melanin biosynthesis pathway, cellular stress, and innate and adaptive immune
responses.[22] [23]
Classification
Revised classification/nomenclature of vitiligo[1]
Vitiligo has been classified clinically into two major forms: vitiligo and the segmental variant.
Vitiligo
• Formerly known as vitiligo vulgaris

• Most common form of vitiligo. Characterised by asymptomatic, well-circumscribed, milky-white
macules involving various body parts, usually in a symmetrical pattern. May begin at any site of the
body, but the face, genitals, fingers, and hands are frequently the initial sites. Subtypes include:
• Acrofacial vitiligo: involved sites are usually limited to the face, head, hands, and feet. A
distinctive feature is depigmentation of the distal fingers and around the facial orifices. It may
later include other body sites, resulting in typical generalised vitiligo
• Vitiligo universalis: the most extensive form of the disease, which generally occurs in adulthood.
This term is generally used when depigmentation is virtually universal (>80% of the body
surface), but some pigmentation may still be present. Hairs may also be spared
• Mucosal vitiligo: typically refers to the involvement of the oral and/or genital mucosa. It may
present as part of generalised vitiligo when associated with other sites of skin involvement, or as
an isolated condition
• Mixed vitiligo: refers to concomitant occurrence of segmental vitiligo with additional remote
depigmentation on the contralateral side of the body
• Focal vitiligo: refers to a small isolated patch that may evolve into more widely distributed vitiligo
or the segmental variant.
Segmental vitiligo (SV)
• Monosegmental vitiligo is the most common form of SV. It refers to the presence of one or more white
depigmented macules distributed on one side of the body, 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 an overall protracted course
• Rarely, segmental vitiligo may refer to multiple segmental lesions distributed either unilaterally or
bilaterally. The onset may be simultaneous or not. A clear segmental distribution of the lesions with
midline demarcation, together with the associated features described in monosegmental cases (i.e.,
leukotrichia, protracted course) distinguishes this diagnosis from vitiligo with bilateral distribution.
This PDF of the BMJ Best Practice topic is based on the web version that was last updated: Dec 08, 2017.
5
Vitiligo Basics
Fit zpatrick skin type[2]
Type I: always burns, never tans (pale white skin)
Type II: always burns easily, tans minimally (white skin)

BASICS
Type III: burns moderately, tans uniformly (light brown skin)
Type IV: burns minimally, always tans well (moderate brown skin)
Type V: rarely burns, tans profusely (dark brown skin)
Type VI: never burns (deeply pigmented dark brown-to-black skin)
Vitiligo Prevention
Screening
There is no benefit from screening for vitiligo. Vitiligo is more common in patients with a FHx of vitiligo
or other auto-immune disorders. Patients with halo nevi (a melanocytic naevus surrounded by a halo of
depigmentation) may be more likely to develop vitiligo, and should have a total body skin examination for
additional lesions.
[Fig-7]
[Fig-8]
Secondary prevention
Sun protection including broad-spectrum sunscreens may prevent unwanted effects of overexposure to UV
radiation.
PREVENTION
7
Vitiligo Diagnosis
Case history
Case history #1
A 21-year-old Hispanic woman presents with localised sunburn around her eyes. The affected skin is
notable for pigment loss with additional white macules appearing around the fingertips and on the elbows.
On PE there are well-demarcated and depigmented patches of skin, without scaling or infiltration, in a
generalised distribution over the body. Small patches are noted on the perineum. Additional affected
areas were identified on both hips with Wood's lamp examination. A depigmented halo surrounds
1 naevus on the right scapula. Patches in sun-exposed areas show small, perifollicular macules of
pigmentation.
Other presentations
Other presentations of vitiligo include inflammatory vitiligo (inflammatory infiltrate at the tissue level
reflected in a slightly raised erythematous border, often with scale, of actively enlarging lesions),
trichrome vitiligo (a third colour, a shade between depigmented and normally pigmented skin, is present),
and confetti-like depigmentation (many tiny 1-2 mm macules of depigmentation in clusters). These
presentations frequently mark highly active disease.[3] [4] [5]
Step-by-step diagnostic approach

The primary care physician will make the diagnosis in most cases of vitiligo. Only in atypical cases will the
expertise of a dermatologist be required.[28]
History
Discomfort or pain from sunburn may be a presenting symptom, and patients may report a history of
DIAGNOSIS
sunburn localised to lesional skin.[29] Cutaneous trauma may be implicated in the appearance of new
vitiligo or the spread of existing areas. Physical trauma (e.g., surgical incisions and friction) and sunburn
have been widely described as triggers (Koebner's phenomenon). The clinical evaluation is supported by
history to exclude other genetic, inflammatory, or trauma-induced disorders. Age less than 30 years and a
family history of vitiligo or related auto-immune diseases are strong risk factors for development of vitiligo.
In up to 20% of patients at least 1 first-degree relative also suffers from vitiligo.[25] These patients may
have earlier-onset disease.
A family history of auto-immune disorders is found in up to 20% of patients with vitiligo.[25]The disease
most often associated with vitiligo is auto-immune thyroid disease.[24] [26] Less commonly, associated
conditions include pernicious anaemia, SLE, alopecia areata, type 1 diabetes, and Addison's disease.
Physical examination
The hallmark of vitiligo is completely depigmented, rather than hypopigmented skin. Examination of the
whole skin may reveal involvement at any site, although the face, genitals, acral, and periorificial skin are
most commonly affected. Three different levels of pigmentation may be observed in so-called trichrome
vitiligo, in which a rim of hypopigmented epidermis typically surrounds completely achromic areas of skin.
Vitiligo Diagnosis
Halo (Sutton) naevus is found 10 times more commonly in patients with vitiligo.[24] Erythema, oedema,
and blistering of the affected areas from sunburn can also be presenting signs. Depigmentation may
affect the epithelium of the retina and the choroid in up to 40% of patients. Uveitis is also an associated
finding in patients with vitiligo.[30]
[Fig-1]
[Fig-2]
[Fig-3]
[Fig-4]
[Fig-5]
[Fig-6]
[Fig-7]
[Fig-8]
[Fig-9]
Wood's lamp examination

Wood's lamp (black light) emits UV-A radiation (315-400 nm) with a peak at 365 nm and almost no visible
light. Two criteria are helpful in diagnosing vitiligo:
• Enhancement: due to the loss of epidermal pigment and the limited penetration depth of UV-A,
Wood's lamp accentuates the contrast between involved and uninvolved skin
• Fluorescence: a distinctive bluish fluorescence that is seen in vitiligo but not in skin that is simply
hypopigmented.
[Fig-10]
DIAGNOSIS
Laboratory investigations
The association between vitiligo and auto-immune thyroiditis is quite common. Clinical evaluation for
thyroid disease should be done in all patients, but routine screening for thyroid dysfunction in patients
with no symptoms of thyroid disease is not recommended.[31] Serological testing for other conditions is of
limited value in the diagnosis of vitiligo.
Skin biopsy
A skin biopsy is rarely needed to make the diagnosis of vitiligo. Fontana-Masson stain highlights melanin
pigment in the epidermis, although melanocytes are best identified using special stains. Dihydroxy-
phenylalanine stain of frozen sections may reveal unpigmented melanocytes by showing tyrosinase
activity.
Risk factors
Strong
9
Vitiligo Diagnosis
age <30 years
• Average onset is 20 years of age, and in 80% of patients first symptoms are encountered before 30
years of age.[4] [24]
FHx of vitiligo
• Up to 20% of patients with vitiligo have at least 1 first-degree relative with the condition.[25]
auto-immune disease
• There is an increased incidence of auto-immune diseases in patients and relatives of vitiligo patients.
Patients with such association may have an early onset of vitiligo.[24] [26]
• The disease most often associated with vitiligo is auto-immune thyroid disease.[24] [26] Less
commonly associated conditions include pernicious anaemia, SLE, alopecia areata, type 1 diabetes,
and Addison's disease.
• A FHx of auto-immune disorders is found in up to 20% of patients with vitiligo.[25]
chemical contact
• Exposure to a number of chemicals has been implicated in initiating or worsening vitiligo, including
monobenzyl ether of hydroquinone, rhododendrol, and other phenols. One large population-based
study implicated the use of hair dyes as risk factors for developing vitiligo, which supported earlier
case reports.[27]
History & examination factors

