Dermoscopy as a Diagnostic and Evaluation Tools in Childhood

Alopecia Totalis

Nani Kumala Dewi

, Nelva K. Jusuf

Department of Dermatology and Venereology, Faculty of Medicine Universitas Sumatera Utara, Jln. Dr. Mansur No. 66,

Medan, Sumatera Utara

Department of Dermatology and Venereology, Faculty of Medicine Universitas Sumatera Utara, Jln. Dr. Mansur No. 66,

Medan, Sumatera Utara

Keywords: Alopecia totalis, dermoscopy, alopecia areata, children

Abstract: Alopecia totalis is a manifestation of alopecia areata that is characterized by total scalp hair loss and have a

higher risk for poor prognosis and treatment failure. Early-onset, nail involvement, history of atopy, and

long duration of disease are associated with poor prognosis. Total scalp hair loss in alopecia totalis might

have a sudden onset or following partial alopecia. In the case of diffuse scalp hair loss, the clinical finding

might be similar to telogen effluvium and trichotillomania. Hence, a biopsy is required to establish the

proper diagnosis. However, this invasive diagnostic method is not favorable for most patients, especially

children. Several studies have been done to show the reliability of dermoscopy to diagnose alopecia areata.

Some dermoscopic features, such as yellow dots, black dots, broken hairs, tapering hair (exclamation

marks), and short vellus hairs, are known as characteristic findings in this disease. Sign of hair regrowth

might be detected earlier with this technique, and even it is not yet visible with naked eyes. We are

reporting an 8-year-old girl with a history of recurrent total scalp hair loss for four years and clinical

findings of diffuse hair loss on smooth surface scalp skin, nail pitting, and transverse leukonychia. Initial

dermoscopy evaluation revealed characteristic findings of alopecia areata and regrew of short vellus hair

was detected earlier with this examination. Therefore, we would like to report the use of dermoscopy as a

diagnostic and evaluation tools in childhood alopecia totalis.

1 INTRODUCTION

Alopecia areata is an autoimmune disease with

nonscarring hair loss that affects any hair-bearing

areas (Alkhalifah et al, 2010). However, the

pathogenesis is not known, and prevalence of this

disease varied from 0.7 – 3.8% around the globe

with similar gender proportion (Wasserman et al,

2007). Most patients have the onset of the first

lesion before 20 years old and 20% of cases found in

childhood (Nanda et al, 2002).

Clinical manifestation

may vary from single patch to multiple or diffuse

hair loss. In severe cases, the disease can extend to

total scalp hair loss or known as alopecia totalis, and

even including body hair in alopecia universalis

(Alkhalifah et al, 2010). Around 5% of alopecia

cases evolve into these long forms and associated

with poor prognosis, treatment failure, and high

relapse rate (Alkhalifah et al, 2010; Jang et al,

2017). Other prognostic factors such as early-onset

at a young age, long duration, ophiasis pattern, nail

abnormalities, history of atopy, other autoimmune

diseases, and family member are also related to a

poor outcome (Alkhalifah et al, 2010; Olsen, 2011).

In general, clinical diagnosis of alopecia areata is

made based on the typical pattern of hair loss with

the presence of characteristic exclamation mark hair

(Alkhalifah et al, 2010). However, in some cases,

the clinical diagnosis may not be straightforward

where biopsy evaluation may be required to confirm

the diagnosis, but this invasive technique is not

favored by most patients, especiallychildren. In the

case of alopecia totalis with diffuse or total scalp

hair loss, dermoscopy examination might be

necessary to exclude some differential diagnosis

such as telogen effluvium and trichotillomania.

Dermoscopy is a noninvasive diagnostic method

that allows evaluation of microstructures of the

epidermis, the dermo-epidermal junction, and the

papillary dermis which are not visible to the naked

430

Dewi, N. and Jusuf, N.

Dermoscopy as a Diagnostic and Evaluation Tools in Childhood Alopecia Totalis.

DOI: 10.5220/0009990904300435

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 430-435

ISBN: 978-989-758-469-5

eye (Mane et al, 2011). Characteristic dermoscopic

features of alopecia areata are yellow dots, black

dots, broken hairs, tapering hair (exclamation

marks), and short vellus hairs (Mane et al, 2011; Inui

et al, 2008). In the remission phase, the white

perihilar sign might be found, and short vellus hair

becomes more prominent (Jha et al, 2017).

