Treatment of Linear Scleroderma in Children (Under 18) with Severe Skin or Musculoskeletal Involvement
Clinical Scenario
This protocol applies to children and adolescents under 18 years of age with linear localized scleroderma who have severe involvement — affecting fat tissue, fascia, muscle, joints, or bones — or widespread skin changes. Linear forms of localized scleroderma are more prevalent in childhood than in adults. Early, potent systemic intervention is critical to prevent late complications including contractures, growth disturbances, and limb deformities.
Treatment Approach (Partial Overview)
Management centres on systemic disease-modifying therapy as the primary intervention. During the active phase, a systemic anti-inflammatory agent is added alongside it. When joint or muscle function is restricted, a physical and manual therapy component is incorporated. The full regimen — including agent sequencing, remission criteria, duration guidance, and escalation thresholds — is detailed in the complete protocol.
Treatment Goals
The primary objective is reduction of skin sclerosis. Clinical improvement should not be expected immediately — response typically becomes apparent after at least 8 to 12 weeks of sustained treatment.
References
DOI: 10.1111/ddg.15328
- While the limited type of LoS (morphea) primarily occurs in adults, linear types are more prevalent in childhood.
- However, in all other cases and for active linear LoS in childhood, potent systemic therapy should be initiated as early as possible to prevent potential late complications (contractures, growth disturbances, limb deformities, etc.).
- We recommend methotrexate (MTX) (off-label) as the first-line systemic therapy for LoS with severe skin and/or musculoskeletal involvement.
- We suggest considering systemic glucocorticosteroid therapy in addition to the systemic treatment with MTX during the active phase of LoS.
- We recommend physical therapy and manual therapy in all subtypes of LoS with restricted mobility (for example joint contracture, muscle imbalance [atrophy/hypotrophy]) and for the prevention of joint contracture in cases of skin involvement spanning the joint.
- When assessing the effectiveness of the chosen therapeutic concept, it should be noted that the reduction of sclerosis typically takes at least 8 to 12 weeks in most cases.
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