Vitiligo in children

Vitiligo in children
Talia Kakourou
Athens, Greece

Data sources: Articles on vitiligo in children published

after 1995 were retrieved from PubMed. The prevalence,
etiology, clinical presentation, differential diagnosis,
treatment and management of vitiligo in children were
summarized.
Results: Vitiligo is characterized by acquired, sharply
demarcated depigmented macules or patches on the skin,
the mucous membranes and/or white hair and it is mainly
differentiated from congenital achromic skin lesions. It is
frequently associated with various autoimmune diseases.
Hashimoto's thyroiditis is the most common association
in children. Information on the nature, possible causes
and course of the disease leads to acceptance of the
disorder and higher compliance with the treatment. The
choice of medical treatment depends on the type, location
and duration of lesions as well as the eagerness of the
child and his/her parents to pursue therapy.
Conclusion: The management of childhood vitiligo
includes information and reassurance of young patients
and their parents on the disease, thyroid investigation,
avoidance of trigger factors, topical treatment and proper
follow-up.
World J Pediatr 2009;5(4):265-268
Key words: management;
thyroiditis;
treatment;
vitiligo

Author Affiliations: First Pediatric Department Athens University, Aghia

Sophia Children's Hospital, Athens, Greece (Kakourou T)
Corresponding Author: Talia Kakourou, MD, First Pediatric Department
Athens University, Aghia Sophia Children's Hospital, Athens, Greece (Tel:
+30 210 6755437; Fax: +30 210 6745117; Email: [email protected])

Introduction

itiligo is an acquired depigmentary disorder

affecting around 1% of the world's population.
Approximately 50% of the cases have the onset
of their disease prior to the age of 20 years and 25%
prior to the age of 14 years.[1,2] Vitiligo is characterized
by selective destruction of melanocytes of the basal
layer of the epidermis and/or occasionally the hair
follicle resulting in white patches on the skin, the
mucous membranes and/or white hair.

Etiology

Various theories have been proposed for the etiology

of vitiligo, including genetic, neural, autocytotoxic/
metabolic and autoimmune theories, all of which have
been encompassed in the convergence theory. It seems
that vitiligo has a multifactorial etiology, where genetic
factors, various kinds of stress (emotional stress, oxidative
stress with the accumulation of free radicals), accumulation
of toxic melanin precursors in melanocyte (e.g., DOPA
dopachrome, 5, 6-dihydroxyindole), disturbance of
melanocyte homeostasis (e.g., impaired intracellular and
extracellular calcium), and autoimmunity can all contribute
to the development of the disorder.[3-5]
Vitiligo is frequently associated with various
organ specific autoimmune diseases, e.g., Hashimoto's
thyroiditis, Addison's disease, diabetes mellitus type 1,
and pernicious anemia.[3] Hashimoto's thyroiditis is the
most common association in children. In a group of 54
Greek children and adolescents with known vitiligo,
Hashimoto's thyroiditis was found to be 2.5 times and
hypothyroidism 10 times more frequent than in a healthy
age- and sex-matched population.[6] It must be noted that
in only 2 out of 13 patients with vitiligo and Hashimoto's
disease, thyroiditis with overt hypothyroidism preceded
vitiligo by one year while in the remaining 11 patients
Hashimoto's thyroiditis with subclinical hypothyroidism
was revealed by laboratory investigation after the onset
of vitiligo.[6] This finding is important as far as the
management of children with vitiligo is concerned.

This paper was presented in the 25th International Congress of Pediatrics,

Athens, 25-30 August 2007.
doi:10.1007/s12519-009-0050-1
2009, World J Pediatr. All rights reserved.

Review article

Background: Vitiligo is an acquired depigmentary

disorder affecting around 1% of the world's population.
In 25% of cases it has its onset prior to the age of 14
years.

Clinical presentation

Vitiligo is characterized by acquired, sharply demarcated

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World Journal of Pediatrics

Review article

depigmented macules or patches that can appear

anywhere on the skin though there is a predilection
for orifices and bony prominences. The disease is
classified according to the distribution and extent of
depigmentation as follows:
Generalized vitiligo is the most common type in both
children and adults and consists of scattered macules over
the entire body, usually in a symmetrical distribution;
Acrofacial vitiligo affects facial orifices and distal
fingers;
Segmental vitiligo occurs in a dermatomal or
quasidermatomal region (It is more common in children
than in adults. It tends to spread rapidly over the affected
area, although activity usually ceases after a short period
and the involvement of other body sites is unusual);
Focal vitiligo with one or more macules in the same
area but not segmentally distributed;
Universal vitiligo is characterized by a complete or
nearly complete depigmentation of the body (>80% of
surface area).[1,3]

