Pityriasis Alba

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Pediatric

ediatric D
Dermatology
ermatology
Series Editor: Camila K. Janniger, MD

Pityriasis Alba Revisited: Perspectives


on an Enigmatic Disorder of Childhood
Yuri T. Jadotte, MD; Camila K. Janniger, MD

Pityriasis alba (PA) is a localized hypopigmented


disorder of childhood with many existing clinical
variants. It is more often detected in individuals
with a darker complexion but may occur in individuals of all skin types. Atopy, xerosis, and mineral deficiencies are potential risk factors. Sun
exposure exacerbates the contrast between normal and lesional skin, making lesions more visible
and patients more likely to seek medical attention. Poor cutaneous hydration appears to be a
common theme for most risk factors and may help
elucidate the pathogenesis of this disorder. The
end result of this mechanism is inappropriate melanosis manifesting as hypopigmentation. It must
be differentiated from other disorders of hypopigmentation, such as pityriasis versicolor alba, vitiligo,
nevus depigmentosus, and nevus anemicus. Alleviation of the various risk factors via patient education on proper skin care and hygiene, use of
lubricants and emollients, topical corticosteroid
therapy in the presence of inflammation, and the
novel administration of topical anti-inflammatory
drugs such as calcineurin inhibitors can play a
crucial role in promoting remission or resolution.
Cutis. 2011;87:66-72.

80 years ago.2 Mainly seen in the pediatric population, it primarily affects the head and neck region,
with the face being the most commonly involved
site.1-3 Pityriasis alba is present in individuals with
all skin types, though it is more noticeable in those with
a darker complexion.1,3 This condition also is known
as furfuraceous impetigo, erythema streptogenes,
and pityriasis streptogenes.1 The term pityriasis alba
remains accurate and appropriate given the etiologic
elusiveness of the disorder.

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ityriasis alba (PA) is derived from the Greek word


pityron and the Latin word albus, which signify
bran or branlike and white, respectively. Widely
recognized as an idiopathic hypopigmentary disorder
evident as macules,1 it was first described more than
From the New Jersey Medical School, Newark. Dr. Jadotte is from
Dermatology and Dr. Janniger is from Dermatology and Pediatrics.
The authors report no conflict of interest.
Correspondence: Camila K. Janniger, MD, New Jersey Medical
School, MSB H-576, 185 South Orange Ave, Newark, NJ 07103
([email protected]).
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Epidemiology
Pityriasis alba primarily affects preadolescent children
aged 3 to 16 years,4 with onset typically occurring
between 6 and 12 years of age.5 Most patients are
younger than 15 years,3 with up to 90% aged 6 to
12 years and approximately 10% aged 13 to 16 years.6
The belief that there is equal prevalence and incidence among males and females3,7-10 is questionable.
For example, a point-prevalence study in Romania
showed a statistically significant male preponderance of the disease (P5.007).11 Others suggest male
to female ratio estimates as high as 2 to 1.8,12,13
The prevalence of PA in the overall pediatric
population ranges from 1.9% to 8.4%.7-9,11,13-15 In
children with poorer socioeconomic backgrounds,
incidence rates are even higher, with an overall prevalence of up to 90%.9,16 Pityriasis alba has a worldwide distribution.3,7-9,17-19
Etiology and Pathogenesis
We believe that PA results from simultaneous exposure to different culprits or repeated exposure to any
single agent, and the hypopigmentation seen in PA is
due to changes in melanosis resulting from persistently
poor skin hydration. The latter may be secondary to
an inflammatory disorder such as atopy, or secondary to noninflammatory factors such as frequent
bathing, drying soaps, or overall poor skin hygiene.
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The end result is a decrease in the number of melanocytes and melanosomes with normal melanin synthesis and melanosome transfer to epidermal cells.3,10,20,21
Atopic dermatitis is a risk factor for
PA.3,6,10,17,20,22-24 Atopy is more common in developed
countries and may be related to socioeconomic and
environmental factors. Atopic dermatitis is identified
in as many as 85% of patients.25 We believe that PA
lesions may occur secondary to preexisting atopic dermatitis, a common etiologic agent in hypomelanosis.
Sun exposure may be a risk factor. Patients with
PA are more likely to have substantial sun exposure
and less likely to consistently use sunscreen.12 However, sunlight simply makes PA more apparent,22
which is especially true in individuals with higher
Fitzpatrick skin types who tan more easily and are
more likely to remain in the sun longer.12 Findings for pityriasis versicolor alba are comparable26;
the dose of sun exposure is more important than
UV susceptibility.12
Other risk factors include xerosis, mineral deficiency, and inappropriate skin care. Pityriasis alba can
be precipitated by xerosis.7,9,10,12,17,27,28 Pityriasis alba
lesions are exacerbated by dry skin.25 There is evidence of a lower state of hydration in PA relative to
normal skin.29,30 Frequent bathing, hot baths, soaps,
and wind,12 as well as poor socioeconomic status and
hygiene, have all been implicated.9,16 Hypocupremia
was found to be remarkably related to PA.31 Given
the role of copper in melanogenesis, it could conceivably be involved in the pathogenesis of PA.31

