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Year : 2022  |  Volume : 23  |  Issue : 1  |  Page : 98-100

Successful remission of early-onset pediatric diffuse systemic sclerosis with suprapharmacological pulse dosing of dexamethasone and cyclophosphamide: A case report

1 Department of Dermatology, Ims and Sum Hospital, Bhubaneswar, Odisha, India
2 Consultant Dermatology, Skin Tech City Clinic, Bhubaneswar, Odisha, India

Date of Submission04-Jun-2021
Date of Decision30-Oct-2021
Date of Acceptance26-Nov-2021
Date of Web Publication31-Dec-2021

Correspondence Address:
Monali Pattnaik
Department of Dermatology, IMS and SUM Hospital, K-8 Kalinga Nagar, Bhubaneshwar - 751 003, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.ijpd_79_21

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How to cite this article:
Panda M, Agarwal A, Pattnaik M, Jena AK. Successful remission of early-onset pediatric diffuse systemic sclerosis with suprapharmacological pulse dosing of dexamethasone and cyclophosphamide: A case report. Indian J Paediatr Dermatol 2022;23:98-100

How to cite this URL:
Panda M, Agarwal A, Pattnaik M, Jena AK. Successful remission of early-onset pediatric diffuse systemic sclerosis with suprapharmacological pulse dosing of dexamethasone and cyclophosphamide: A case report. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 Jan 16];23:98-100. Available from: https://www.ijpd.in/text.asp?2022/23/1/98/334680


Juvenile systemic sclerosis (JSS) is a rare chronic multisystem connective tissue disorder with an incidence of 0.27–0.50 per million per year.[1] It differs from adults in having a lower incidence of internal organ involvement, less specific autoantibody profile, and a better long-term outcome. Because of its rarity, the disease remains poorly understood and poses a diagnostic and therapeutic challenge for the treating dermatologist.

A 4-year-old girl presented with Raynaud's phenomenon and generalized skin tightening for 1 year with difficulty in swallowing solids more than liquids and episodes of regurgitation for 3 months. History of breathlessness on exertion was present for 1 month. Cutaneous examination revealed diffuse sclerodermoid skin with extensive salt and pepper pigmentation over the trunk (Barnett neck's sign) and back [Figure 1]. Mask-like face, restricted mouth opening, inability to retract the lower eyelid (Ingram sign), sclerodactyly, and finger and toe-tip scarring were present [Figure 2]. Movement at elbow and knee joint and ability to supinate and pronate was markedly impaired. Routine blood tests were within the normal limits. Pulmonary function tests showed a restrictive pattern. High-resolution computed tomography scan of thorax and abdomen revealed dilated distal end of the esophagus. Antinuclear antibody and anti-topoisomerase antibody were strongly positive. Skin biopsy was not carried out due to the parent's apprehension. A diagnosis of progressive systemic sclerosis (indurated phase going toward fibrotic stage) was made based on all findings. JSS score was 25 (out of 40) with Modified Rodnan Skin Score (MRSS) being 42 (out of 51) at baseline. Considering disease severity and the associated morbidity and mortality, aggressive therapy was warranted to arrest the ongoing fibrotic process. After much consideration, the patient was started on monthly pulsed doses of dexamethasone (50 mg) and cyclophosphamide (500 mg) along with interval weekly methotrexate (5 mg), after counseling the patient's parents. After about 3 pulsed doses, improvement in disease severity was noticed. By the end of the 6th pulse, significant improvement in the skin sclerosis and salt pepper pigmentation was visible. Joint mobility restriction was significantly reduced and gastrointestinal complaints improved substantially improving the patient's quality of life. Pulse therapy was stopped after the 9th pulse. MRSS at end of the 9th pulse was 12 [Figure 3]. Currently, the patient is on regular follow-up with oral methotrexate therapy in maintenance dose. No growth development delay was noted. No serious adverse effects occurred during therapy.
Figure 1: Diffuse scleroderma with widespread salt and pepper pigmentation over the truck and back. Baseline Modified Rodnan skin score of 42 and Juvenile systemic score of 25

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Figure 2: Mask like facies, reduced mouth opening, and Barnett neck sign; fingertip scarring; toe tip scarring

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Figure 3: After 9 pulsed doses of modified dexamethasone-cyclophosphamide therapy, significant reduction in skin thickening and salt pepper pigmentation was noted with Modified Rodnan Skin score being 12

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The use of pulsed dexamethasone-cyclophosphamide therapy has been described in adult systemic sclerosis with encouraging results and minimal adverse effects.[2] In a large retrospective study of JSS, pulse dexamethasone was reported to be successful and advocated to be considered in severe disease.[3] The mechanism postulated was the destruction of autoantibody-producing cells and subsequent failure to proliferate and regenerate. Iwata et al. have also reported successful use of cyclophosphamide pulse therapy in 3 cases (2 females) of JSS.[4]

Our patient presented with the severe disease along with features of two organ involvement (gastrointestinal and lung) and was thus considered for modified pulsed dosing of dexamethasone and cyclophosphamide. This combination in suprapharmacological dosing has not been attempted in patients with JSS to the best of our knowledge. An encouraging sustained remission was achieved both in terms of clinical and objective assessment.

The incidence of systemic sclerosis in childhood is very rare with only two cases being reported <5 years of age, adding to the uniqueness of our case.[5],[6],[7] Through our report, we would like to emphasize the importance of aggressive management in dealing with pediatric progressive systemic sclerosis to curb disease progression, prevent morbidity, and improve the quality of life of both the patient and the parents. Further large-scale studies are needed however to confirm the safety and efficacy of dexamethasone-cyclophosphamide pulse therapy in JSS.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Stevens AM, Torok KS, Li SC, Taber SF, Lu TT, Zulian F. Immunopathogenesis of juvenile systemic sclerosis. Front Immunol 2019;10:1352.  Back to cited text no. 1
Dogra D, Dogra N, Gupta G, Mushtaq S. Dexamethasone-cyclophosphamide pulse therapy in systemic sclerosis: A retrospective study from a tertiary care hospital. Indian J Dermatol Venereol Leprol 2017;83:494-6.  Back to cited text no. 2
[PUBMED]  [Full text]  
Bagri NK, Raj D, Kaur J, Punia H, Saini I, Lodha R, et al. Juvenile systemic sclerosis: Experience from a tertiary care center from India. Rheumatol Int 2017;37:1687-91.  Back to cited text no. 3
Iwata N, Miyamae T, Imagawa T, Katakura S, Mori M, Aihara Y, et al. Cyclophosphamide pulse therapy for pediatric systemic sclerosis. Ryumachi 2003;43:660-6.  Back to cited text no. 4
Arya S, Kushwaha RK, Bunkar ML, Jain S. Progressive systemic sclerosis in a child: A rare phenomenon. Indian J Paediatr Dermatol 2017;18:65-7.  Back to cited text no. 5
  [Full text]  
Mishra N, Shrestha D, Poudyal RB, Shiva Raj KC. Atypical presentation of scleroderma in infancy. Rheumatol Int 2012;32:1069-74.  Back to cited text no. 6
Sato S, Ishida W, Takehara K. A case of juvenile systemic sclerosis with disease onset at six months old. Clin Rheumatol 2003;22:162-3.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]


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