Key diagnostic factors
presence of risk factors (common)
• Age <30 years, FHx of vitiligo, and associated auto-immune diseases are strong risk factors for
development of vitiligo. The disease most often associated with vitiligo is auto-immune thyroid
disease.[24] [26] Less commonly associated conditions include pernicious anaemia, SLE, alopecia
DIAGNOSIS
areata, type 1 diabetes, and Addison's disease.
acral and periorificial depigmentation (common)

• Vitiligo is defined by round depigmented skin areas that have a predilection to acral and periorificial
skin, but can also be confined to localised regions.
[Fig-2]
perianal and genital depigmentation (common)

• The presence of perianal and genital skin involvement may help to establish generalised disease.
[Fig-5]
recent cutaneous trauma (common)

• Cutaneous trauma may be implicated in the appearance of new vitiligo or the spread of existing areas.
Physical trauma (e.g., surgical incisions and friction) and sunburn have been widely described as
triggers (Koebner's phenomenon).
localised sunburn pain (common)

• Depigmented skin is more susceptible to sunburn than unaffected areas.
Vitiligo Diagnosis
enhancement and fluorescence with UV-A exposure (common)
• Wood's lamp accentuates the contrast between affected and unaffected skin (enhancement) while
fluorescence is a characteristic bluish tint typical of vitiligo.
[Fig-10]
halo naevus (common)

• Halo (Sutton) naevus is found 10 times more commonly in patients with vitiligo.[24]
[Fig-7]
[Fig-8]
universal depigmentation (uncommon)

• Depigmentation of the vast majority of the body surface, so that the remaining unaffected skin appears
as dark patches on a white skin background.
lip depigmentation (uncommon)

• Less commonly vitiligo affects mucosal surfaces only (e.g., lips).
erythema, oedema, or blistering (uncommon)

• May be presenting signs when affected areas are sun-exposed.
retinal depigmentation (uncommon)

• Depigmentation may affect the epithelium of the retina and the choroid in up to 40% of patients.
Diagnostic tests
1st test to order
Test Result
DIAGNOSIS
clinical diagnosis features of vitiligo
• There are no initial investigations; diagnosis is usually clinical.
Other tests to consider
Test Result
skin biopsy absent melanocytes from
lesional skin
• Rarely needed to make a diagnosis.
• Fontana-Masson stain highlights melanin pigment in the epidermis,
although melanocytes are best identified using special stains.
Dihydroxy-phenylalanine stain of frozen sections may reveal
unpigmented melanocytes by showing tyrosinase activity.
• Immunohistochemistry identifies molecular markers of melanocytes
(e.g., Sox10, tyrosinase, HMB 45, NKI/beteb, Mel-5, or MITF).
11
Vitiligo Diagnosis
Differential diagnosis
Condition Differentiating signs / Differentiating tests

symptoms
Piebaldism • Present at birth, non- • Clinical diagnosis.
progressive, coalescing
depigmented patches,
usually near the midline on
the front, including a forelock
of white hair.
Waardenburg's syndrome • Achromic patches present • Clinical diagnosis.

at birth, white forelock,
heterochromia iridium,
dystopia canthorum,
deafness and other
neurological symptoms,
mental retardation.
Tuberous sclerosis • Typical ash-leaf-shaped • Hypopigmentation and

hypopigmented macules, incomplete pigment loss
seizures, angiofibromas, and when examined by Wood's
mental retardation. lamp (UV light).
• Occurs predominately on the
thorax and legs.
Lichen sclerosis • Women: typically presents • Skin biopsy: reveals

in females as pruritic white lichenoid infiltrate in the
plaques in the genital area dermal-epidermal junction,
associated with epidermal hyperkeratosis, thinning, and
atrophy and scarring. loss of normal rete ridge in
Vulvar involvement may the epidermis.
present with dysuria and
dyspareunia.
DIAGNOSIS
• Men: occurs almost

exclusively in those who are
uncircumsised.
Naevus depigmentosus • Congenital condition usually • Hypopigmentation seen

noted at birth or in early under Wood's lamp
childhood. examination.
• Hypopigmented solitary
patch with jagged edges,
typically on the trunk.
Usually remains at the
same site, but may grow in
proportion to body growth.
Vitiligo Diagnosis

symptoms
Naevus anaemicus • Congenital vascular anomaly • Lesions not accentuated
that is not considered a true under Wood's lamp
pigmentary disorder. examination.
• Typically presents at birth or • Lesional pallor is due to
first noted in early childhood. vasoconstriction, not melanin
• Presents as pale macules loss; therefore, diascopy
or patches. Lesions remain (pressing on the skin with
unchanged throughout the a glass slide) makes it
patient’s life. indistinguishable from the
surrounding area.
• No melanocyte abnormalities
in histology.
Hypomelanosis of Ito • Mosaic distribution of • Clinical diagnosis.

hypomelanotic macules
and bands of speckled or
mottled, greyish-brown to
blue-black patches involving
the skin, conjunctiva, sclera,
tympanic membrane, and
oral and nasal mucosa.
• Hypochromic lesions in
distinctive patterns (e.g.,
whirls, patches, streaks) on
trunk, arms, legs, and face.
Incontinentia pigmenti • Distribution along Blaschko's • Genetic testing for NF-

lines, history of vesicular kappa-B essential modulator
eruption perinatally, female (NEMO) mutation.
gender.
Pityriasis alba • Asymptomatic ill-defined • Clinical diagnosis.

small patches with fine
DIAGNOSIS
scaling typically on the
cheeks of children and
adolescents, often with
atopic dermatitis.
Discoid lupus • Dyspigmentation • Lesional biopsy positive for

erythematosus (areas of hypo- and direct immunofluorescence
hyperpigmentation), (e.g., basement membrane).
infiltrated rim around • Hyperkeratosis with
leukoderma, follicular interface, perivascular,
plugging, scarring alopecia. and periappendageal
lymphocytic infiltrate in the
dermis, as well as mucin
infiltration.
Pityriasis versicolour • Polycyclic, well-demarcated • Pityrosporum orbiculare

lesions with fine scaling, fluoresces yellow-green
typically on the upper trunk. when exposed to Wood's
lamp examination.
• A scrape of skin scales
reveals the organism on
KOH wet mount.
13
Vitiligo Diagnosis

symptoms
Pinta • Widespread pigmentary • Positive serology, T pallidum
change with a mixture of carateum can be seen on
hyperpigmentation and darkfield microscopy of
depigmentation that can be samples taken from the early
disfiguring. papules.
Leprosy • Hypopigmentation, • Tests for diagnosis include

hypoaesthesia, thickening of viral culture, serology,
nerves, extended residence pp65 antigenaemia test,
in or migration from endemic histopathology and nucleic
area. acid amplification, and
detection systems, most
commonly PCR.
Melanoma-associated • May be indistinguishable • Biopsy of suspicious

leukoderma from vitiligo and thus is likely melanocytic lesions
a form of vitiligo rather than characterised by loss of
a distinct entity; history of pigmentation in the primary
melanoma, leukoderma at an lesion and an eccentrically
excision site, or melanoma placed hypopigmented
or metastases found upon macule.
examination.
Frostbite • Well-defined leukoderma in • Clinical diagnosis.