of PA.5 Spongiosis is a consistent histologic finding.7,10,21,27,33 Dermal perivascular lymphocytic infiltrates,21 acanthosis, hyperkeratosis, and parakeratosis
often are seen.4,7,10,25,27,33 However, when present, the
epidermal changes occur in all 3 stages of PA.1 Each
stage presents some unique histologic findings. Follicular plugging and atrophic sebaceous glands are
prominent in the early stage. Damaged hair follicles
are visible in the second stage. Irregular melanization,
manifesting as hypopigmented macules often with
small areas of hyperpigmentation, is most prominent
in the late stage. Evidence of long-term dermatitis34
as well as reduced numbers of abnormally patterned
melanocytes and melanosomes appear at this stage.
Follicular plugging and spongiosis, atrophic sebaceous
glands, and irregular melanization of the basal layer
are the key diagnostic findings, with the highest yield
achieved during the first 2 stages.34

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Clinical Manifestations
Pityriasis alba can be pruritic, though it usually is
asymptomatic and often is incidentally detected.3,5,10
It most often occurs on the face, particularly the
forehead and malar ridges,6 but it also may occur on
the extremities.3,32 Pityriasis alba usually is evident
with 2 or 3 macules or patches at a time that progress
in several stages. The first (early) stage begins as an
erythematous patch with an elevated border that
may last for weeks. The second (intermediate) stage
manifests with the replacement of the patch by a
smooth scaly layer.5 The early and intermediate stages
are marked by the presence of pinpoint follicular
papules.32 The third stage presents as a visible, round,
hypopigmented macule 0.5 to 5 cm in diameter with
well-defined borders and loosely adherent scales.4,10
The patient usually seeks medical treatment during
this stage.4
Histologic Manifestations
Pityriasis alba can be diagnosed using clinical findings
alone. Histology aids in unclear diagnoses, though
it may be quite variable21 due to the different stages
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Clinical Variation
There are 2 clinical variants of classic PA (CPA):
endemic PA, which occurs in children living in poor
socioeconomic conditions, and atopy-related PA.16
The improvement of endemic PA in a few months
with mild topical hydrating creams, topical antimicrobials, and sunscreens, and its deterioration with
the use of topical corticosteroids and calcineurin
inhibitors, suggest distinctive etiologies,16 with clear
prognostic and therapeutic implications. Classic PA
also must be distinguished from extensive PA (EPA).
Both show fewer melanocytes and melanosomes,10 and
both manifest as hypopigmented macules with scaling. However, EPA is more common in adults, has a
more generalized and symmetric distribution, tends to
appear on the trunk, and usually is not associated with
a history of atopy or evidence of erythema.1 There may
be both an atopic as well as an idiopathic variant of
EPA.35 It also tends to have a more prolonged course
than CPA,27 with a female preponderance.33 The
Figure illustrates an unusual case of late-stage PA with
diffuse anatomic distribution, no erythema, and no
facial involvement in a child. We believe this patient
may have EPA, which usually occurs in adults, making
it a rather unusual case. The patient did not have a
history of atopy, pruritus, or pain.
Classic PA and EPA may be different diseases
altogether. Extensive PA and progressive extensive
or progressive macular hypomelanosis (PMH) are
almost indistinguishable as separate disease entities.33 The term extensive pityriasis alba may be a
misnomer given the complete absence of the eczematous changes that are characteristic of CPA.36,37
Progressive macular hypomelanosis, similar to EPA,
occurs mostly in adults and demonstrates ill-defined,
nonscaly, hypopigmented macules on the trunk
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Pediatric Dermatology