Differential diagnosis

266

The diagnosis of vitiligo is based on the patient's

history and clinical presentation. The doctors should
keep in mind that vitiligo lesions are acquired, show
complete depigmentation, and have well demarcated
sometimes hyperpigmented borders and normal skin
texture. The disease can be easily differentiated from
achromic nevus (congenital lesion with irregular nonhyperpigmented borders), albinism or piebaldism
(The lesions are present at birth and in piebaldism are
usually confined to the head and upper trunk), morphea
or lichen sclerosus (abnormal skin texture), and post
infectious/post inflammatory hypopigmentation (e.g.,
tinea versicolor, varicella, pityriasis alba) where the
lesions are hypopigmented rather than depigmented. It
must be noted that examination under Wood's light is
helpful in discriminating between partial versus complete
depigmentation.

Treatment

The goal of treatment is to suppress depigmentation

and stimulate repigmentation. This is achieved by
suppression of inflammation (e.g., topical corticosteroids,
immunomodulators) or oxidation (e.g., vitamin D3
analogues, pseudocatalase) in early active lesions and/or
stimulation of melanocyte division and migration (e.g.,
phototherapy [PUVA, narrowband UVB, 308 nm excimer
laser, heliotherapy] and vitamin D3 analogues). It must
be noted that the melanocytes that migrate into the basal
layer of the depigmented skin come from contiguous

pigmented skin (Melanocytes migrate about 2-3 mm

into the depigmented skin) and from the hair follicle of
the lesion.[7] Therefore, early small lesions have a better
response to treatment than the long-standing larger ones.
Besides, lesions on the face and neck respond better to
treatment than those located on the trunk and especially
on the distal extremities and bony prominences with
a low density of hair follicles or without hair follicles.
Since vitiligo skin is slow to respond to treatment, topical
preparation should be applied for at least 4 months and
ultraviolet light exposure continued for 6 months[8,9] prior
to discontinuation for lack of response.
Studies showed that only 16%-36% of dermatologists
actively treat vitiligo for a better recovery.[10,11] The
following is a brief review of treatment of childhood
vitiligo, suggesting that this is an unjustifiably pessimistic
attitude toward the disease management.

Topical corticosteroids
Among the various therapeutic regimens proposed for
vitiligo the most widely prescribed treatment in children
is the application of topical corticosteroids. A prospective
study[12] showed that: a) 13 of 23 children (57%) with
vitiligo (mean age: 7.9 years, mean duration of vitiligo
1.3 years) treated with a medium strength topical
steroid (prednicarbate 0.25%) twice a day for at least
four months had 50% or greater repigmentation to all
involved skin areas and b) children with non-segmental
vitiligo had a better response than those with segmental
vitiligo (71.4% vs 33.3%). Topical corticosteroids,
however, have local (e.g., atrophy, striae, telangiectasia)
and systemic side-effects.[13]
Calcineurin inhibitors
Following the introduction of topical immunomodulators, several studies have shown their equal
or near equal efficacy on topical corticosteroids.[14-18]
In a retrospective study,[16] 57 children with vitiligo
(mean age: 9.2 years; mean duration of vitiligo 2.9
years) were treated with 0.03% tacrolimus or 0.1%
ointment once or twice daily for at least 3 months. As
a result, 50% regimentation was achieved in 67% of
patients with vitiligo on the head and neck including
the segmental type and 41% of patients with vitiligo on
the trunk/extremities. The authors noted that the overall
response rates were not significantly different based on
concentration and that the response to tacrolimus twice
daily was greater than once daily. They concluded that
tacrolimus ointment should be used as the treatment
of choice for vitiligo of the head and neck, including
segmental vitiligo, in pediatric patients and as an
alternative to topical corticosteroids for patients with
vitiligo involving the trunk and extremities.

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Vitiligo in children

UV therapy
Narroband UVB (NB-UVB) phototherapy is considered
as a safe and effective therapeutic option in the treatment
of vitiligo in children. Studies[8,9,23,24] have shown that
exposure to NB-UVB 2-3 times a week on nonconsecutive
days for 6-12 months resulted in >75% repigmentation in
at least 50%-75% of children. The response to treatment
depends on the location, extent and duration of vitiligo
as well as the duration of treatment. Children affected by
recent vitiligo and/or with lesions located on the face and
neck had a better response to the therapy.[8,9,23,24] Unlike
NB-UVB phototherapy, the 308 nm excimer laser device
delivers radiation to vitiligo skin only, so it is indicated
for localized vitiligo.[25-27] Exposure to artificial UV light,
however, is time-consuming and interferes with childhood
activities including school attendance; it can lead to the loss
of many school hours. Heliotherapy (exposure to natural
UV light) is an alternative although care must be taken to
avoid sunburns.[28] The common perception that vitiliginous
skin is at increased risk for cancer from UV is not based on
epidemiological studies. It seems that mechanisms other
than that offered by melanin pigmentation, for example,
the antioxidant status may also play a protective role.[29]
Carefully controlled exposure to sunlight may therefore be
beneficial.
Pseudocatalase
Topically applied pseudocatalase PC-KUS activated by
a low-dose NB-UVB phototherapy has recently been
used in the treatment of vitiligo in children. Schallreuter
et al[30] in a retrospective study of 71 children with
vitiligo (mean age: 10.3 years) found that more than 75%
repigmentation was achieved in 66 of the 71 children on
the face/neck, 48 of 61 children on the trunk, and 40 of
55 children on the extremities after NB-UVB activated
pseudocatalase daily treatment for 8-12 months. The
total dose of NB-UVB per annum for each child was
in the range of 42-60 mJ/cm2, which is equivalent to
approximately 5.6 hours of sun exposure per annum. The

