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LETTERS TO EDITOR
Year : 2016  |  Volume : 17  |  Issue : 2  |  Page : 156-158

Juvenile systemic sclerosis in 11-year-old male child responding to methotrexate


1 Department of Dermatology, MIMER Medical College, Talegaon, Maharashtra, India
2 Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

Date of Web Publication30-Mar-2016

Correspondence Address:
Mukherjee Samipa Samir
Bangalore Medical College and Research Institute, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.175654

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How to cite this article:
Gaikwad RP, Samir MS. Juvenile systemic sclerosis in 11-year-old male child responding to methotrexate. Indian J Paediatr Dermatol 2016;17:156-8

How to cite this URL:
Gaikwad RP, Samir MS. Juvenile systemic sclerosis in 11-year-old male child responding to methotrexate. Indian J Paediatr Dermatol [serial online] 2016 [cited 2019 Nov 19];17:156-8. Available from: http://www.ijpd.in/text.asp?2016/17/2/156/175654

Sir,

Scleroderma literally means hardening of the skin and is an auto immune condition of unknown origin. It is characterized by the presence of antibodies in the body, deposition of excess collagen in the dermis and can present in localized scleroderma (LS) and systemic sclerosis (SSc) forms. Systemic form of scleroderma is considered a severe disease generally affecting the adults whereas LC is more common in the pediatric population. Both forms are uncommon in children, with the estimated annual incidence of LS being 1–3/100,000 children [1] and that of SSc being 1/million children.[2] It becomes imperative to identify these cases, cause of their rarity and the profound impact they can have on the survival and quality of life of the patient. Herein, we report a case of an 11-year-old male child with SSc showing good response to oral methotrexate therapy.

An 11-year-old male, first child of nonconsanguineous marriage, full term normal delivery with an uneventful ante natal history came with complaints of discoloration of face, upper limbs, and trunk with altered skin texture since 6–8 months. The symptoms have been gradually progressing since then. History also revealed development of spontaneous ulceration over the digital tips and edema of the fingers. There was history of dyspnea on exertion and inability to consume adequate meals due to abdominal discomfort. There was no history dyspepsia and Raynaud phenomenon.

General and systemic examination revealed reduced chest expansion with normal physical and developmental milestones.

Cutaneous examination showed diffuse Addisonian pigmentation with ironing of the forehead skin, positive Ingram, and neck sign with reduced mouth opening, salt and pepper pigmentation were noted on the chest [Figure 1] and [Figure 2]. Binding down of skin was noted over the face, neck, and trunk along with upper limbs. Edema of all fingers of both hands was noted with digital tip ulcers and stellate scars [Figure 3]. Nail examination revealed ragged cuticles while the hair and mucosa were normal.
Figure 1: Binding down of skin with dyspigmentation and ichthyotic skin on upper trunk

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Figure 2: Ironing out of forehead skin, positive Ingram's sign and reduced mouth opening

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Figure 3: Digital tip ulcers

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Nail fold capillaroscopy showed dilated capillary loops and drop outs.

Routine blood investigations were within normal limits. Specific tests conducted for Scl-70 showed an increased titer. Radiological investigations for esophageal dysfunction and pulmonary involvements were within normal limits. Pulmonary function tests were normal.

Biopsy done from the bound down skin revealed increased collagenization of the dermis.

Thus based on the clinical, histopathology, and laboratory findings a diagnosis of diffuse cutaneous juvenile SSc was made as per the guidelines by Zulian et al.[3]

He was treated with 2.5 mg/week of oral methotrexate along with folic acid supplementation to which he showed marked improvement.


  Discussion Top


Scleroderma is unusual in children; however when present can manifest as localized form that is more common and the less common systemic scleroderma. Unfortunately, there is a significant delay in diagnosis, with an average 1.9–2.8 years for SSc [4] and 1.2–1.6 years for localized sclerosis (LS).[5] The approximate female to male ratio of pediatric SSc is 4:1 and for pediatric LS 2:1.[4],[5]

A number of distinguishing features have been noted between juvenile SSc and the adult counterpart.[6] The most common antibodies associated with pediatric scleroderma include Scl-70, anti-centromere antibody, anti-U1-ribonucleoprotein, and anti-Pm-Scl antibodies.

The cutaneous features of an adult onset SSc resemble that of the juvenile type. Musculoskeletal involvement in pediatric SSc is more than the adults.

Pulmonary involvement in progressive SSc in pediatric age group ranges from 30% to 70% and occurs in the form of interstitial lung disease, the severity of which can be predicted by the Scl-70 antibody levels. In our patient, although there was complaint of dyspnea the pulmonary function tests did not reveal any derangement.

Although, esophageal dysfunction is a complaint in most patients symptomatic dysphagia occurs in only half of them.[7]

Cardiac abnormalities such as pericarditis, left, or biventricular failure, or arrhythmias are common in pediatric SSc which was not noted in our patient.

Renal involvement heralds a guarded prognosis which was also absent in our patient.

He was managed with weekly methotrexate orally with which at the end of 2 months of treatment he reported an increased sense of well-being, improved mouth opening, reduction of breathlessness, and decrease in the binding down of skin. The other modalities of management being systemic corticosteroids, mycophenolate mofetil, and phototherapy using ultraviolet A, biological, intravenous immunoglobulins, and cyclophosphamide.

The prognosis in case of juvenile SSc depends on the visceral involvement and rate of progress of disease is not predictable, thereby posing a therapeutic challenge. Specific treatment recommendations for different organ manifestations have been agreed on by a consensus conference of international scleroderma experts, and were published in 2009.[8] Foeldvari et al. recently reported Kaplan–Meier survival rates at 10 years for children to be 98% versus 75% in adults.[9]

We herein report this case for its rarity in pediatric population, moreover in a male child and also stress upon the early diagnosis and management which improves the quality of life of the patient and the disease outcome.

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.

 
  References Top

1.
Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol 1997;24:73-80.  Back to cited text no. 1
    
2.
Pelkonen PM, Jalanko HJ, Lantto RK, Mäkelä AL, Pietikäinen MA, Savolainen HA, et al. Incidence of systemic connective tissue diseases in children: A nationwide prospective study in Finland. J Rheumatol 1994;21:2143-6.  Back to cited text no. 2
    
3.
Zulian F, Woo P, Athreya BH, Laxer RM, Medsger TA Jr, Lehman TJ, et al. The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum 2007;57:203-12.  Back to cited text no. 3
    
4.
Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino AV Jr, et al. Childhood onset systemic sclerosis: Classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol 2006;33:1004-13.  Back to cited text no. 4
    
5.
Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG, et al. Juvenile localized scleroderma: Clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 2006;45:614-20.  Back to cited text no. 5
    
6.
Blaszczyk M, Janniger CK, Jablonska S. Childhood scleroderma and its peculiarities. Cutis 1996;58:141-4, 148-52.  Back to cited text no. 6
    
7.
Singsen BH. Scleroderma in childhood. Pediatr Clin North Am 1986;33:1119-39.  Back to cited text no. 7
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8.
Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research Group (EUSTAR). Ann Rheum Dis 2009;68:620-8.  Back to cited text no. 8
    
9.
Foeldvari I, Nihtyanova SI, Wierk A, Denton CP. Characteristics of patients with juvenile onset systemic sclerosis in an adult single-center cohort. J Rheumatol 2010;37:2422-6.  Back to cited text no. 9
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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