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Hypopigmentation of the left knee and left
arm (A). Late-stage pityriasis alba with smooth
macules greater than 5 cm in diameter evident
on the medial aspect of the patients right lower
extremity (B). Diffuse anatomic involvement of
pityriasis alba is demonstrated. Hypopigmentation of the left antecubital fossa and the right
leg are particularly prominent (C).

without preceding inflammation, pain, or any associated pruritus.37,38 Progressive macular hypomelanosis is
caused by Propionibacterium acnes, which can produce
a depigmenting agent.37,38 The fact that antimicrobials
work better than anti-inflammatory drugs
in achieving clinical resolution of PMH is
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supportive of this theory.37-39 Progressive macular


hypomelanosis can be distinguished from CPA with
Wood lamp examination, which shows red fluorescence within lesional skin.38 Histologic analysis reveals
decreased epidermal melanin and abnormal melanosome distribution.40
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Pigmenting PA (PPA) and CPA both typically


occur on the face and manifest as hypopigmented
macules with scaling. However, PPA appears as bluish
macules with substantial dermal melanin deposition
within a hypopigmented scaly region similar to CPA
lesions. Up to 65% of patients with PPA also have
a superficial fungal infection; many also have concurrent CPA.22,41

Differential Diagnosis
Conditions associated with postinflammatory
hypopigmentation usually have a history of dermatitis or other rash preceding the hypopigmented macules33,42 and include contact dermatitis, seborrheic
dermatitis, and psoriasis.38 Pityriasis alba sometimes
presents with a distribution similar to psoriasis,4,20
though the scales are flatter and much smoother and
the Auspitz sign cannot be elicited in PA.4
Vitiligo typically is found in the perioral and periocular areas and does not present with scaling.43-45
Unlike PA, the hypopigmented macules of vitiligo
actually are depigmented with total loss of melanocytes. This finding can be easily demonstrated with a
Wood lamp examination; vitiligo appears chalk white,
even in individuals with a lighter complexion.43-45
Tinea versicolor (TV) needs to be considered in
the differential diagnosis of PA.7,46-48 Pityriasis versicolor alba is a well-known variant of TV.48,49 Most
commonly seen on the trunk, groin, and axillae, it
also may be present on the face.48 Pityriasis alba and
TV are the 2 most common conditions that manifest
as hypopigmented macules with scaling. However, it
can be easily distinguished from PA with a potassium
hydroxide preparation, which will show spores and
hyphae.7 Wood lamp examination reveals a typical
yellow fluorescence not seen in PA.48 It also tends to
occur in older children and can cause widespread skin
infection, particularly in seborrheic areas.49-51
Two neurocutaneous disorders must be distinguished from PA. Hypomelanosis of Ito appears
within the first year of life as hypopigmented patches
with irregular borders. Epileptic seizures may occur,
though there is no correlation between their severity and the presence of the patches.52 The so-called
Fitzpatrick patches (ash-leaf spots) of tuberous sclerosis primarily occur on the trunk and usually are
present at birth.7,53 The majority of patients also have
central nervous system problems.54 A thorough investigation of other systems should be undertaken for
all patients.53
Nevus depigmentosus, also known as nevus achromicus, appears as a hypopigmented patch that is
often present at birth7 but usually is evident before
3 years of age.54 Its unusual distribution patterns
dermatomal, in discrete patches, or with a splashed

Differential Diagnosis of
Pityriasis Alba (PA)
Classic PA
Extensive PA
Progressive macular hypomelanosis
Pigmenting PA
Postinflammatory hypopigmentation
Vitiligo
Pityriasis versicolor alba (tinea versicolor)

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Hypomelanosis of Ito
Tuberous sclerosis