areas of previous traumatic
cold exposure.
Hypopigmented • Less well-circumscribed • Immunophenotype CD4 is

cutaneous T-cell than vitiligo, primarily positive, and CD7 negative;
lymphoma hypopigmentation present on CD25 positivity is variable.
sun-protected areas of the
body.
DIAGNOSIS
Hydroquinone-induced • Ill-defined or patchy areas of • Clinical diagnosis.

dermatopathy hypopigmentation, history of
using bleaching creams.
Corticosteroid-induced • Ill-defined hypopigmentation, • Clinical diagnosis.

dermatopathy localised to area of topical
or local corticosteroid
therapy; associated dermal
and epidermal atrophy,
telangiectasia, purpura,
acne.
Vitiligo Diagnosis

symptoms
Melanocyte receptor • Therapeutic agents • Clinical diagnosis.
antagonist-induced (specifically with Imatinib
dermatopathy mesilate and SU11428)
targeting C-kit (receptor
molecule in a signalling
pathway essential for
melanocyte development
and survival) may trigger
or worsen vitiligo, may also
induce hypopigmentation of
skin or hair.
Vogt-Koyanagi-Harada • Acquired depigmentation • Clinical diagnosis.

syndrome associated with ocular and
auditory compromise.
Idiopathic gut tate • Multiple small depigmented • Clinical diagnosis.

hypomelanosis macules preferring
lower extremities, slowly
progressive accumulation of
isolated lesions.
Progressive macular • Progressive hypopigmented • Skin biopsy with decreased

hypomelanosis patches on the back, chest, pigment in the epidermis,
and abdomen. but presence of melanocytes
and a normal-looking dermis.
• Wood's lamp examination
may reveal an orange
fluorescence due
to the presence of
Propionibacterium acnes .
• Electron microscopy
shows a shift from large
DIAGNOSIS
melanosomes in normal-
looking skin to small
aggregated, membrane-
bound melanosomes in
hypopigmented skin.
Diagnostic criteria
Vitiligo European Task Force[32]
Developed and validated by a panel of experts using a scoring system supported by Wood's lamp
examination to evaluate extent, stage, and progression of vitiligo. A workshop found good interobserver
concordance among the panelists.
15
Vitiligo Treatment
Step-by-step treatment approach

While vitiligo is not life-threatening, the quality of life and self-esteem in patients with vitiligo may be severely
compromised. Vitiligo may have more significant psychological impact in women and in people with darker
pigmentation, although all patients may be impacted.[33]
The therapeutic goal in vitiligo is to improve the appearance of the affected skin, and this can be approached
in different ways, which depend on the type of vitiligo, extent of disease, and wishes of the patient.
Regardless of the chosen treatment, an assessment of the patient’s perspective should be included as part
of the evaluation. An option of not treating the patient might be considered if the patient is unconcerned.
Supportive therapies
Patients should be advised to avoid cutaneous trauma where possible (e.g., physical trauma, surgical
incisions, friction, sunburn).[32] Psychological or psychiatric support should be considered in all patients.
Cosmetic coverage may be an adequate choice of treatment in some patients. The strong contrast
between affected and unaffected skin in people with darker pigmentation makes the disease more visible,
for which make-up products can be customised to match the patient's skin tone.
Over-the-counter self-tanning products containing dihydroxyacetone may also be useful in camouflaging

affected skin in patients with a lighter complexion.[34]
Segmental vitiligo/limited vitiligo

Topical therapies (i.e., corticosteroids, calcineurin inhibitors) are recommended as a first-line option
in patients with limited vitiligo (i.e., lesions that cover <2% to 3% of the body surface) or segmental
vitiligo.[32]
Topical corticosteroids work by suppressing the immune response against melanocytes, and can be
used in adults and children. They have the best response on sun-exposed areas, in dark skin, and in
recent lesions.[32] Potent corticosteroids (e.g., clobetasol, mometasone) should be tested for at least 3
months to evaluate the response. In some areas - particularly the face, genitals, axillae, and breasts - this
treatment, when used over the long term, may result in corticosteroid-induced atrophy and hypertrichosis.
Therefore, patients should be evaluated regularly for adverse effects such as skin thinning, purpura, and
striae distensae.[35] Therapy can be used on a discontinuous basis to provide benefit and potentially
avoid side effects. One useful approach is to use topical corticosteroids twice daily for 1 week, and then
no treatment for 1 week.
Topical tacrolimus has shown therapeutic efficacy without the side effects associated with
corticosteroids.[36] [37] It can be used in place of corticosteroids (particularly on the face), or during the
off-week when using corticosteroids discontinuously.
Targeted phototherapy devices (excimer laser or monochromatic lamp) that deliver light in the UV-B
range (peak at 308 nm) can be considered as second-line treatment in patients who do not respond to
topical therapies.[32] [38] [39] Treatments are given 2 to 3 times weekly for several months. This avoids
TREATMENT
unnecessary side effects due to total body irradiation.
Surgery can be considered if medical treatment is unsatisfactory to the patient and disease is stable.[32]
It aims to replace the lost melanocytes with ones from a normally pigmented autologous donor site.
Several techniques are available to achieve this, including punch grafting, epidermal blister grafting, or
Vitiligo Treatment
ultrathin epidermal sheet grafting. Cellular grafting is another technique that consists of an autologous
epidermal suspension containing melanocytes and keratinocytes. Pure cultured melanocytes could
be expanded in vitro to treat larger areas, but this is more time-consuming and needs to be done in a
laboratory by specialised staff. Regardless of the technique chosen to transplant melanocytes, surgery
is only indicated in a small number of patients with very stable vitiligo. It is usually recommended that the
disease be inactive for at least 6 months prior to surgery (and some recommend up to 2 years), and the
patient should not have a history of Koebner's phenomenon. Surgery is relatively contraindicated in areas
such as dorsum of hands.[40] [41] [42]
Widespread vitiligo
Narrow-band UV-B therapy is recommended as a first-line treatment option in these patients and should
be continued for at least 3 months, and potentially much longer if the patient responds to treatment.[32]
Narrow-band UV-B therapy can be combined with topical therapies (i.e., corticosteroids or tacrolimus) or
systemic (i.e., corticosteroids) therapies as necessary, which may result in improved efficacy.
Systemic corticosteroids are recommended as a second-line option to stabilise disease if the condition
is progressing rapidly or progresses despite therapy.[32] Minipulse therapy (i.e., the intermittent
administration of larger doses) or alternate-day dosing has been advocated when using systemic
corticosteroids.[32] [43] Use of other systemic immunosuppressants (e.g., cyclophosphamide,
cyclosporin) has been evaluated in only a limited number of studies.
Surgical options may be considered in areas that do not respond, especially those areas with a high
cosmetic impact, if the disease is stable.[32] It is usually recommended that the disease be inactive for
at least 6 months prior to surgery (and some recommend up to 2 years), and the patient should not have
a history of Koebner's phenomenon. Surgery is relatively contraindicated in areas such as dorsum of
hands.[40] [41] [42]
Depigmentation techniques could be considered as a last option in patients with unresponsive,

widespread (i.e., >50%), or highly visible recalcitrant vitiligo of face or hands.[32] Monobenzone (the
monobenzyl ether of hydroquinone) has been used to permanently depigment unaffected skin in patients
with vitiligo. Mequinol/tretinoin has also been used.[44] [45] Treatment induces depigmentation that
usually starts at the application site, but eventually affects remote areas of the body. This may take
up to 1 year and is not always permanent, although re-treatment is possible if repigmentation occurs.
Depigmentation is associated with permanent photosensitivity.
For remaining areas that do not respond to chemical depigmentation, or for small pigmented islands,
laser-assisted melanocyte removal may be considered. The Q-switched ruby laser is well suited for this
approach, and works much faster than chemical depigmentation.[46] [47]
Treatment details overview

