|Year : 2018 | Volume
| Issue : 3 | Page : 269-271
Extensive childhood alopecia areata responding to combination of oral cyclosporine and corticosteroid therapy – clinical experience in four patients
Nibedita Patro1, Maitreyee Panda2, Sibasish Patro3, Madhuchhanda Mohapatra2
1 Department of Skin and VD, Hi-Tech Medical College and Hospital, Utkal University, Bhubaneswar, Odisha, India
2 Department of Skin and VD, IMS and SUM Hospital, SOA University, Bhubaneswar, Odisha, India
3 Department of Skin and VD, MKCG Medical College and Hospital, Brahmapur University, Brahmapur, Odisha, India
|Date of Web Publication||28-Jun-2018|
Dr. Nibedita Patro
Department of Skin and VD, Hi-Tech Medical College and Hospital, Utkal University, Bhubaneswar - 751 025, Odisha
Source of Support: None, Conflict of Interest: None
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
|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
|How to cite this URL:|
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 [serial online] 2018 [cited 2020 Jan 26];19:269-71. Available from: http://www.ijpd.in/text.asp?2018/19/3/269/217483
| 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|| |
A 6-year-old girl presented with a large patch of alopecia [Figure 1]a 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 time of presentation, she was already on treatment with topical corticosteroids and topical calcineurin inhibitors on and off for the past 5 months without any signs of hair regrowth. After routine evaluation of blood parameters, urinalysis, chest X-ray, 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 1]b of around 80% was seen at the end of 12 weeks of combination therapy.
|Figure 1: (a) Alopecia areata on vertex, (b) post 12 weeks of combination therapy|
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A 7-year-old boy presented with two patches of hair loss [Figure 2]a 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 size approximately 6 cm × 5 cm each on the occipital area. There was no associated scaling, follicular prominences, uneven hair length, or any pigmentary changes. Hence, a clinical diagnosis of AA under steroid therapy was made. After routine blood investigations, chest x-ray, urinalysis, and blood pressure measurement, the child was started on oral cyclosporine (4 mg/kg) therapy along with continuation of oral steroids in the same dose. No topical therapy was advised. The patient was monitored every 2 weeks, and the hair growth was seen around 40% within 4 weeks [Figure 2]b and 90% at the end of 16 weeks [Figure 2]c of combination therapy, based on visual analog scale (VAS).
|Figure 2: (a) Two patches of alopecia areata on occipital area, (b) post 4 weeks, (c) post 16 weeks of combination therapy|
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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 3]a and [Figure 3]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 pulse therapy, that is, 2 mg of betamethasone twice weekly, barring any topical. The child showed around 70% improvement at 12-week [Figure 3]c and [Figure 3]d follow-up when graded on VAS scoring.
|Figure 3: (a and b) Ophiasis pattern of alopecia areata, (c and d) post 12 weeks of combination therapy|
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A 5-year-old girl presented with extensive AA involving almost whole of the vertex area [Figure 4]a 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 4]b. 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 4]c was recorded on VAS scoring sparing a small patch of alopecia on which intralesional corticosteroid was administered.
|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|
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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 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. The main goal of treatment should involve cosmetically acceptable hair regrowth to improve the patients quality of life., 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. 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. 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. 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.
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.,, One meta-analysis of CsA use in atopic dermatitis suggests that the efficacy is same in both adults and children, whereas the tolerability is superior in pediatrics age group.
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
Conflicts of interest
There are no conflicts of interest.
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