علاج تصلب الجلد الموضعي مع إشراك جلدي أو عضلي هيكلي حاد لدى المرضى دون 18 عامًا

References

DOI: 10.1111/ddg.15328

• In subtypes with severe skin or musculoskeletal involvement, systemic therapy with methotrexate is recommended.
• We recommend methotrexate (MTX) (off-label) as the first-line systemic therapy for LoS with severe skin and/or musculoskeletal involvement.
• 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.).
• A longer duration until the start of therapy is associated with a higher frequency of treatment failure.
• 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.