What is the recommended treatment approach for morphea (localized scleroderma)?

Treatment of Morphea (Localized Scleroderma)

All patients with active, potentially disfiguring or disabling morphea should be treated with methotrexate 15 mg/m²/week (oral or subcutaneous) combined with systemic corticosteroids during the initial inflammatory phase. 1, 2, 3

Treatment Algorithm Based on Disease Subtype and Severity

Circumscribed Superficial Morphea (Limited Lesions)

• Topical therapies are sufficient for limited, superficial circumscribed lesions that are primarily of cosmetic concern 1, 2
• Topical imiquimod reduces skin thickening by upregulating interferons that inhibit collagen production 1, 2, 3
• Medium-dose UVA1 phototherapy improves skin softness and reduces thickness, though it requires prolonged maintenance with cumulative radiation exposure concerns 1, 2, 3
• Topical calcipotriene or tacrolimus can be used for limited, superficial, inflammatory lesions 4

Linear, Deep, Generalized, or Pansclerotic Morphea (Severe Disease)

First-Line Systemic Therapy:

• Methotrexate 15 mg/m²/week (oral or subcutaneous) is the cornerstone of treatment 1, 2, 3
• Systemic corticosteroids must be started simultaneously with methotrexate as bridge therapy during the initial 2-3 months 1, 2, 3
• Two accepted corticosteroid regimens: oral prednisone 1-2 mg/kg/day for 2-3 months with gradual tapering, or pulsed intravenous methylprednisolone 30 mg/kg 3
• Methotrexate must be maintained for at least 12 months after achieving clinical improvement before considering tapering to prevent relapse 1, 2, 3

Second-Line Therapy for Refractory or Intolerant Patients:

• Mycophenolate mofetil 500-1000 mg/m² is recommended for patients who are methotrexate-refractory or methotrexate-intolerant 1, 2, 3
• Biologics (TNF or IL-6 inhibitors) can be considered for severe recalcitrant disease, though high-level evidence is lacking 1, 3

Critical Timing and Monitoring

Therapeutic Window

• Treatment must not be delayed as patients need to be treated in the "therapeutic window" before significant irreversible fibrosis develops 3
• This is particularly crucial in linear scleroderma affecting the face or extremities in children, where functional and cosmetic outcomes can be severely compromised 5, 3

Assessment Tools

• Use the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) for standardized monitoring 5, 1, 2
• LoSCAT includes the LoSSI (Localized Scleroderma Skin Severity Index) for activity assessment and LoSDI (Localized Scleroderma Skin Damage Index) for damage assessment 5, 1

Monitoring for Side Effects

• Regular monitoring for methotrexate side effects is essential, including nausea, headache, and transient hepatotoxicity 1, 2

Specialized Referral and Multidisciplinary Evaluation

• All patients with suspected morphea should be referred to a specialized rheumatology center (pediatric rheumatology for children) given the disease rarity of 3.4 cases per million children annually 5, 2, 3
• Patients with linear scleroderma involving the face and head require MRI of the head at diagnosis to assess for neurological involvement 5
• Ophthalmological assessment, including screening for uveitis, is mandatory at diagnosis for patients with facial/scalp lesions 5
• Orthodontic and maxillofacial evaluation is required for all patients with face and head involvement 5

Common Pitfalls to Avoid

• Inadequate treatment duration is the most common pitfall - treatment must continue for at least 12 months after clinical improvement to prevent relapse, as recurrence rates are 25-48% in the first years after discontinuation 2, 3
• Underestimating the importance of aggressive treatment leads to significant physical and psychological morbidity 1
• Premature discontinuation of corticosteroids before methotrexate takes effect (minimum 2-3 months of bridge therapy required) 3
• Failing to recognize that disease activity, severity, progression, and depth must all guide therapeutic decisions 4

Treatment Withdrawal

• Methotrexate or alternative disease-modifying drugs should be withdrawn only once the patient is in remission and off steroids for at least 1 year 1
• Long-term follow-up is mandatory given the high recurrence rate 2