Morphea, also known as localized scleroderma, is a rare autoimmune condition that primarily affects the skin and sometimes the underlying tissues. It is characterized by patches of hardened, thickened, and discolored skin resulting from the overproduction of collagen. While generally not life-threatening, morphea can significantly impact appearance and function, especially when lesions cross joints. The goals of therapy are to halt disease progression, soften existing lesions, and improve quality of life. Although there is no definitive cure, various treatments can effectively manage the condition and induce remission.
Topical Medications for Localized Morphea
Treatment for mild or limited morphea lesions often begins with topical medications applied directly to the affected skin. High-potency topical corticosteroids are frequently used as first-line agents due to their anti-inflammatory effects, helping to calm active patches. These are prescribed for a limited duration to mitigate the risk of local side effects, such as skin atrophy or thinning. Vitamin D analogs like calcipotriene may be recommended to help soften the hardened plaques, sometimes applied under occlusion to enhance penetration. Another option is topical calcineurin inhibitors, such as tacrolimus ointment, which suppresses local immune activity without the risk of steroid-induced skin thinning.
Light Therapy Options
When morphea is widespread or fails to respond to topical treatments, phototherapy becomes a next-step option. This treatment exposes the affected skin to specific wavelengths of ultraviolet (UV) light to suppress the immune processes driving the disease. Ultraviolet A-1 (UVA-1) phototherapy is effective, using longer wavelengths that penetrate the deep dermis where collagen accumulates. UVA-1 works by inducing programmed cell death in inflammatory T-cells and activating fibroblasts to produce MMP-1, an enzyme that breaks down excess collagen. Narrowband or broadband Ultraviolet B (UVB) is used for more superficial morphea.
Systemic Therapies for Advanced Cases
Systemic therapies are necessary for morphea that is rapidly progressing, widespread, or involves deeper tissues like fascia, muscle, or bone, such as linear morphea crossing a joint. The primary systemic treatment is often a combination of the immunosuppressant methotrexate and oral corticosteroids, given simultaneously. Methotrexate is typically administered weekly and continued for an extended period, often for at least a year after the disease becomes inactive. Oral corticosteroids, such as prednisone, are given for a short time to quickly reduce inflammation until methotrexate takes effect. If patients do not respond to methotrexate, mycophenolate mofetil may be used as an alternative.
Monitoring and Follow-Up Care
Long-term management requires regular monitoring to track disease activity, assess treatment effectiveness, and address complications. Clinicians often use standardized tools, such as the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), to objectively measure active inflammation and permanent damage. LoSCAT helps differentiate active disease, which requires treatment escalation, from inactive disease. Supportive care is important, especially for linear morphea affecting limbs. Physical and occupational therapy is prescribed to maintain a full range of motion and prevent joint contractures, while consistent use of emollients is encouraged to improve skin comfort and texture.