However, the proportion of these diagnostic features

might vary according to the literature. Therefore, we

would like to report the use of dermoscopy as a

diagnostic and evaluation tool in the case of

childhood alopecia totalis.

2 CASE

An 8-year-old girl came with a chief complaint of

diffuse scalp hair loss since the age of 4.

Approximately four years ago, parents incidentally

found a single hair loss patch on the scalp without

any associated symptoms. The year after, the patient

developed total scalp hair loss following an episode

of high fever and upper respiratory tract infection.

Eyebrows, eyelashes, and other hair-bearing areas

were not involved. Eventually, hair regrowth

observed after resolution of fever and infection.

However, relapses were noted at least twice a year,

following episodes of high heat and

tonsillopharyngitis. The parents denied any

application of topical or oral medications before the

onset of disease. The patient has a history of rhinitis

allergic (atopy). However, no prior history of

autoimmune diseases and a family history of hair

loss were noted. Parents brought her to a

dermatologist, and unknown topical medications

were given with good response. However, two

months prior to a consultation, the patient developed

a recurrent episode of total scalp hair loss three

weeks after hospital admission due to typhoid fever.

Even hair regrow already been noted,the frequent

relapses and lack of self-confidence prompted this

consult.

In physical examination, the patient was in

good general condition and nutritional status.All of

the vital signs were within the normal range.

Dermatologic examination revealed skin-colored

smooth surface macule with diffuse hair loss,

decreased of terminal hairs, and predominance of

vellus and broken hairs on the scalp. Furthermore,

multiple discrete lenticular hypopigmented macules

with indistinct borders and fine scales also observed

on the face. Small superficial pitting and transverse

leukonychia on fingernails were also noted.

Eyebrows and eyelashes were intact (Figure 1a – e).

From the initial assessment, the differential

diagnosis for the hair loss, in this case, are alopecia

totalis, telogen effluvium, and

trichotillomania.Wood's lamp examination on

hypopigmented facial lesions did not show any

enhancement and skin scrapping with KOH 10%

also failed to show fungal elements. These findings

are compatiblewith pityriasis alba. Serology

examination showed typical results for ANA, anti-

dsDNA, free T3, T4, TSH, which ruled out possible

associated autoimmune conditions such as lupus,

and thyroid disease. Initial dermoscopy evaluation

on the scalp revealed yellow dots, black dots,

exclamation hairs, broken hairs, and short vellus

hairs (Figures 1f – g). Based on these findings, the

diagnosis of alopecia totalis was established.

Hence, the patient was given topical minoxidil

2% solution applied once a day. On follow up visit

two months after treatment initiation, scalp hair has

regrown with 3 – 4 cm length in most areas.The

dermoscopic examination also showed signs of hair

regrowth with an increasing proportion of terminal

hairs and significant reduction of broken and short

vellus hair. Exclamation hair, yellow dots, and black

dots were not seen anymore (Figures 2). Therefore,

the patient was advised to continue the medications

until the scalp hair fully regrows with proper

compliance, do regular monthly visit, and avoid

triggering factors such as fever and infection.

3 DISCUSSION

Alopecia totalis is a manifestation of alopecia areata

that is characterized by total scalp hair loss and

associated poor prognosis and treatment failure with

only less than 10% of patients achieved complete

resolution. (Jang et al.,2017) Only limited data are

available regarding alopecia areata in children.

Prevalence of childhood alopecia is around 11.1% in

a multiethnic community of Singapore. Even though

there is a slight male predominance, the proportion

of severe alopecia is found higher in female patients.

In the case of extensive alopecia, such as alopecia

totalis and universalis, the onset usually is more than

six months, and most of them have recurrent

episodes.(Tan et al.,2002) As an autoimmune

disease with multifactorial etiologies, alopecia areata

can be triggered by microtrauma or destruction of

hair follicles, bacteria or virus infection, and

emotional stress.(Ito,203)Clinical manifestation of

alopecia totalis commonly found as sudden onset of

asymptomatic total scalp hair loss on the healthy and

smooth skin surface with the characteristic

Dermoscopy as a Diagnostic and Evaluation Tools in Childhood Alopecia Totalis

431

Figures 1. Dermatologic examination revealed (a), (b) diffuse scalp hair loss with decreased of terminal hairs and

predominance of vellus and broken hairs, (c) intact eyebrows and eyelashes, (d) multiple hypopigmented macules with fine

scales on the face which are consistent with pityriasis alba,and (e) multiple small superficial pitting and transverse

leukonychia on fingernails.(f), (g) Initial dermoscopic examination showed yellow dots(black arrow), exclamation hairs

(blue arrow), black dots (black circle), broken hairs (red arrow), and short vellus hairs (green arrow).