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drug interactions, and alternative dosing. ( see Disclaimer )
TREATMENT
Ongoing ( summary )
Patient group Tx line Treatment
17
Vitiligo Treatment
Ongoing ( summary )
segmental vitiligo or limited 1st topical corticosteroid and/or tacrolimus
vitiligo
plus supportive therapies
2nd phototherapy
3rd surgery
widespread vitiligo (more than 3% 1st phototherapy ± topical or oral

body surface area) corticosteroid or topical tacrolimus
2nd oral corticosteroid
3rd surgery
4th depigmentation therapies

TREATMENT
Vitiligo Treatment
Treatment options
Ongoing
segmental vitiligo or limited 1st topical corticosteroid and/or tacrolimus

vitiligo
» Recommended as a first-line option in patients
with segmental vitiligo or limited vitiligo (i.e.,
lesions that cover <2% to 3% of the body
surface).[32]
» Topical corticosteroids have the best response

on sun-exposed areas, in dark skin, and in
recent lesions.[32] Potent corticosteroids (e.g.,
clobetasol, mometasone) should be tested for
at least 3 months to evaluate the response. In
some areas - particularly the face, genitals,
axillae, and breasts - this treatment, when used
over the long term, may result in corticosteroid-
induced atrophy and hypertrichosis. Therefore,
patients should be evaluated regularly for
adverse effects such as skin thinning, purpura,
and striae distensae.[35]
» Therapy can be used on a discontinuous basis

to provide benefit and potentially avoid side
effects. One useful approach is to use topical
corticosteroids twice daily for 1 week, and then
no treatment for 1 week.
» Topical tacrolimus has shown therapeutic

efficacy without the side effects associated with
corticosteroids.[36] [37] It can be used in place
of corticosteroids (particularly on the face), or
during the off-week when using corticosteroids
discontinuously.
Primary options
» clobetasol topical: (0.05%) apply to the

affected area(s) twice daily, maximum 50 g/
week
OR
Primary options
» mometasone topical: (0.1%) apply to the

affected area(s) once daily
TREATMENT
OR
Primary options
» tacrolimus topical: (0.1%) apply to the

affected area(s) twice daily
19
Vitiligo Treatment
Ongoing
» Patients should be advised to avoid cutaneous
trauma where possible (e.g., physical trauma,
surgical incisions, friction, sunburn).[32]
» Psychological or psychiatric support should be

considered in all patients.
» Cosmetic coverage may be an adequate

choice of treatment in some patients. The strong
contrast between affected and unaffected skin
in people with darker pigmentation makes
the disease more visible, for which make-
up products can be customised to match the
patient's skin tone.
» Over-the-counter self-tanning products

containing dihydroxyacetone may also be useful
in camouflaging affected skin in patients with a
lighter complexion.[34]
2nd phototherapy
» Targeted phototherapy devices (excimer laser
or monochromatic lamp) that deliver light in the
UV-B range (peak at 308 nm) can be considered
as second-line treatment in patients who do not
respond to topical therapies.[38] [39]
» Treatments are given 2 to 3 times weekly for

several months. This avoids unnecessary side
effects due to total body irradiation.



3rd surgery
TREATMENT
» Surgery can be considered if medical

treatment is unsatisfactory to the patient and
disease is stable.[32]
Vitiligo Treatment
Ongoing
» Several techniques are available to achieve
this, including punch grafting, epidermal blister
grafting, or ultrathin epidermal sheet grafting.
» Cellular grafting is another technique that

consists of an autologous epidermal suspension
containing melanocytes and keratinocytes.
» Pure cultured melanocytes could be expanded

in vitro to treat larger areas, but this is more
time-consuming and needs to be done in a
laboratory by specialised staff.
» Regardless of the technique chosen to

transplant melanocytes, surgery is only indicated
in a small number of patients with stable vitiligo.
It is usually recommended that the disease be
inactive for at least 6 months prior to surgery
(and some recommend up to 2 years), and the
patient should not have a history of Koebner's
phenomenon.
» Surgery is relatively contraindicated in areas



widespread vitiligo (more than 3% 1st phototherapy ± topical or oral

body surface area) corticosteroid or topical tacrolimus
» Narrow-band UV-B therapy is recommended
as a first-line treatment option in these patients
and should be continued for at least 3 months,
and potentially much longer if the patient
responds to treatment.[32]
» May be combined with topical therapies (i.e.,

corticosteroids or tacrolimus) or systemic (i.e.,
corticosteroids) therapies as necessary, which
may result in improved efficacy.
Primary options
TREATMENT
» phototherapy
OR
Primary options
21
Vitiligo Treatment
Ongoing
» phototherapy
--AND--
» clobetasol topical: (0.05%) apply to the
affected area(s) twice daily, maximum 50 g/
week
-or-
» mometasone topical: (0.1%) apply to the
affected area(s) once daily
-or-
» tacrolimus topical: (0.1%) apply to the
-or-
» dexamethasone: consult specialist for
guidance on oral dose

» Cosmetic coverage may be an adequate

choice of treatment in some patients. The strong
contrast between affected and unaffected skin
in people with darker pigmentation makes
the disease more visible, for which make-
up products can be customised to match the
patient's skin tone.

2nd oral corticosteroid

» Systemic corticosteroids are recommended as
a second-line option to stabilise disease if the
condition is progressing rapidly or progresses
despite therapy.[32]
» Minipulse therapy (i.e., the intermittent

administration of larger doses) or alternate-day
dosing has been advocated when using systemic
corticosteroids.[32] [43]
Primary options
TREATMENT
» dexamethasone: consult specialist for

guidance on oral dose
Vitiligo Treatment
Ongoing


3rd surgery
» Surgical options may be considered in areas
that do not respond, especially those areas
with a high cosmetic impact, if the disease is
stable.[32]
» Several techniques are available to achieve

this, including punch grafting, epidermal blister
grafting, or ultrathin epidermal sheet grafting.
» Cellular grafting is another technique that

consists of an autologous epidermal suspension
containing melanocytes and keratinocytes.
» Pure cultured melanocytes could be expanded

in vitro to treat larger areas, but this is more
time-consuming and needs to be done in a
laboratory by specialised staff.
» Regardless of the technique chosen to

transplant melanocytes, surgery is only indicated
in a small number of patients with stable vitiligo.
It is usually recommended that the disease be
inactive for at least 6 months prior to surgery
(and some recommend up to 2 years), and the
patient should not have a history of Koebner's
phenomenon.
» Surgery is relatively contraindicated in areas



TREATMENT
4th depigmentation therapies
23
Vitiligo Treatment
Ongoing
» Last option in patients with non-responding,
widespread (i.e., >50%), or highly visible
recalcitrant vitiligo of face or hands.[32]
» Topical monobenzone has been used to

permanently depigment unaffected skin in
patients with vitiligo. Mequinol/tretinoin has also
been used.[44] [45]
» Treatment induces depigmentation that

usually starts from the application site, but
eventually affects remote areas of the body.
This may take up to 1 year and is not always
permanent, although re-treatment is possible
if repigmentation occurs. Depigmentation is
associated with permanent photosensitivity.
» For remaining areas that do not respond to

chemical depigmentation, or for small pigmented
islands, laser-assisted melanocyte removal may
be considered. The Q-switched ruby laser is well
suited for this approach, and works much faster
than chemical depigmentation.[46] [47]
Primary options
» monobenzone topical: (20%) apply to the

affected area(s) twice to three times daily
OR
Primary options
» mequinol/tretinoin topical: (2%

mequinol/0.01% tretinoin) apply to the

TREATMENT
Vitiligo Treatment
Emerging
Afamelanotide
A potent synthetic analogue of the naturally occurring melanocortin peptide hormone. It has been used in
combination with narrowband UV-B phototherapy. Preliminary results suggest that the combination may
promote faster and more extensive repigmentation compared with phototherapy alone. However, further
studies are required to confirm this observation.[48]
Janus kinase (JAK) inhibitors

