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Localized scleroderma

Key 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).
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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.
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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.
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2.Diagnostic investigations

Physical examination: assess for uveitis and arthritis in patients with linear localized scleroderma affecting the joints.
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  • Laboratory testing

  • Diagnostic imaging

3.Diagnostic procedures

Skin biopsy: consider performing skin biopsy in patients with inconclusive clinical presentation.
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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.
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  • DMARDs

  • Systemic corticosteroids

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.
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6.Therapeutic procedures

Phototherapy: offer medium-dose UVA1 as the first choice of phototherapy in patients with limited types of localized scleroderma. Consider offering bath- or cream-PUVA phototherapy as alternative options.
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7.Surgical interventions

Indications for surgery
Perform functionally indicated surgical interventions in the inactive stage of disease and concern patients with linear localized scleroderma.
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Consider performing plastic surgery procedures for linear localized scleroderma 'en coup de sabre' and progressive facial hemiatrophy.
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8.Specific circumstances

Pediatric patients, physical examination, SHARE initiative: 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.
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  • Pediatric patients (diagnostic imaging)

  • Pediatric patients (severity assessment)

  • Pediatric patients (setting of care)

  • Pediatric patients (topical therapy)

  • Pediatric patients (DMARDs)

  • Pediatric patients (systemic corticosteroids)

  • Pediatric patients (phototherapy)

  • Pediatric patients (serial clinical assessment)

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.
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