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Case Report
Extensive Childhood Alopecia Areata Responding to Combination of
Oral Cyclosporine and Corticosteroid Therapy – Clinical Experience in
Four Patients
Abstract
Alopecia areata (AA) is a disease of unpredictable treatment outcome. Due to a great psychosocial
impact associated with the disease, multitudes of therapy have been tried. We recommend safe
consideration of oral cyclosporine in cases of extensive nonresponsive childhood AA.
Keywords: Alopecia areata, corticosteroid, cyclosporine A
Introduction
Alopecia areata (AA) is a common
nonscarring, autoimmune hair disorder
involving the hair‑bearing areas of the
body. The course of disease is unpredictable
having spontaneous remission and relapse,
and the plethora of treatment modalities
shows lack of adequate efficacy. Childhood
AA is a concern because of its bad
prognosis, associated atopy, psychological
impact, and poor response to therapy.
The autoimmune pathophysiology in
AA rationalizes the use of systemic
immunomodulators in severe cases.
Our clinical experience with the use of
oral cyclosporine A (CsA) along with
combination of oral corticosteroids in
extensive childhood AA not responsive to
conventional treatment, demonstrated good
clinical efficacy, and acceptable safety. We
are reporting four cases of extensive AA
responding satisfactorily to CsA plus oral
steroids with cosmetically acceptable hair
growth.
Case Reports
Case 1
A 6‑year‑old girl presented with a large
patch of alopecia [Figure 1a] of size
around 12 cm × 7 cm on vertex area of
scalp for 8‑month duration. Dermoscopic
evaluation showed black dots, yellow
dots, and broken and tapering hairs. She
was diagnosed clinically as AA. At the
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© 2018 Indian Journal of Paediatric Dermatology | Published by Wolters Kluwer - Medknow
Nibedita Patro,
Maitreyee Panda1,
Sibasish Patro2,
Madhuchhanda
Mohapatra1
Department of Skin and VD,
Hi‑Tech Medical College and
time of presentation, she was already on Hospital, Utkal University,
treatment with topical corticosteroids and 1
Department of Skin and VD,
IMS and SUM Hospital, SOA
topical calcineurin inhibitors on and off
University, Bhubaneswar,
for the past 5 months without any signs of
2
Department of Skin and VD,
hair regrowth. After routine evaluation of MKCG Medical College and
blood parameters, urinalysis, chest X‑ray, Hospital, Brahmapur University,
Brahmapur, Odisha, India
and blood pressure recording, she was
started on combination therapy with oral
cyclosporine (4 mg/kg) along with daily
dose of oral prednisolone (0.5 mg/kg)
tapering every 2 weeks. All the parameters
were monitored every 2 weeks. Remarkable
hair growth [Figure 1b] of around 80% was
seen at the end of 12 weeks of combination
therapy.
Case 2
A 7‑year‑old boy presented with two
patches of hair loss [Figure 2a] over
occipital area of scalp for 6‑month duration.
The patient was on treatment with daily
dose of oral prednisolone (1 mg/kg) for
the past 6 months. On examination, there
were two nonscarring alopecic patch of Address for correspondence:
size approximately 6 cm × 5 cm each on Dr. Nibedita Patro,
the occipital area. There was no associated Department of Skin and VD,
scaling, follicular prominences, uneven Hi‑Tech Medical College and
Hospital, Utkal University,
hair length, or any pigmentary changes. Bhubaneswar ‑ 751 025,
Hence, a clinical diagnosis of AA under Odisha, India.
steroid therapy was made. After routine E‑mail: nibeditapatro@gmail.
com
blood investigations, chest x‑ray, urinalysis,
and blood pressure measurement, the child
was started on oral cyclosporine (4 mg/kg) Access this article online
therapy along with continuation of oral
steroids in the same dose. No topical therapy Website: www.ijpd.in
was advised. The patient was monitored DOI: 10.4103/ijpd.IJPD_60_17
every 2 weeks, and the hair growth was Quick Response Code:
How to cite this article: Patro N, Panda M, Patro S,
Mohapatra M. Extensive childhood alopecia areata
responding to combination of oral cyclosporine and
corticosteroid therapy – clinical experience in four
patients. Indian J Paediatr Dermatol 2018;19:269-71.
269
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Patro, et al.: Cyclosporine in alopecia areata
seen around 40% within 4 weeks [Figure 2b] and 90% at
the end of 16 weeks [Figure 2c] of combination therapy,
based on visual analog scale (VAS).
Case 3
A male child of 4‑years age presented with sudden diffuse
hair loss on the scalp of 4‑month duration. On examination,
there was diffuse nonscarring alopecia of ophiasis
pattern [Figure 3a and b] involving frontal, bilateral
temporal, and occipital area. After excluding other causes
of nonscarring alopecia clinically, he was diagnosed as AA
and was confirmed with suggestive dermoscopic findings.
The child was already under treatment with high dose of
on and off oral steroids at the time of presentation to us.
Hence, after routine investigations, he was started on oral
cyclosporine (4 mg/kg) along with oral betamethasone
a b
Figure 1: (a) Alopecia areata on vertex, (b) post 12 weeks of combination
therapy
a b
c d
Figure 3: (a and b) Ophiasis pattern of alopecia areata, (c and d) post 12
weeks of combination therapy
270 Indian Journal of Paediatric Dermatology | Volume 19 | Issue 3 | July-September 2018
pulse therapy, that is, 2 mg of betamethasone twice
weekly, barring any topical. The child showed around 70%
improvement at 12‑week [Figure 3c and d] follow‑up when
graded on VAS scoring.