Figures 2. Follow up visit two months after therapy initiation, (a), (b) hair regrowth was observed in most areas of the scalp.

(c) The dermoscopic evaluation also revealed significantly increased of terminal hairs, reduction of broken and short vellus

hairs, but exclamation hairs, yellow dots, and black dots not seen.

(d)

(e)

(a) (b) (c)

(f)

(g)

(a)

(b)

(c)

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432

exclamation hairs and sparing of white hairs. In some

cases, the onset might be following episodes of

partial alopecia. (Alkhalifah et al, 2010; Otberg et al.,

2012) Nail involvement found in 7 – 66% of cases,

with small superficial pitting as the most common

finding, followed by trachyonychia, Beau’s lines,

onychorrhexis, thinning or thickening of nails,

onychomadesis, koilonychia, punctate or transverse

leukonychia, and red lunulae. (Alkhalifah et al, 2010)

In childhood alopecia, 8.4% of patients developed

nail abnormalities, including pitting, trachyonychia,

and longitudinal ridging, which correlated with the

severity of the disease.(Tan et al.,2002) Furthermore,

history of atopy, autoimmune diseases such as

vitiligo, thyroid disease, lupus, and other conditions

are also associated with a higher incidence of

extensive alopecia (totalis and universalis).

(Alkhalifah et al, 2010)

Diagnosis of alopecia areata usually mostly made

based on clinical findings, and ancillary tests might

not be needed to confirm the diagnosis. However, in

some cases with diffuse hair loss, as seen in our

patients, the clinical appearance might be similar to

telogen effluvium and trichotillomania. (Alkhalifah

et al, 2010) Hence, histopathology evaluation from a

scalp biopsy is required to rule out other etiology.

The common histopathological findings are

generalized miniaturization, a marked increase in the

catagen and telogen hair follicles and peribulbar

lymphocytic infiltrate (a swarm of bees)as the

hallmark of the acute phase. These features help the

physician to confirm the diagnosis of alopecia

areata.

However, this invasive diagnostic method is not

favorable or routinely done, especially in children.

Therefore, there are many studies have done lately to

verify the diagnostic features of alopecia areata in

dermoscopy. In the literature, characteristic

dermoscopic findings of alopecia areata are yellow

dots, black dots, broken hairs, exclamation mark hair,

and short vellus hairs (Table 1).(Mane et al.,

2011;Inui et al., 2008;Mahmoudi et al., 2018)

Among these features, yellow dotscan be regarded as

a sensitive marker for alopecia areata due to its high

frequency.(Jha et al., 2017) According to Inui et al.,

yellow dots and short vellus hairs were the most

sensitive markers for the diagnosis, and black dots,

exclamation mark hairs, and broken hairs were the

most specific markers. (Inui et al., 2008)

Some

studies have tried to correlate these dermoscopic

findings with disease activity and severity.

Guttikonda et al. found that among those features,

black dots, broken hairs, and exclamation mark hairs

are correlated with disease activity.(Guttikonda et al.,

2016) Furthermore, black dots and yellow dots

correlated positively with the severity of the alopecia

areata. (Inui et al., 2008;Guttikonda et al., 2016)

On the other hand, short vellus hairs correlated

negatively with either disease activity or severity.

