Juvenile localised scleroderma management -International consensus recommendations

Following are the major recommendations:

Recommendations regarding diagnosis and assessment

All children with suspected localised scleroderma should be referred to a specialised paediatric rheumatology center.

• LoSSI, which is part of LoSCAT, is a good clinical instrument to assess activity and severity in JLS lesions and is highly recommended in clinical practice.


• LoSDI, which is part of LoSCAT, is a good clinical instrument to assess damage in JLS and is highly recommended in clinical practice.


• Infrared thermography can be used to assess the activity of the lesions in JLS, but skin atrophy can give false-positive results.


• A specialised US imaging, using standardized assessment and colour Doppler, may be a useful tool for assessing disease activity, the extent of JLS and response to treatment.


• All patients with JLS at diagnosis and during follow-up should be carefully evaluated with a complete joint examination, including the temporomandibular joint.


• MRI can be considered a useful tool to assess musculoskeletal involvement in JLS, especially when the lesion crosses the joint.


• It is highly recommended that all patients with JLS involving face and head, with or without signs of neurological involvement, have an MRI of the head at the time of the diagnosis.


• All patients with JLS involving face and head should undergo an orthodontic and maxillofacial evaluation at diagnosis and during follow-up.


• Ophthalmological assessment, including screening for uveitis, is recommended at diagnosis for every patient with JLS, especially in those with skin lesions on the face and scalp.


• Ophthalmological follow-up, including screening for uveitis, should be considered for every patient with JLS, especially in those with skin lesions on the face and scalp.

Recommendations regarding treatment

• Systemic corticosteroids may be useful in the active inflammatory phase of JLS. At the same time as starting systemic corticosteroids, MTX or an alternative DMARD should be started.


• All patients with active, potentially disfiguring or disabling forms of JLS should be treated with oral or subcutaneous methotrexate at 15 mg/m²/week.


• If acceptable clinical improvement is achieved, methotrexate should be maintained for at least 12 months before tapering.


• Mycophenolate mofetil may be used to treat severe JLS or MTX-refractory or MTX-intolerant patients.


• Medium-dose UVA1 phototherapy may be used to improve skin softness in isolated (circumscribed) morphoea lesions.


• Topical imiquimod may be used to decrease skin thickening of circumscribed morphoea.