Case 4
A 5‑year‑old girl presented with extensive AA involving
almost whole of the vertex area [Figure 4a] and left temporal
area of the scalp of 5 months duration. She was started on oral
betamethasone pulse therapy (2 mg‑twice weekly). Minimal
response was seen at the end of 8 weeks [Figure 4b]. Hence,
she was considered for oral CsA (4 mg/kg) along with
continuation of oral corticosteroids after due investigations.
After 12 weeks of combination therapy, 75% improvement
in hair growth [Figure 4c] was recorded on VAS scoring
sparing a small patch of alopecia on which intralesional
corticosteroid was administered.
Cyclosporine was tapered at the rate of 1 mg/kg every
4 weeks in each of the cases and on reaching the dose of
1 mg/kg it was continued for another 2 months. One‑year
follow‑up of three cases showed mild recurrence of
lesions on stopping therapy after gradual tapering and
oral cyclosporine was restarted at a lower dose in all of
them along with addition of topical medications. Case 1
showed persistent hair growth at the end of 1 year after
stopping oral medications and was maintained only on
topicals. No alarming side effects of cyclosporine requiring
discontinuation of the drug were seen in any of our cases
except one child developed mild anemia, which was
managed with oral iron supplementation.
Discussion
Clinically, AA most commonly presents as
well‑circumscribed patchy hair loss over the scalp. On the
a b c
Figure 2: (a) Two patches of alopecia areata on occipital area,
(b) post 4 weeks, (c) post 16 weeks of combination therapy
a b c
Figure 4: (a) Extensive alopecia areata on vertex, (b) minimal response post
8 weeks of steroid pulse therapy, (c) post 12 weeks of combination therapy
[Downloaded free from http://www.ijpd.in on Tuesday, June 30, 2020, IP: 180.246.33.40]
Patro, et al.: Cyclosporine in alopecia areata
basis of larger patches and extensive involvement, it can be
AA multilocularis, alopecia totalis, and alopecia universalis.
Based on the pattern of involvement, it has been classified
into reticular, ophiasis, and sisipho types. Diagnosis is
mainly clinical, with the presence of exclamatory mark
hairs with positive hair‑pull test (>6 hairs) and positive
dermoscopy findings such as black dots, broken hairs,
and tapering hair helping in the diagnosis. Characteristic
histopathological findings of peribulbar and intrabulbar
lymphocytic infiltrate around anagen follicles, resembling
“swarm of bees” in acute phase, high ratio of catagen/
telogen hair follicles in subacute phase and follicular
miniaturization in chronic phase establishes the disease.[1]
The main goal of treatment should involve cosmetically
acceptable hair regrowth to improve the patients quality of
life.[2,3] In childhood AA, the biggest concern is counseling
of the parents regarding the disease course, prognosis, and
poor treatment response.
The pathogenesis is still unclear. The HLA‑DQB1*0301
and HLA‑DRB1*1104 MHC class II alleles are widely
associated with AA.[4] In genetically predisposed
individuals, there is an autoimmune process in the hair
follicles involving the loss of immune privilege mechanism
with upregulation of MHC expression and attack by
cytotoxic CD8+ cells.[4] In childhood AA where the disease
onset is before the age of 15 years, a positive family history
is strongly associated.
In recent years taking into account the patient’s compliance
and associated pain in treatment, oral cyclosporine has
been used in a vast array of autoimmune diseases. It has
been included in the third‑line therapeutic options in the
treatment ladder of AA. CsA inhibits the transcription of
interleukin 2 mRNA, thereby blocking proliferation of
T‑cells. It also suppresses interferon‑gamma production.
Moreover, its documented adverse effect of hypertrichosis
by prolongation of anagen phase of hair growth cycle
can add to its use in AA.[5] However, there is conflicting
opinion regarding efficacy of the drug in AA, and it takes
a back step while considering its side effects and high
relapse rate.[6]
The side effect profile of CsA includes hypertension,
nephrotoxicity, and immune suppression. Safety and
efficacy of CsA in pediatric age group have been
documented in many studies on atopic dermatitis and
psoriasis. Attributing to decreased sensitivity to CsA,
higher clearance and decreased bioavailability of the drug,
children are less susceptible to CsA‑induced nephropathy
than adults.[7‑9] One meta‑analysis of CsA use in atopic
dermatitis suggests that the efficacy is same in both
Indian Journal of Paediatric Dermatology | Volume 19 | Issue 3 | July-September 2018
adults and children, whereas the tolerability is superior in
pediatrics age group.[9]
In our cases, although the response to therapy with
cyclosporine plus corticosteroids was not sustained after
stopping the medications, it can be aptly concluded that in
cases of extensive childhood AA with delusive prognosis,
anxious parents, elaborate side effects of long‑term oral
corticosteroids, and minimal response to conventional
therapy, cyclosporine should be considered as a first‑line
therapy with a much safer drug profile, especially in
children.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and
other clinical information to be reported in the journal. The
patients understand that their names and initials will not
be published and due efforts will be made to conceal their
identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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