# Scleroderma in Children > Scleroderma in children for NEET-PG Paediatrics: localised morphea and linear disease, methotrexate therapy, and juvenile systemic sclerosis differences. - Canonical URL: https://prepelephant.com/topics/neet-pg/paediatrics/scleroderma-children - Exam / course: NEET-PG · Subject: Paediatrics - Publisher: PrepElephant (https://prepelephant.com) — Prepared and reviewed by the PrepElephant Academic Review Team - First published: 2026-10-02 - Last updated: 2026-10-02 - How to cite: "Scleroderma in Children", PrepElephant, https://prepelephant.com/topics/neet-pg/paediatrics/scleroderma-children ## Direct answer In children, scleroderma usually means localised disease — morphea patches or linear bands — while juvenile systemic sclerosis, the multi-organ fibrosing disease, is rare and inverts the adult organ hierarchy. Localised scleroderma evolves through inflammatory (lilac ring, oedema) into sclerotic (ivory-hard centre) and atrophic phases; linear subtypes crossing joints or striking the face and scalp (en coup de sabre, with Parry-Romberg at the extreme) cause the real damage — limb growth arrest, contractures and facial deformity, not internal disease. Juvenile systemic sclerosis presents with Raynaud phenomenon in nearly all, then skin induration, involving gut and lungs more prominently than heart or kidney at diagnosis — where adult series lead with renal crisis. Methotrexate with corticosteroids is the mainstay for both; physiotherapy and contracture prevention matter as much as immunosuppression in the linear forms. ## What you must remember - **Classification split:** localised (circumscribed morphea, linear scleroderma, generalised morphea, pansclerotic) versus systemic sclerosis — children skew towards localised, the reverse of adult practice. - **Morphea lesion biography:** erythema and oedema with a violaceous lilac ring, hardening to an ivory or hyperpigmented centre bound down to deeper planes, leaving atrophy and hair loss; the active inflammatory edge is where biopsy and treatment decisions live. - **Linear subtype stakes:** bands crossing joints cause contractures and limb-length discrepancy from epiphyseal arrest; face and scalp involvement (en coup de sabre) may coexist with seizures and uveitis; Parry-Romberg facial hemiatrophy sits at the severe end. - **Juvenile systemic sclerosis picture:** Raynaud phenomenon in almost all (capillaroscopy shows dilated loops and dropout), progressive skin induration (modified Rodnan score), oesophageal reflux, interstitial lung disease (screening pulmonary function and computed tomography), rarer cardiac and renal involvement; antinuclear antibody positive in most, anti-Scl-70 and anti-centromere aid subtyping. - **First-line therapy:** methotrexate (about 15 mg per square metre weekly) with corticosteroids — pulsed methylprednisolone for active phases; mycophenolate as second agent; physiotherapy and splinting throughout. - **Systemic-sclerosis-specific care:** proton pump inhibitors for reflux, iloprost or calcium channel blockers for severe Raynaud with digital ulcers, ACE inhibitors immediately if renal crisis develops (rare), and infection vigilance on immunosuppression. - **Monitoring toolkit for localised disease:** serial photography with a ruler, thermography where available, activity scores, and limb length and girth comparison in linear disease — activity must be tracked to justify therapy. - **Course reality:** localised disease often burns out over three to five years leaving fixed damage; juvenile systemic sclerosis progresses slower but demands long-horizon surveillance. ## Working through the linear band An eight-year-old girl has a year-long tight, shiny band from the right thigh across the knee; the limb is subtly thinner, knee extension lags 20 degrees, a lilac halo rims the edge. First, subtype and activity: linear scleroderma crossing a joint, currently inflammatory (violaceous rim) — active disease is treatable; a burnt-out plaque is not. Second, stage the damage: limb length and girth, growth-plate assessment, range-of-motion documentation, MRI in deep or facial cases. Third, treat: methotrexate weekly with folate plus corticosteroid (pulses then taper) — the evidence-supported standard — continued a year past inactivity to prevent reactivation. Fourth, run the defence in parallel: daily stretching, night splints, physiotherapy, and growth monitoring with epiphysiodesis discussion if significant discrepancy develops. Fifth, screen for systemic crumbs: capillaroscopy, antinuclear antibody and a Raynaud history. Contrast the second patient, a thirteen-year-old with two years of Raynaud, tightening facial skin and new dysphagia: her workup — capillaroscopy, pulmonary function, chest computed tomography, echocardiography, antibodies — organises systemic therapy (methotrexate or mycophenolate, reflux control, Raynaud vasodilation) and long-horizon surveillance, a different grammar despite the shared name. ## Where students slip Localised disease is dismissed as cosmetic — linear scleroderma in a growing child is a limb-threatening disease of the growth plate, and methotrexate is the standard of care for active lesions, not a last resort. Second, quoting the adult complications script at children: renal crisis and severe cardiac disease are uncommon at juvenile presentation, whereas gastrointestinal and pulmonary involvement dominate — the hierarchy is inverted. Third, the lilac ring is missed as a sign of activity; treating an inactive atrophic lesion earns nothing, and the exam asks which lesion to treat. ## Frequently asked questions ### Which form of scleroderma is commonest in children? Localised scleroderma — morphea and linear subtypes — far outnumbering juvenile systemic sclerosis in paediatric rheumatology clinics. ### What is en coup de sabre? A linear band of the frontal scalp and face resembling a sabre cut, potentially associated with cranial involvement, seizures or eye disease, overlapping with Parry-Romberg facial hemiatrophy. ### What is the first-line systemic therapy for active localised scleroderma? Methotrexate weekly with corticosteroids (often pulses) continued well beyond clinical inactivity, paired with intensive physiotherapy. ### How does juvenile systemic sclerosis differ from adult disease? Children almost always present with Raynaud phenomenon, involvement skews to gut and lung rather than kidney and heart, and progression is slower — but interstitial lung disease remains the key long-term threat. ### Which sign marks active inflammation in a morphea lesion? A violaceous lilac ring surrounding the indurated plaque, often with warmth — the feature that justifies immunosuppressive treatment rather than observation.