Definition of Active Morphea
Active morphea is characterized by the presence of inflammation and fibrosis of the skin and underlying tissues, manifesting as red, warm, swollen areas with sclerotic plaques in a person with morphea. 1, 2
Clinical Characteristics of Active Morphea
- Active morphea presents with inflammatory signs including erythema, warmth, and swelling in the affected areas, indicating ongoing disease activity 1, 2
- The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is recommended for standardized assessment of disease activity, which includes the Localized Scleroderma Skin Severity Index (LoSSI) for measuring activity 1
- Active morphea lesions typically appear as sclerotic plaques with surrounding erythema or violaceous borders, indicating the inflammatory phase of the disease 2, 3
- The inflammatory phase precedes the development of fibrosis and represents the period when the disease is most responsive to treatment 2, 3
Diagnostic Considerations
- Skin biopsy should be taken from the most active sclerotic area when there is diagnostic uncertainty, atypical features, or features mimicking other conditions 1
- Active morphea must be distinguished from inactive or "burned out" morphea, which lacks inflammatory signs but may still show fibrosis and tissue damage 2, 4
- Regular clinical assessment using validated tools like LoSCAT is essential for monitoring disease activity and guiding treatment decisions 1
- The active phase of morphea can lead to permanent cosmetic and functional sequelae if not properly identified and treated early 2, 4
Clinical Implications of Active Disease
- Active, potentially disfiguring or disabling forms of morphea require prompt treatment with systemic medications, typically methotrexate combined with systemic corticosteroids during the initial inflammatory phase 1
- The active phase represents a critical window for therapeutic intervention, as treatment during this period may prevent or minimize permanent tissue damage 2, 3
- Active disease may progress to involve not only the skin but also underlying tissues including fascia, muscle, bone, and in some cases, the central nervous system 2
- Failure to recognize and treat active morphea can result in significant functional limitations, especially in linear morphea affecting the extremities or face 5
Monitoring Active Disease
- Regular assessment of disease activity is crucial, with treatment typically maintained for at least 12 months after achieving clinical improvement before considering tapering 1
- Reactivation of disease can occur, requiring vigilant monitoring even after apparent resolution of active inflammation 2, 3
- The transition from active to inactive disease is gradual and may be difficult to determine clinically, often requiring serial evaluations 4, 3
- Active disease monitoring should include assessment for potential extracutaneous manifestations, particularly in more severe forms of morphea 2, 5