This feature is commonly found in patients under

treatment and indicates an early sign of disease

remission. Regrowth of short vellus hair after surgery

can be seen in dermoscopy even before they can be

perceived by the naked eye.(Guttikonda et al., 2016)

Table 1. Dermoscopic features of alopecia areata

Findings Definition Frequency

Yellow dots Round or polycyclic yellow to yellow-pink dots that represent distended

follicular infundibula filledwith sebum and keratin remnants

63,7 – 89,6%

Black dots The remnant of broken hair shafts inside follicular ostia 40,9 – 78,4%

Exclamation mark hair Broken hairs that tapered toward follicles (diameter of proximal hair

follicle < distal)

31 – 66,6%

Short vellus hair Thin, unpigmented hairs with length ≤10 mm may demonstrate early

disease remission

12,1 – 31,7%

Broken hair Fracture of dystrophic hair shafts or rapid regrowth of hairs that formerly

manifested as blackdots

9,5 – 55,4%

(references: Mahmoudi H, Salehi M, Moghadas S, Ghandi N, Teimourpour A, Daneshpazhooh M. Dermoscopic findings in

126 patients with alopecia areata: A cross-sectional study. International Journal of Trichology 2018;10(3):118-23.)

In contrast, other condition with prolonged

diffuse hair loss such as chronic telogen effluvium

did not show specific dermoscopic findings.

Diagnosis of telogen effluvium may be suspected

when empty hair follicles (sometimes appearing as

yellow dots) and short, dark, regrowing hairs with

standard thickness are present in the absence of the

characteristic features of other scalp disorders. In

trichotillomania, the presence of broken hair shafts

with different lengths and signs of plucking, such as

a bleeding point on the scalp may help to establish

the diagnosis. These findings are not usually seen in

alopecia areata and remain as the hallmark of this

psychiatric disorder.(Lacarrubba et al., 2015)

Dermoscopy as a Diagnostic and Evaluation Tools in Childhood Alopecia Totalis

433

Treatment of childhood alopecia areata has to be

pro-active due to its chronicity and high risk of

extensive involvement. However, only limited data

are available regarding treatment options in this

population. Topical medications such as

corticosteroid, minoxidil, anthralin, and

immunotherapy (diphenylcyclopropenone (DPCP),

squaric acid dibutyl ester (SADBE)) remains as first-

line therapy. The physician might use a combination

of 2 or 3 topicals as second-line therapy. Inadequate

data are available to support the use of systemic

treatment in childhood alopecia to prevent extensive

alopecia. Therefore, systemic medications remain

the last frontier in treatment options.(Cranwell et al.,

2018).

In our case, we found an 8-year-old girl with a 4-

year history of multiple episodes of sudden onset

total scalp hair loss which were triggered by fever

and infection. Nail abnormalities such as small

superficial pitting, transverse leukonychia, and

history of atopy (rhinitis allergic) and also a minor

feature of atopic dermatitis (pityriasis alba) are

found in this patient. Clinical findings of diffuse

scalp hair loss can be found not only in alopecia

totalis but also telogen effluvium and

trichotillomania. However, diagnosis of alopecia

totalis was established by characteristic dermoscopic

findings of yellow dots, black dots, exclamation

hairs, broken hairs, and short vellus hairs. Hence,

telogen effluvium could be ruled out. Furthermore,

trichotillomania was ruled out due to no signs of a

bleeding point on the scalp, and all the broken hairs

were within a similar length.

Sensitive markers for disease severity, black

dots, and yellow dots, are found in the initial

dermoscopy evaluation, which is compatible with

the clinical manifestation of extensive alopecia

totalis. Hence, our patient was immediately started

on topical minoxidil solution as the first-line therapy

in childhood alopecia. Within two months, scalp hair

has regrown in most areas and dermoscopic markers

for disease activity, including black dots, broken

hairs and exclamation mark hairs, were not found

anymore. Short vellus hairs, as the sign of remission

phase in alopecia, were found as the dominant

dermoscopic findings in the follow-up visit

andindicated good treatment response. These

findings are similar to several studies done

previously and prove the reliability of dermoscopy,

not only as a diagnostic tool but also for evaluation

of treatment.

4 CONCLUSION

Alopecia totalis in children is associated with long

duration, a severe progression of the disease, and

inadequate response to treatment. In the case with

diffuse scalp hair loss, the clinical findings might not

lead to a straightforward diagnosis of alopecia

totalis. Dermoscopy has been used to confirm a

diagnosis in which biopsy may not be visible. Black

dots, broken hairs, and exclamation mark hairs are

correlated with disease activity. While short vellus

hairs might be the early sign of remission, even the

hair regrowth sign has not been visible with naked

eyes. Therefore, dermoscopy is an advantageous and

practical non-invasive diagnostic and evaluation

method in childhood alopecia totalis.

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