What is the treatment for morphea (localized scleroderma)?

Treatment for Morphea (Localized Scleroderma)

Treatment for morphea should be tailored based on the type, extent, and depth of lesions, with circumscribed morphea typically requiring only topical treatments while more extensive or deep forms necessitate systemic therapy with methotrexate and corticosteroids. 1

Classification and Assessment

• Morphea (localized scleroderma) should be classified based on subtype, extent, and depth to guide treatment decisions 1, 2
• Patients with suspected morphea should be referred to specialized centers for comprehensive evaluation 2
• The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is recommended for standardized assessment of disease activity and damage 2, 3
• Skin biopsy from the most active sclerotic area is recommended when diagnostic uncertainty exists or atypical features are present 2

Treatment Algorithm

For Circumscribed Morphea (Limited, Superficial Lesions)

• Topical treatments are generally sufficient as these lesions are usually of cosmetic concern only 1
• Options include:
  • Topical corticosteroids (class IV) 4
  • Topical calcipotriene 5, 3
  • Topical tacrolimus 5, 3
  • Topical imiquimod - shown to decrease skin thickening by upregulating interferons that inhibit collagen production 1
• Phototherapy options:
  • Medium-dose UVA1 therapy is effective in improving skin softness and reducing thickness in adults 1
  • Narrowband UVB is appropriate for progressive or widespread superficial dermal lesions 5
  • Broadband UVA/UVA-1 is appropriate for deeper dermal lesions 5

For Linear, Deep, Generalized or Pansclerotic Morphea

• Systemic therapy is indicated for:

  • Linear morphea (especially in children) 1
  • Deep or function-impairing lesions 5
  • Rapidly progressive or widespread disease 5, 4

• First-line systemic therapy:

  • Methotrexate (MTX) at 15 mg/m²/week (oral or subcutaneous) 1, 2
  • Combined with systemic corticosteroids during the initial inflammatory phase 1, 2
  • Corticosteroid options include oral prednisone (1-2 mg/kg/day for 2-3 months with tapering) or pulsed high-dose intravenous methylprednisolone (30 mg/kg) 1
  • MTX should be maintained for at least 12 months after achieving clinical improvement before tapering 1, 2

• Second-line therapy (for MTX-refractory or MTX-intolerant patients):

  • Mycophenolate mofetil (MMF) at 500-1000 mg/m² 1, 2, 3
  • A retrospective study showed clinical improvement in all patients with severe refractory disease treated with MMF, mostly in combination with MTX 1

• For severe recalcitrant cases:

  • Biologics (including TNF or IL-6 inhibitors) have been used in some patients with resistant disease 1, 6, 3
  • Tocilizumab (IL-6R antibody) has shown promising results in pansclerotic morphea 6
  • Other options include JAK inhibitors and IVIG in severe cases 3

Monitoring and Duration of Treatment

• Regular clinical assessment using LoSCAT is recommended to monitor disease activity and response to treatment 2, 3
• MTX or alternative disease-modifying drugs should be withdrawn once the patient is in remission and off steroids for at least 1 year 1
• Close monitoring for medication side effects is essential, particularly with MTX which may cause nausea, headache, and transient hepatotoxicity 1

Special Considerations

• Phototherapy limitations in children include the need for prolonged maintenance therapy and increased risk of long-term effects such as skin aging and carcinogenesis 1
• Supportive measures like physiotherapy and psychological support are important components of management 3
• Orthopedic surgery and autologous fat transfer may be considered once the disease is inactive 3

Common Pitfalls

• Mistaking morphea for other conditions such as lichen sclerosus, especially in extragenital sites 2
• Inadequate treatment duration - treatment should be continued for sufficient time to prevent relapse 1
• Underestimating the importance of treating skin disease aggressively, as it is associated with significant physical and psychological morbidity 1