Guideline sources The following summarized guidelines for the evaluation and management of localized scleroderma are prepared by our editorial team based on guidelines from the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019), the Pediatric Rheumatology European Society (PReS 2018), and the European Dermatology Forum (EDF 2017). 1 2 3 Guidelines 1. Classification and risk stratification Severity assessment: Use the LoSCAT clinical score for the severity assessment of localized scleroderma. Obtain photodocumentation of clinical lesions. E https://web.pathway.md/diseases/recvNKbo6Jn2laaZa 1/5 6/29/23, 5:54 AM Localized scleroderma Pathway Consider obtaining ultrasound scanning, cutometer, durometer, thermography, laser doppler flowmetry or using the computerized skin score to evaluate disease activity and clinical course of localized scleroderma over time. E 2. Diagnostic investigations Physical examination: assess for uveitis and arthritis in patients with linear localized scleroderma affecting the joints. E Show 2 more Laboratory testing: obtain blood tests including blood differential and serum biochemistry in patients with localized scleroderma before initiating systemic therapy. Do not obtain antinuclear antibodies and Borrelia testing routinely. E Show 2 more Diagnostic imaging: Consider obtaining MRI or CT to help with surgical planning and to detect muscle or bone involvement. E Obtain MRI of the skull to exclude brain affection in patients with linear localized scleroderma 'en coup de sabre' or progressive facial hemiatrophy. E 3. Diagnostic procedures Skin biopsy: consider performing skin biopsy in patients with inconclusive clinical presentation. E Show 2 more 4. Medical management Topical therapy: consider offering topical corticosteroids (high-potent corticosteroids for up to 4 weeks, mid-potent corticosteroids for up to 12 weeks) in the active stage of patients with limited types of localized scleroderma. Consider offering an occlusive application to increase the efficacy. Conduct longer treatment as interval therapy. E Show 2 more Disease-modifying antirheumatic drugs: initiate methotrexate for at least 12 months as first-line therapy in patients with subtypes of localized scleroderma with severe skin affection or musculoskeletal involvement. Consider reducing the dose after the first signs of clinical improvement. E Systemic corticosteroids: Initiate systemic corticosteroids, if not contraindicated, concomitantly with DMARDs in the active stage of disease, especially in severe cases (linear or deep localized scleroderma) or in cases with extracutaneous involvement. E https://web.pathway.md/diseases/recvNKbo6Jn2laaZa 2/5 6/29/23, 5:54 AM Localized scleroderma Pathway Consider initiating mycophenolate mofetil as an alternative option in cases of failure or contraindications to methotrexate. E 5. Nonpharmacologic interventions Physical therapy: offer physiotherapy and manual therapy in addition to topical and systemic therapy in all types of localized scleroderma resulting in restrictions of motion. Offer massage and lymphatic drainage concomitantly in sclerotic types of localized scleroderma. E 6. Therapeutic procedures Phototherapy: offer medium-dose UV A1 as the first choice of phototherapy in patients with limited types of localized scleroderma. Consider offering bath- or cream-PUVA phototherapy as alternative options. E 7. Surgical interventions Indications for surgery: Perform functionally indicated surgical interventions in the inactive stage of disease and concern patients with linear localized scleroderma. E Consider performing plastic surgery procedures for linear localized scleroderma 'en coup de sabre' and progressive facial hemiatrophy. E 8. Specific circumstances Pediatric patients, physical examination: As per SHARE initiative 2019 guidelines, obtain a careful evaluation with a complete joint examination, including the TMJ, at diagnosis and during follow-up in all patients with juvenile localized scleroderma. B Show 2 more As per EDF 2017 guidelines, assess for uveitis and arthritis in patients with juvenile localized scleroderma affecting the head (localized scleroderma 'en coup de sabre' or progressive facial hemiatrophy). E Pediatric patients, diagnostic imaging: As per SHARE initiative 2019 guidelines: Consider obtaining MRI to assess musculoskeletal involvement in patients with juvenile localized scleroderma, especially when the lesion crosses the joint. C Obtain MRI of the head at diagnosis in all patients with juvenile localized scleroderma involving the face and head, with or without signs of neurological involvement. B https://web.pathway.md/diseases/recvNKbo6Jn2laaZa 3/5 6/29/23, 5:54 AM Localized scleroderma Pathway Pediatric patients, severity assessment: as per SHARE initiative 2019 guidelines, use the LoSSI (a part of the LoSCAT) to assess activity and severity in patients with juvenile localized scleroderma. B Show 3 more Pediatric patients, setting of care: As per SHARE initiative 2019 guidelines, refer all pediatric patients with suspected localized scleroderma to a specialized pediatric rheumatology center. B As per EDF 2017 guidelines, ensure a multidisciplinary approach (including dermatologists and rheumatologists) in pediatric patients with linear or mixed types of localized scleroderma because of the high rate of extracutaneous involvement. E Pediatric patients, topical therapy: as per SHARE initiative 2019 guidelines, consider offering topical imiquimod to decrease skin thickening of circumscribed morphea. C Pediatric patients, DMARDs: as per SHARE initiative 2019 guidelines, initiate oral or SC methotrexate at 15 mg/m /week in all patients with active, potentially disfiguring or disabling forms of juvenile localized scleroderma. B Show 2 more Pediatric patients, systemic corticosteroids: as per SHARE initiative 2019 guidelines, consider initiating systemic corticosteroids concomitantly with methotrexate, or an alternative DMARD, in the active inflammatory phase of juvenile localized scleroderma. C Pediatric patients, phototherapy: as per SHARE initiative 2019 guidelines, consider offering medium-dose UV A1 phototherapy to improve skin softness in isolated (circumscribed) morphea lesions. B Pediatric patients, serial clinical assessment: as per SHARE initiative 2019 guidelines, obtain a careful evaluation with a complete joint examination, including the TMJ, during follow-up in all patients with juvenile localized scleroderma. B Show 2 more 9. Follow-up and surveillance Serial clinical assessment: arrange clinical follow-up visits at least once a year in patients with localized scleroderma with a high risk for recurrences after successful treatment. E Clinical findings Symptoms Past medical history Arthralgia Alopecia areata Fatigue Diabetes mellitus type 1 Myalgia Multiple sclerosis Psoriasis https://web.pathway.md/diseases/recvNKbo6Jn2laaZa 4/5 6/29/23, 5:54 AM Localized scleroderma Pathway Rheumatoid arthritis Head and neck exam SLE Coup de sabre Systemic sclerosis Vitiligo Integument exam Musculoskeletal exam Contractures Erythematous macules Arthritis Hyperpigmented skin patches Lab findings Skin atrophy Skin induration ESR Skin plaques Skin striae Serological findings Violaceous plaques ANA Hematological findings RF anti-Scl70 antibodies blood eosinophils anti-cardiolipin antibodies anti-centromere antibodies anti-deoxyRNA topoisomerase II alpha anti-double stranded deoxyRNA antibodies anti-histone antibodies anti-phospholipid antibodies References 1. Francesco Zulian, Roberta Culpo, Francesca Sperotto et al. Consensus-based recommendations for the management of juvenile localised scleroderma. Ann Rheum Dis. 2019 Aug;78 8 1019 1024. Open 2. Tam s Constantin, Ivan Foeldvari, Clare E Pain et al. Development of minimum standards of care for juvenile localized scleroderma. Eur J Pediatr. 2018 Jul;177 7 961 977. Open 3. R Knobler, P Moinzadeh, N Hunzelmann et al. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1 localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol. 2017 Sep;31 9 1401 1424. Open https://web.pathway.md/diseases/recvNKbo6Jn2laaZa 5/5