Janus kinases are a family of intracellular proteins involved in the signal transduction of several cytokines,
including interferon gamma. Two patients with widespread vitiligo were reported to demonstrate significant
repigmentation after being treated with two different oral JAK inhibitors, tofacitinib and ruxolitinib. This
effect seems to be due to interference with interferon gamma signalling, which is critical for vitiligo
pathogenesis.[49] [50]Further studies suggest this response may require concomitant light exposure. [51]In
addition, an open-label proof-of-concept trial of topical ruxolitinib showed significant repigmentation in facial
vitiligo.[52]
Pseudocatalase
The use of topical oxygen radical scavengers is based on research showing increased levels of hydrogen
peroxide (H2O2) in affected skin. Pseudocatalase is proposed to work as an H2O2 scavenger and lead to
repigmentation when combined with narrowband UV-B phototherapy. Only limited studies have been able to
show good results; subsequent studies have not confirmed efficacy. More independent studies are needed
before recommending this treatment option.[53]
Antioxidants
The concept of a pathogenic role of noxious compounds derived from metabolic by-products (e.g.,
reactive oxygen species, melanin precursor radicals) or environmental sources has resulted in therapeutic
approaches using supplementation with vitamins and antioxidants. In a double-blind, placebo-controlled
study of narrowband UV-B with oral antioxidants, the combination regimen yielded mildly better results.
Gingko biloba extract has also shown promise in several studies. Polypodium leucotomos extract seems to
have antioxidant and immunomodulatory effects, and may work well in combination with phototherapies. All
of these regimens need substantiation of efficacy by larger studies.[54] [55] [56]
Helium neon laser

Repigmentation of vitiligo may occur after long-term courses of exposure to 633-nm radiation from a helium
neon laser with or without topical tacrolimus. Larger confirmatory studies are needed.[57] [58]
Combination phototherapy
The combinations of khellin or phenylalanine with UV-A irradiation (as described for PUVA) share the
advantage of being clinically non-phototoxic. These regimens are not recommended because of limited data
on their effectiveness.
TREATMENT
25
Vitiligo Follow up
Recommendations
Monitoring
FOLLOW UP
Regular clinical examinations are required to monitor disease course, especially in patients undergoing
treatment. Patients should also be evaluated for signs and symptoms of associated auto-immune
disorders, especially thyroid disease.
Patient instructions
Patients are offered information about epidemiology, aetiology, and pathomechanism to demystify the
disease process. [National Institute of Arthritis and Musculoskeletal and Skin Diseases] Patients are
also encouraged to voice psychosocial concerns and to seek help for these issues. Sun protection and
avoidance of Koebnerisation (i.e., depigmentation in response to trauma) should be discussed, and
patients may be referred to support groups that provide encouragement, additional information, and social
support. [Vitiligo Clinic & Research Center]
Complications
Complications Timeframe Likelihood

Koebner's phenomenon short term medium
Up to 50% of patients report Koebner's phenomenon (i.e., depigmentation in response to trauma), which
may indicate progressive disease. Prevention is primary.[61]
photosensitivity (sunburn) long term high
Most vitiligo patients experience some degree of photosensitivity. Sunburn in the skin that lacks protection
by melanin may be the first symptom of the disease. Sun protection including broad-spectrum sunscreen is
effective in preventing sunburn.
treatment-induced permanent photosensitivity long term high
Chemically induced depigmentation is associated with permanent photosensitivity on the entire body.
Laser-assisted melanocyte removal is often employed to avoid this complication where areas of
involvement are small or sparse and localised depigmentation is desired.
corticosteroid-induced atrophy and hypertrichosis long term medium
Potent corticosteroids are commonly used for at least 2 months to evaluate response. With treatment of
large areas, systemic corticosteroid effects may result. A minipulse or alternate-day therapy has been
advocated when using systemic corticosteroids.[43]
dermatoheliosis long term low
Dermatoheliosis is the sum of chronic sun-induced cutaneous changes, ranging from wrinkling to potential
cutaneous malignancies. Although lack of pigment in albinism (skin phototype I) is associated with early
and enhanced dermatoheliosis, this association is not clearly present in vitiligo.[62]
skin cancer long term low
Vitiligo Follow up
Complications Timeframe Likelihood

Conceptually, skin cancer risk should be increased in skin devoid of melanin protection.[63] There have
been few reports of non-melanoma skin cancer developing in vitiligo, including in patients who had
FOLLOW UP
photochemotherapy. Reduced skin cancer risk has also been reported.[64] [65] [66]
psychological dysfunction variable high
The contrast between affected and unaffected skin may create substantial interpersonal burdens.
Clinicians should aim for early psychosocial evaluation and initiate support and treatment where
needed.[33]
retinal and choroid depigmentation, uveitis variable high
Depigmentation may affect the epithelium of the retina and the choroid in up to 40% of patients. The
incidence of uveitis is also elevated in patients with vitiligo.
audiological compromise variable low
Audiological abnormalities can be measured in some patients with vitiligo, but this compromise is usually
subclinical and generally does not affect their lives.[67]
Prognosis
Vitiligo is a progressive disease that may arrest after an initial phase of variable duration. Phases of partial
repigmentation, depigmentation, or progression may follow. Complete repigmentation without treatment is
extremely rare. Localised forms of vitiligo may remain stable and restricted to a limited area, but may also
spread to become generalised. About one third of vitiligo patients experience partial repigmentation in sun-
exposed patches. This finding may predict a good treatment response. The majority of patients experience
greater than 75% repigmentation when following first-line therapies. A general rule of thumb is to expect 25%
improvement after 3 months, 50% improvement after 6 months, and 75% improvement after 9 months of
therapy. Long-standing disease, a segmental distribution, and poliosis are considered predictors of a less-
favourable treatment outcome. Although 50% repigmentation rates in affected skin are highly significant in
statistical terms, they may not be satisfactory to the patient. Notably, glabrous skin such as the penis, hands,
wrists, feet, and fingertips often do not respond at all.[59] [60]
27
Vitiligo Guidelines
Diagnostic guidelines
Europe
Guideline for the management of vitiligo

Published by: Vitiligo European Task Force; European Academy Last published: 2013
of Dermatology and Venereology; Union Européenne des Médecins
Spécialistes
Summary: This guideline has been developed by the members of the Vitiligo European Task Force and
their colleagues to summarise evidence-based and expert-based recommendations for the diagnosis of
vitiligo.
Revised classification/nomenclature of vitiligo and related issues: the

Vitiligo Global Issues Consensus Conference
Published by: Vitiligo Global Issue Consensus Conference Panelists Last published: 2012
GUIDELINES
Summary: First international consensus to revise classification/nomenclature of vitiligo and related

issues, with the goal of guiding clinical research and optimal patient management.
Treatment guidelines
Europe
Guideline for the management of vitiligo

Published by: Vitiligo European Task Force; European Academy Last published: 2013
of Dermatology and Venereology; Union Européenne des Médecins
Spécialistes
Summary: This guideline has been developed by the members of the Vitiligo European Task Force and
their colleagues to summarise evidence-based and expert-based recommendations for the treatment of
vitiligo.
Asia
Standard guidelines of care for vitiligo surgery

