Linear localized scleroderma can affect adults with deep or extensive tissue involvement — spanning fat, fascia, muscle, joints, bones, or widespread skin. This clinical presentation warrants a structured systemic approach distinct from limited disease.
Adults aged 18 years or older with linear localized scleroderma who have severe skin and/or musculoskeletal involvement — including fat tissue, fascia, muscle, joints, bones, or widespread skin involvement. This depth and extent of involvement places patients in the subset requiring systemic management.
For this presentation, systemic therapy with methotrexate is the recommended first-line approach, with additional interventions incorporated during the active phase of disease.
Full dosing, sequencing, duration, and the complete therapeutic algorithm are available in the structured protocol.
The primary objective is reduction of skin sclerosis. A clinical response typically becomes apparent after at least 8 to 12 weeks of treatment.
DOI: 10.1111/ddg.15328
We recommend methotrexate (MTX) (off-label) as the first-line systemic therapy for LoS with severe skin and/or musculoskeletal involvement.
In subtypes with severe skin or musculoskeletal involvement, systemic therapy with methotrexate is recommended.
While the limited type of LoS (morphea) primarily occurs in adults, linear types are more prevalent in childhood.
We suggest considering systemic glucocorticosteroid therapy in addition to the systemic treatment with MTX during the active phase of LoS.
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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