What is the treatment for localized linear scleroderma?

Treatment for Localized Linear Scleroderma

For active, potentially disfiguring or disabling forms of linear scleroderma, methotrexate at 15 mg/m²/week combined with systemic corticosteroids is the recommended first-line treatment. 1

First-Line Treatment Approach

• Methotrexate (MTX) should be administered at 15 mg/m² weekly as an oral or subcutaneous dose 1, 2
• Systemic corticosteroids should be used concurrently during the initial inflammatory phase as "bridge therapy" for the first 3 months 1
• Two corticosteroid regimens are acceptable:
  • Oral prednisone at 1-2 mg/kg/day for 2-3 months with gradual tapering 1
  • Pulsed high-dose intravenous methylprednisolone (30 mg/kg) with various administration schedules 1, 3
• Once clinical improvement is achieved, MTX should be maintained for at least 12 months before considering tapering 1

Second-Line Treatment Options

• For MTX-refractory cases or MTX-intolerant patients, mycophenolate mofetil (MMF) at a dose of 500-1000 mg/m² is recommended 1, 2
• MMF has shown clinical improvement in severe refractory cases, though evidence is more limited than for MTX 1

Treatment for Circumscribed Morphea

• Circumscribed morphea (which differs from linear morphea) is generally of cosmetic concern only and can be managed with topical treatments 1
• Topical imiquimod has shown efficacy in decreasing skin thickening in isolated plaques 1
• Medium-dose UVA1 phototherapy may improve skin softness in circumscribed morphea lesions 1

Monitoring and Follow-up

• Regular assessment using validated tools such as the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is recommended 1, 2
• Monitor for MTX side effects, which are generally mild and include nausea, headache, and transient hepatotoxicity 1
• Consider withdrawing immunosuppressive treatment once the patient has been in remission and off steroids for at least 1 year 1

Emerging Therapies

• For resistant cases, other agents being used in clinical practice include tacrolimus, cyclophosphamide, and biologics (TNF or IL-6 inhibitors), though high-quality evidence is lacking 1
• Platelet-rich plasma injections have shown promise in some case reports but require further study 4

Important Considerations

• Linear scleroderma can cause permanent disability if not treated appropriately, making early aggressive treatment crucial 5
• The median time to response with MTX and corticosteroid therapy is approximately 3 months 5
• Disease may flare upon discontinuation of therapy, requiring reinstitution of treatment 5
• For pediatric patients, careful monitoring is essential due to potential growth-related complications of systemic corticosteroids 6

Treatment Algorithm

1. Confirm diagnosis (clinical evaluation, possibly skin biopsy from active sclerotic area)
2. Assess disease activity and extent using LoSCAT
3. For active, potentially disfiguring linear scleroderma:
   • Start MTX 15 mg/m²/week + systemic corticosteroids
   • Continue MTX for at least 12 months after clinical improvement
4. If inadequate response or intolerance to MTX:
   • Switch to MMF 500-1000 mg/m²
5. For circumscribed morphea only:
   • Consider topical treatments (imiquimod) or UVA1 phototherapy