Published by: IADVL Dermatosurgery Task Force Last published: 2008
Summary: Provides standards of care for various surgical methods of treatment of vitiligo.
Vitiligo Online resources
Online resources
1. National Institute of Arthritis and Musculoskeletal and Skin Diseases (external link)
2. Vitiligo Clinic & Research Center (external link)
ONLINE RESOURCES
29
Vitiligo References
Key articles
• Ortonne J-P. Vitiligo and other disorders of hypopigmentation. In: Bolognia JL, Jorrizzo JL, Rapini RP,
REFERENCES
eds. Dermatology. New York, NY: Mosby; 2008:913-938.
• Picardo M, Dell'Anna ML, Ezzedine K, et al. Vitiligo. Nat Rev Dis Primers. 2015;1:15011. Abstract
• Taieb A, Picardo M. Clinical practice: vitiligo. N Engl J Med. 2009;360:160-169. Abstract
• Alkhateeb A, Fain PR, Thody A, et al. Epidemiology of vitiligo and associated autoimmune diseases in
Caucasian probands and their families. Pigment Cell Res. 2003;16:208-214. Abstract
• Parsad D, Gupta S. Standard guidelines of care for vitiligo surgery. Indian J Dermatol Venereol Leprol.
2008;74:S37-S45. Full text Abstract
• Falabella R. Surgical approaches for stable vitiligo. Dermatol Surg. 2005;31:1277-1284. Abstract
• Gawkrodger DJ, Ormerod AD, Shaw L, et al. Guideline for the diagnosis and management of vitiligo.
Br J Dermatol. 2008;159:1051-1076. Abstract
References
1. Ezzedine K, Lim HW, Suzuki T, et al; Vitiligo Global Issue Consensus Conference Panelists. Revised
classification/nomenclature of vitiligo and related issues: the Vitiligo Global Issues Consensus
Conference. Pigment Cell Melanoma Res. 2012;25:E1-E13. Full text Abstract
2. Halder RM, Taliaferro SJ. Vitiligo. In: Wolff K, Goldsmith LA, Katz SI, et al., eds. Fitzpatrick's
dermatology in general medicine. New York, NY: McGraw-Hill; 2008:616-622.
3. Dupré A, Christol B. Cockade-like vitiligo and linear vitiligo a variant of fitzpatrick's trichrome vitiligo.
Arch Dermatol Res. 1978;262:197-203. Abstract
4. Ortonne J-P. Vitiligo and other disorders of hypopigmentation. In: Bolognia JL, Jorrizzo JL, Rapini RP,
eds. Dermatology. New York, NY: Mosby; 2008:913-938.
5. Sosa JJ, Currimbhoy SD, Ukoha U, et al. Confetti-like depigmentation: a potential sign of rapidly
progressing vitiligo. J Am Acad Dermatol. 2015;73:272-275. Abstract
6. Picardo M, Dell'Anna ML, Ezzedine K, et al. Vitiligo. Nat Rev Dis Primers. 2015;1:15011. Abstract
7. Taieb A, Picardo M. Clinical practice: vitiligo. N Engl J Med. 2009;360:160-169. Abstract
8. Alikhan A, Felsten LM, Daly M, et al. Vitiligo: a comprehensive overview. Part I. Introduction,
epidemiology, quality of life, diagnosis, differential diagnosis, associations, histopathology, etiology,
and work-up. J Am Acad Dermatol. 2011;65:473-491. Abstract
Vitiligo References
9. van den Boorn JG, Konijnenberg D, Dellemijn TA, et al. Autoimmune destruction of skin melanocytes
by perilesional T cells from vitiligo patients. J Invest Dermatol. 2009;129:2220-2232. Full text
Abstract
REFERENCES
10. Ogg GS, Rod Dunbar P, Romero P, et al. High frequency of skin-homing melanocyte-specific cytotoxic
T lymphocytes in autoimmune vitiligo. J Exp Med. 1998;188:1203-1208. Full text Abstract
11. Passeron T, Ortonne JP. Activation of the unfolded protein response in vitiligo: the missing link? J
Invest Dermatol. 2012;132:2502-2504. Full text Abstract
12. Shah AA, Sinha AA. Oxidative stress and autoimmune skin disease. Eur J Dermatol. 2013;23:5-13.
Abstract
13. Laddha NC, Dwivedi M, Mansuri MS, et al. Vitiligo: interplay between oxidative stress and immune
system. Exp Dermatol. 2013;22:245-250. Full text Abstract
14. Toosi S, Orlow SJ, Manga P. Vitiligo-inducing phenols activate the unfolded protein response in
melanocytes resulting in upregulation of IL6 and IL8. J Invest Dermatol. 2012;132:2601-2609. Full text
Abstract
15. Glassman SJ. Vitiligo, reactive oxygen species and T-cells. Clin Sci (Lond). 2011;120:99-120. Full text
Abstract
16. Schallreuter KU, Bahadoran P, Picardo M, et al. Vitiligo pathogenesis: autoimmune disease,
genetic defect, excessive reactive oxygen species, calcium imbalance, or what else? Exp Dermatol.
2008;17:139-140. Full text Abstract
17. Richmond JM, Frisoli ML, Harris JE. Innate immune mechanisms in vitiligo: danger from within. Curr
Opin Immunol. 2013;25:676-682. Full text Abstract
18. Mosenson JA, Zloza A, Klarquist J, et al. HSP70i is a critical component of the immune response
leading to vitiligo. Pigment Cell Melanoma Res. 2012;25:88-98. Full text Abstract
19. Mosenson JA, Zloza A, Nieland JD, et al. Mutant HSP70 reverses autoimmune depigmentation in
vitiligo. Sci Transl Med. 2013;5:174ra28. Full text Abstract
20. Rashighi M, Agarwal P, Richmond JM, et al. CXCL10 is critical for the progression and maintenance of
depigmentation in a mouse model of vitiligo. Sci Transl Med. 2014;6:223ra23. Full text Abstract
21. van den Boorn JG, Melief CJ, Luiten RM. Monobenzone-induced depigmentation: from enzymatic
blockade to autoimmunity. Pigment Cell Melanoma Res. 2011;24:673-679. Abstract
22. Spritz RA. Six decades of vitiligo genetics: genome-wide studies provide insights into autoimmune
pathogenesis. J Invest Dermatol. 2012;132:268-273. Full text Abstract
23. Jin Y, Andersen G, Yorgov D, et al. Genome-wide association studies of autoimmune vitiligo identify
23 new risk loci and highlight key pathways and regulatory variants. Nat Genet. 2016;48:1418-1424.
Abstract
31
Vitiligo References
24. Barona MI, Arrunategui A, Falabella R, et al. An epidemiologic case-control study in a population with
vitiligo. J Am Acad Dermatol. 1995;33:621-625. Abstract
REFERENCES
25. Shankar DS, Shashikala K, Madala R. Clinical patterns of vitiligo and its associated comorbidities: a
prospective controlled cross-sectional study in South India. Indian Dermatol Online J. 2012;3:114-118.
Full text Abstract
26. Alkhateeb A, Fain PR, Thody A, et al. Epidemiology of vitiligo and associated autoimmune diseases in
Caucasian probands and their families. Pigment Cell Res. 2003;16:208-214. Abstract
27. Wu S, Li WQ, Cho E, et al. Use of permanent hair dyes and risk of vitiligo in women. Pigment Cell
Melanoma Res. 2015;28:744-746. Abstract
28. Mason CP, Gawkrodger DJ. Vitiligo presentation in adults. Clin Exp Dermatol. 2005;30:344-345.
Abstract
29. Caron-Schreinemachers AL, Kingswijk MM, Bos JD, et al. UVB 311 nm tolerance of vitiligo skin
increases with skin photo type. Acta Derm Venereol. 2005;85:24-26. Abstract
30. Wagoner MD, Albert DM, Lerner AB, et al. New observations on vitiligo and ocular disease. Am J
Ophthalmol. 1983;96:16-26. Abstract
31. Kroon MW, Joore IC, Wind BS, et al. Low yield of routine screening for thyroid dysfunction in
asymptomatic patients with vitiligo. Br J Dermatol. 2012;166:532-538. Abstract
32. Taieb A, Alomar A, Böhm M, et al; Vitiligo European Task Force (VETF); European Academy of
