What is the treatment for coup de Sabre morphea?

From the Guidelines

The treatment for coup de sabre morphea should involve a combination of systemic corticosteroids and methotrexate (MTX), with MTX being started at 15 mg/m²/week and continued for at least 12 months before tapering, as recommended by the most recent consensus-based guidelines 1.

Key Treatment Considerations

• Systemic corticosteroids may be useful in the active inflammatory phase of the disease, and should be started in combination with MTX 1.
• MTX is the preferred disease-modifying antirheumatic drug (DMARD) for the treatment of coup de sabre morphea, due to its proven safety and efficacy in the treatment of juvenile localised scleroderma 1.
• The treatment regimen should be individualized based on the particular subtype of disease, the site of lesions, and the degree of activity.
• Other treatment options, such as topical calcineurin inhibitors, UVA1 phototherapy, and immunosuppressants like mycophenolate mofetil, may be considered in certain cases, but the evidence for their use is less strong 1.

Treatment Goals

• The primary goal of treatment is to halt disease progression and minimize scarring.
• Treatment should aim to suppress the abnormal immune response and inflammation that drives excessive collagen production and fibrosis in affected skin and underlying tissues.
• Physical therapy may be helpful in maintaining function if the condition affects mobility.

Important Considerations

• Treatment duration typically continues for 12-24 months or until disease stabilization occurs.
• Prolonged remission off medication is more likely to occur in patients treated for more than 12 months after achieving clinical remission on medication 1.
• The use of systemic corticosteroids and MTX should be carefully monitored and adjusted based on response, due to the potential for side effects and toxicity.

From the Research

Treatment Options for Coup de Sabre Morphea

The treatment for coup de sabre morphea, a form of localized scleroderma, can vary depending on the severity and progression of the disease. Some of the treatment options include:

• Methotrexate, which is often considered the first-line treatment 2, 3, 4, 5, 6
• Systemic corticosteroids, which can be used in combination with methotrexate 3, 5, 6
• Phototherapy, such as UVA1 or PUVA therapy 4, 5
• Mycophenolate mofetil, which can be used as an alternative to methotrexate 4, 5
• Hydroxychloroquine, which can be used in some cases 4
• Abatacept, tocilizumab, and cyclosporine, which have been used in some studies 4
• Platelet-rich plasma (PRP) injections, which can be a valid and safe alternative to traditional treatments 2
• Reconstructive surgery, which can be used to improve the cosmetic appearance of the lesions 4

Considerations for Treatment

When considering treatment for coup de sabre morphea, it's essential to take into account the individual patient's needs and the severity of the disease. Some factors to consider include:

• The age of the patient, as the disease can affect children and adults differently 3, 4
• The location and extent of the lesions, as this can impact the choice of treatment 2, 5
• The presence of extracutaneous symptoms, such as neurologic or ophthalmologic findings 3, 5
• The potential side effects of treatment, as some medications can have significant adverse effects 2, 6