therapy had no side-effects.

Management of vitiligo in children

After the diagnosis is established, information on the

nature of the disease, treatment options, expected results
and reassurance on the benign course of vitiligo leads
to acceptance of the disorder and higher compliance
with the treatment. Emotional stress may induce and/
or exacerbate vitiligo and vice versa.[31] One of the
most important goals in the management of vitiligo,
therefore, is for the child to not loose his/her self-esteem
and confidence. Young patients should be referred to a
specialist for psychological support if necessary.
The medical record form includes the age, sex,
personal and family history of thyroid disorder/
autoimmune diseases, age at onset, potential precipitating
events including emotional stress, physical illness, skin
trauma occurring 2 to 3 months before the onset of
pigment loss, duration, location, type, extent and activity
of vitiligo as well as previously used treatments. The
above parameters are important for the choice of therapy.
The percentage of depigmentation in relation to the
total body surface is estimated by using the hand-palm
rule, i.e., a lesion the size of the patient's palm equals 1%
of his/her total body surface.[32] The activity of vitiligo is
estimated by the vitiligo disease activity (VIDA) score
which is a useful scoring system of the patient's own
opinion of the present disease activity (Table).[32] Using
the VIDA score, children are actively involved in the
assessment and management of their disorder. Photographs
are taken for all or some representative lesions.
Thyroid dysfunction is screened annually in all
children with vitiligo. In the presence of positive
antithyroid antibodies with normal thyroid function,
thyroid ultrasonography is given by an experienced
radiologist. If echographic findings are compatible with
autoimmune thyroiditis, the patient is referred to an
endocrinologist for monitoring and possible replacement
therapy. In the presence of positive antithyroid antibodies
and an elevated thyroid stimulating hormone level,
confirmed by two tests four weeks apart, the patient is
referred promptly to an endocrinologist for monitoring
and therapy. These patients have the highest rates of
Table. Vitiligo disease activity (VIDA) score on a six-point scale[32]
VIDA score
Disease activity
+4
Active in the past 6 weeks
+3
Active in the past 3 months
+2
Active in the past 6 months
+1
Active in the past year
0
Stable for at least one year
-1
Spontaneous repigmentation
Active: appearance of new lesions or expansion of existing lesions.

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Review article

Vitamin D3 analogues
Vitamin D3 analogues have also been used effectively in
the treatment of vitiligo as monotherapy[19] or combined
with exposure to NB-UVB phototherapy,[20] sunlight[21]
or topical corticosteroids.[22] A prospective study[22] of
12 children with vitiligo (mean age: 13.1 years) showed
that 10 children had a mean of 95% repigmentation after
a combined treatment of topical corticosteroids in the
morning and calcipotriene ointment in the afternoon
for an average of 4.5 months (range: 2-7 months).
Since 4 of the 10 children had previously failed trials of
topical corticosteroids alone, the combination of the two
agents might be more efficacious than the use of topical
corticosteroids as monotherapy.

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World Journal of Pediatrics

Review article

progression to overt hypothyroidism and L-thyroxine

treatment should be started.[6]
Vitiligo lesions frequently appear at sites of
microtraumas (Kbner phenomenon). Therefore, proper
skin care and avoidance of microtraumas is of great
importance. The choice of medical treatment depends on
the type, location and duration of lesions as well as the
eagerness of the child and his/her parents to pursue therapy.
In conclusion, vitiligo is not an uncommon disease in
children and it is frequently associated with Hashimoto's
thyroiditis. It can respond to therapy and this should,
therefore, always be offered. Finally, appropriate
information and psychological support of the patient and
his/her parents is of utmost importance.
Funding: None.
Ethical approval: Not needed.
Competing interest: None.
Contributors: Kakourou T is the single author of this paper.

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World J Pediatr, Vol 5 No 4 . November 15, 2009 . www.wjpch.com

Received October 6, 2008

Accepted after revision May 11, 2009