Nevus depigmentosus
Nevus anemicus

Mycosis fungoides

Nummular eczema
Leprosy

Pharmacologic nevi (ie, benzoyl peroxide, topical


or intralesional steroids, retinoic acid)
Hypopigmentation secondary to procedures (ie,
chemical peels, dermabrasion)

white paint appearanceis distinctive and quite stable.7 The trunk, particularly the back and buttocks, is
most commonly involved.21,50,54
Nevus anemicus, a congenital pediatric disorder,55 is not a pigmentary disorder. Increased localized vascular tone results in a relatively pale patch
of skin,7 usually secondary to an aberrant sympathetic response to medications.16,55 Diascopy leads
to a transient redistribution of blood, blanching the
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Pediatric Dermatology

normal skin while the pale areas of nevus anemicus


are reddened.7,55
Mycosis fungoides, a condition seen mostly in
individuals of darker complexion, rarely manifests
as hypopigmented macules but may be confused
with PA when it does.56-58 However, the presence of
nonscaly lesions on the trunk and extremities as well
as excessive numbers of epidermal T lymphocytes
are diagnostic.56
Nummular eczema; leprosy; and iatrogenic
hypopigmentation from the administration of benzoyl
peroxide, topical or intralesional steroids, retinoic
acid, chemical peels, and dermabrasion also are in
the differential diagnosis.1,59 A clinical diagnosis
is sufficient in most cases, making biopsies generally unnecessary.5 The Table provides a summary of
the differential.

Prognosis
Pityriasis alba may last anywhere from months to
years and lesions can present either in the same stage
or at different stages.32 It typically has a long-term
course, tends to relapse, and may pose a notable
aesthetic concern for patients.60 However, it usually
resolves without treatment, though there may be
some recurrence at the initial location. Pityriasis alba
may be more prominently visible in dry weather, after
tanning, and in patients with atopic dermatitis due
to a prolonged course.5,10 The disorder appears to be
limited to the pediatric population.

results, such as long-term control of the disease,


longer disease-free periods, and decreased need for
continuous treatment.62 A study of 10 patients with
Fitzpatrick skin types IV and V using pimecrolimus cream 1% showed clinical improvement within
3 weeks and near complete resolution of the lesion
at the end of the 12-week treatment period. The
cosmetic results also were well-accepted by patients,
as there was no atrophy or residual odors.63 An
increased risk for local viral infections, such as molluscum contagiosum, has been reported.64 A randomized
controlled clinical trial of tacrolimus ointment 0.1%
combined with a standard moisturizer with sun
protection factor 20 has shown remarkable results.60
Thus, calcineurin inhibitors may be preferable for
facial PA and for patients with an atopic background.
There is no single preventative measure for this
disorder. However, good skin care is always beneficial.
The regular application of sun protection and the use
of mild cleansers are excellent supplemental skin care
methods for patients with PA, though they will not
clear PA lesions.1,12,20,63

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Management
Classic PA is namely treated with lubricants and
emollients.3,6,17 Topical corticosteroids, such as hydrocortisone acetate 1% or desonide, have had some
limited success.1 The use of diiodohydroxyquin,
hydrocortisone, and coal tar may yield acceptable results.32 Only mild nonhalogenated topical
steroids should be used for facial lesions in children.5,61 Topical tretinoin also may be helpful.5,10
Nonfacial PA can be treated with stronger corticosteroids, such as hydrocortisone valerate or alclomethasone diproprionate.20 Pityriasis alba in patients with
poor socioeconomic backgrounds may be alleviated
by improvements in living standards and education
on proper skin care. Lubricants and emollients also
are useful in EPA, but topical corticosteroid therapy is
ineffective. However, EPA may resolve with psoralen
plus UVA therapy.34 Both EPA and PMH seem to
resolve with UVA therapy, which supports the idea
that they are the same disorder.
Given their similarities, treatments initially geared
toward atopic dermatitis have been tested on PA
patients. A study on the use of pimecrolimus cream 1%
in atopic dermatitis patients showed promising
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Conclusion
Pityriasis alba is a common pediatric disorder of
hypopigmentation that is generally self-limited. Current risk factors include atopy, xerosis, and mineral
deficiencies. The pathogenic mechanism remains
elusive. Although there is no definitive treatment,
management should be tailored to the different
clinical variants of the disorder. Given the possibility of psychologic distress secondary to the cosmetic
appearance of the disorder, PA should be treated at
the patients request.
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Pediatric Dermatology

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