Dermatology and Venereology (EADV); Union Européenne des Médecins Spécialistes (UEMS).
Guidelines for the management of vitiligo: the European Dermatology Forum consensus. Br J
Dermatol. 2013;168:5-19. Full text Abstract
33. Ongenae K, Beelaert L, van Geel N, et al. Psychosocial effects of vitiligo. J Eur Acad Dermatol
Venereol. 2006;20:1-8. Abstract
34. Suga Y, Ikejima A, Matsuba S, et al. Medical pearl: DHA application for camouflaging segmental
vitiligo and piebald lesions. J Am Acad Dermatol. 2002;47:436-438. Abstract
35. Westerhof W, Nieuweboer-Krobotova L, Mulder PG, et al. Left-right comparison study of the
combination of fluticasone propionate and UV-A vs. either fluticasone propionate or UV-A alone for the
long-term treatment of vitiligo. Arch Dermatol. 1999;135:1061-1066. Full text Abstract
36. Boone B, Ongenae K, Van Geel N, et al. Topical pimecrolimus in the treatment of vitiligo. Eur J
Dermatol. 2007;17:55-61. Abstract
37. Lepe V, Moncada B, Castanedo-Cazares JP, et al. A double-blind randomized trial of 0.1% tacrolimus
vs 0.05% clobetasol for the treatment of childhood vitiligo. Arch Dermatol. 2003;139:581-585. Full text
Abstract
38. Hofer A, Hassan AS, Legat FJ, et al. Optimal weekly frequency of 308-nm excimer laser treatment in
vitiligo patients. Br J Dermatol. 2005;152:981-985. Abstract
Vitiligo References
39. Mavilia L, Mori M, Rossi R, et al. 308 nm monochromatic excimer light in dermatology: personal
experience and review of the literature. G Ital Dermatol Venereol. 2008;143:329-337. Abstract
REFERENCES
40. Parsad D, Gupta S. Standard guidelines of care for vitiligo surgery. Indian J Dermatol Venereol Leprol.
2008;74:S37-S45. Full text Abstract
41. Na GY, Seo SK, Choi SK. Single hair grafting for the treatment of vitiligo. J Am Acad Dermatol.
1998;38:580-584. Abstract
42. Falabella R. Surgical approaches for stable vitiligo. Dermatol Surg. 2005;31:1277-1284. Abstract
43. Radakovic-Fijan S, Furnsinn-Friedl AM, Honigsmann H, et al. Oral dexamethasone pulse treatment for
vitiligo. J Am Acad Dermatol. 2001;44:814-817. Abstract
44. Oakley AM. Rapid repigmentation after depigmentation therapy: vitiligo treated with monobenzyl ether
of hydroquinone. Australas J Dermatol. 1996;37:96-98. Abstract
45. Catona A, Lanzer D. Monobenzone, Superfade, vitiligo and confetti-like depigmentation. Med J Aust.
1987;146:320-321. Abstract
46. Thissen M, Westerhof W. Laser treatment for further depigmentation in vitiligo. Int J Dermatol.
1997;36:386-388. Abstract
47. Njoo MD, Vodegel RM, Westerhof W. Depigmentation therapy in vitiligo universalis with topical 4-
methoxyphenol and the Q-switched ruby laser. J Am Acad Dermatol. 2000;42:760-769. Abstract
48. Lim HW, Grimes PE, Agbai O, et al. Afamelanotide and narrowband UV-B phototherapy for the
treatment of vitiligo: a randomized multicenter trial. JAMA Dermatol. 2015;151:42-50. Full text
Abstract
49. Craiglow BG, King BA. Tofacitinib citrate for the treatment of vitiligo: a pathogenesis-directed therapy.
JAMA Dermatol. 2015;151:1110-1112. Abstract
50. Harris JE, Rashighi M, Nguyen N, et al. Rapid skin repigmentation on oral ruxolitinib in a patient with
coexistent vitiligo and alopecia areata (AA). J Am Acad Dermatol. 2016;74:370-371. Abstract
51. Liu LY, Strassner JP, Refat MA, et al. Repigmentation in vitiligo using the Janus kinase inhibitor
tofacitinib may require concomitant light exposure. J Am Acad Dermatol. 2017 Oct;77(4):675-682.
Abstract
52. Rothstein B, Joshipura D, Saraiya A, et al. Treatment of vitiligo with the topical Janus kinase inhibitor
ruxolitinib. J Am Acad Dermatol. 2017 Jun;76(6):1054-1060. Abstract
53. Schallreuter KU, Wood JM, Lemke KR, et al. Treatment of vitiligo with a topical application of
pseudocatalase and calcium in combination with short-term UVB exposure: a case study on 33
patients. Dermatology. 1995;190:223-229. Abstract
54. Dell'Anna ML, Mastrofrancesco A, Sala R, et al. Antioxidants and narrow band-UVB in the treatment of
vitiligo: a double-blind placebo controlled trial. Clin Exp Dermatol. 2007;32:631-636. Abstract
33
Vitiligo References
55. Middelkamp-Hup MA, Bos JD, Rius-Diaz F, et al. Treatment of vitiligo vulgaris with narrow-band UVB
and oral Polypodium leucotomos extract: a randomized double-blind placebo-controlled study. J Eur
Acad Dermatol Venereol. 2007;21:942-950. Abstract
REFERENCES
56. Parsad D, Pandhi R, Juneja A. Effectiveness of oral Ginkgo biloba in treating limited, slowly spreading
vitiligo. Clin Exp Dermatol. 2003;28:285-287. Abstract
57. Lan CC, Wu CS, Chen GS, et al. Helium-neon laser and topical tacrolimus combination therapy: novel
treatment option for vitiligo without additional photocarcinogenic risks. J Eur Acad Dermatol Venereol.
2009;23:344-345. Abstract
58. Yu HS, Wu CS, Yu CL, et al. Helium-neon laser irradiation stimulates migration and proliferation
in melanocytes and induces repigmentation in segmental-type vitiligo. J Invest Dermatol.
2003;120:56-64. Full text Abstract
59. Gawkrodger DJ, Ormerod AD, Shaw L, et al. Guideline for the diagnosis and management of vitiligo.
Br J Dermatol. 2008;159:1051-1076. Abstract
60. Whitton ME, Ashcroft DM, Gonzalez U. Therapeutic interventions for vitiligo. J Am Acad Dermatol.
2008;59:713-717. Abstract
61. Njoo MD, Das PK, Bos JD, et al. Association of the Koebner phenomenon with disease activity and
therapeutic responsiveness in vitiligo vulgaris. Arch Dermatol. 1999;135:407-413. Full text Abstract
62. Calanchini-Postizzi E, Frenk E. Long-term actinic damage in sun-exposed vitiligo and normally
pigmented skin. Dermatologica. 1987;174:266-271. Abstract
63. Hexsel CL, Eide MJ, Johnson CC, et al. Incidence of nonmelanoma skin cancer in a cohort of patients
with vitiligo. J Am Acad Dermatol. 2009;60:929-933. Abstract
64. Schallreuter KU, Tobin DJ, Panske A. Decreased photodamage and low incidence of non-melanoma
skin cancer in 136 sun-exposed caucasian patients with vitiligo. Dermatology. 2002;204:194-201.
Abstract
65. Teulings HE, Overkamp M, Ceylan E, et al. Decreased risk of melanoma and nonmelanoma skin
cancer in patients with vitiligo: a survey among 1307 patients and their partners. Br J Dermatol.
2013;168:162-171. Abstract
66. Paradisi A, Tabolli S, Didona B, et al. Markedly reduced incidence of melanoma and nonmelanoma
skin cancer in a nonconcurrent cohort of 10,040 patients with vitiligo. J Am Acad Dermatol.
2014;71:1110-1116. Abstract
67. Hong CK, Lee MH, Jeong KH, et al. Clinical analysis of hearing levels in vitiligo patients. Eur J
Dermatol. 2009;19:50-56. Abstract
Vitiligo Images
Images
IMAGES
Figure 1: Focal vitiligo on wrist
From Dr John E. Harris personal collection
35
IMAGES Vitiligo Images
Figure 2: Typical periocular vitiligo with poliosis of several eyelashes

Photograph from the collection of Sarah Stein, MD, University of Chicago, used with permission
Vitiligo Images
IMAGES
Figure 3: Depigmentation of the hand
From Dr Bernhard Ortel's personal collection
37
Figure 4: Typical distribution of vitiligo: periungual skin, knuckles, and wrist

Vitiligo Images
IMAGES
Figure 5: Perianal and genital skin involvement in generalised vitiligo
39
Figure 6: Trichrome vitiligo. There are areas of hypopigmentation between normal skin and depigmented skin
From Dr John E. Harris' personal collection
Vitiligo Images
IMAGES
Figure 7: Classic appearance of a halo naevus
From Dr John E. Harris' personal collection
41
Figure 8: Halo of depigmentation around congenital naevus in black child

Vitiligo Images
IMAGES
Figure 9: Distribution patterns in vitiligo
43
Figure 10: Accentuation of clinical features by Wood's lamp examination

Vitiligo Disclaimer
Disclaimer
This content is meant for medical professionals situated outside of the United States and Canada. The BMJ
Publishing Group Ltd ("BMJ Group") tries to ensure that the information provided is accurate and up-to-
date, but we do not warrant that it is nor do our licensors who supply certain content linked to or otherwise
accessible from our content. The BMJ Group does not advocate or endorse the use of any drug or therapy
contained within nor does it diagnose patients. Medical professionals should use their own professional
judgement in using this information and caring for their patients and the information herein should not be
considered a substitute for that.
This information is not intended to cover all possible diagnosis methods, treatments, follow up, drugs and
any contraindications or side effects. In addition such standards and practices in medicine change as new
data become available, and you should consult a variety of sources. We strongly recommend that users
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DISCLAIMER
45
Contributors:
// Authors:
John E. Harris, MD, PhD

Associate Professor
Department of Dermatology, University of Massachusetts Medical School, Worcester, MA
DISCLOSURES: JEH has received consulting fees and/or research grants from the following: Pfizer,
AbbVie, Genzyme/Sanofi, Concert Pharmaceuticals, Stiefel/GSK, Mitsubishi Tanabe Pharma, Novartis,
Aclaris Therapeutics, The Expert Institute, Celgene, Biologics MD, and Dermira. JEH is an author of a
number of references cited in this monograph.
Mehdi Rashighi, MD
Physician Resident
Department of Dermatology, University of Massachusetts Medical School, Worcester, MA
DISCLOSURES: MR is an author of a number of references cited in this monograph.
// Acknowledgements:
Dr John E. Harris and Dr Mehdi Rashighi would like to gratefully acknowledge Dr Bernhard Ortel, a previous
contributor to this monograph. BO declares that he has no competing interests.
// Peer Reviewers:
Iltefat Hamzavi, MD
Senior Staff Physician
Multicultural Dermatology Center, Henry Ford Hospital, Detroit, MI
DISCLOSURES: IH is an author of a number of references cited in this monograph.
Piergiacomo Calzavara-Pinton, MD
Chair
Dermatology Department, University of Brescia, Brescia, Italy
DISCLOSURES: PC-P is an author of a number of references cited in this monograph.

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Vitiligo

The right clinical information, right where it's needed

Last updated: Dec 08, 2017

◊ When repigmentation occurs, it typically occurs in a perifollicular pattern.

complete (universal) pigment loss.

• Formerly known as vitiligo vulgaris

Segmental vitiligo (SV)

Type II: always burns easily, tans minimally (white skin)

Type III: burns moderately, tans uniformly (light brown skin)

Type V: rarely burns, tans profusely (dark brown skin)

Type VI: never burns (deeply pigmented dark brown-to-black skin)

Step-by-step diagnostic approach

Wood's lamp examination

History & examination factors

areata, type 1 diabetes, and Addison's disease.

acral and periorificial depigmentation (common)

perianal and genital depigmentation (common)

recent cutaneous trauma (common)

localised sunburn pain (common)

halo naevus (common)

universal depigmentation (uncommon)

lip depigmentation (uncommon)

erythema, oedema, or blistering (uncommon)

retinal depigmentation (uncommon)

Other tests to consider

Condition Differentiating signs / Differentiating tests

Waardenburg's syndrome • Achromic patches present • Clinical diagnosis.

Tuberous sclerosis • Typical ash-leaf-shaped • Hypopigmentation and

Lichen sclerosis • Women: typically presents • Skin biopsy: reveals

• Men: occurs almost

Naevus depigmentosus • Congenital condition usually • Hypopigmentation seen

Condition Differentiating signs / Differentiating tests

Hypomelanosis of Ito • Mosaic distribution of • Clinical diagnosis.

Incontinentia pigmenti • Distribution along Blaschko's • Genetic testing for NF-

Pityriasis alba • Asymptomatic ill-defined • Clinical diagnosis.

Discoid lupus • Dyspigmentation • Lesional biopsy positive for

Pityriasis versicolour • Polycyclic, well-demarcated • Pityrosporum orbiculare

Condition Differentiating signs / Differentiating tests

Leprosy • Hypopigmentation, • Tests for diagnosis include

Melanoma-associated • May be indistinguishable • Biopsy of suspicious

Frostbite • Well-defined leukoderma in • Clinical diagnosis.

Hypopigmented • Less well-circumscribed • Immunophenotype CD4 is

Hydroquinone-induced • Ill-defined or patchy areas of • Clinical diagnosis.

Corticosteroid-induced • Ill-defined hypopigmentation, • Clinical diagnosis.

Condition Differentiating signs / Differentiating tests

Vogt-Koyanagi-Harada • Acquired depigmentation • Clinical diagnosis.

Idiopathic gut tate • Multiple small depigmented • Clinical diagnosis.

Progressive macular • Progressive hypopigmented • Skin biopsy with decreased

Step-by-step treatment approach

Over-the-counter self-tanning products containing dihydroxyacetone may also be useful in camouflaging

Segmental vitiligo/limited vitiligo

unnecessary side effects due to total body irradiation.

Depigmentation techniques could be considered as a last option in patients with unresponsive,

Treatment details overview

plus supportive therapies

plus supportive therapies

plus supportive therapies

widespread vitiligo (more than 3% 1st phototherapy ± topical or oral

plus supportive therapies

2nd oral corticosteroid

plus supportive therapies

plus supportive therapies

4th depigmentation therapies

plus supportive therapies