Table 1 Working jLS Study Entry Criteria: Inclusion, Exclusion, and Active Disease criteria

Inclusion Criteria:

 1. Localized scleroderma diagnosed by a Pediatric Rheumatologist or Pediatric Dermatologist according to Padua Preliminary Classification criteria; these criteria exclude eosinophilic fasciitis [23]

 2. Fulfill active disease criteria:

  a. Either at least one item from Active disease criteria Group 1, or two from Group 2

 3. Moderate to high disease severity that warrants systemic therapy in the opinion of the treating physician

  a. Includes all subtypes that involve deeper tissue, extensive skin involvement, and/or extracutaneous involvement

 4. Age < 18 years at onset of disease

 5. Age < 21 years at onset of treatment

Exclusion criteria:

 1. Treated with systemic corticosteroids in the prior 2 weeks

 2. Treated with methotrexate or mycophenolate mofetil within the prior 3 months^a

 3. Another defined systemic rheumatic disease (e.g., systemic sclerosis)

 4. Intolerance to study medications

Working Active Disease criteria (used for Pilot Consensus Treatment Plan study)^a

Group 1:

 1. New lesion(s) within the prior 3 months, documented by clinician^a

 2. Extension of an existing lesion within the prior 3 months, documented by clinician^a

  a. Lesion extension observed in serial photographs or tracings, or detecting > 30% difference in lesion size (maximum length x width).

 3. Documentation of active or progressive deep tissue involvement^a

  a. Can be by clinical examination, photographs, MRI, or ultrasound

 4. Erythema of moderate or severe level in lesion or an erythematous lesion border

  a. Erythema scoring level based upon LS Scoring Atlas

 5. Violaceous lesion or border color

  a. Can range from lilac ring to deep violaceous color

Group 2:

 1. Patient or parent report of new lesion OR extension of existing lesion occurring within the prior 3 months^a

  a. This criterion ONLY applies for new patients (i.e., first visit to clinician’s office).

 2. Erythema of mild level

  a. Erythema scoring level based upon LS Scoring Atlas

 3. Moderate or severe induration of lesion border^a

  a. Assessed according to modified Rodnan Skin Scoring (mRSS) levels [15]

 4. Tactile warmth of the lesion

  a. Examiner appreciation of temperature difference based upon comparison to control site (unaffected contralateral site if available).

 5. Worsening hair loss in scalp, eyebrow, or eyelashes; documented by clinician^a

 6. Elevated creatine kinase level in the absence of other source^a

 7. Lesion biopsy showing active disease (based upon pathologist report. Typically would be presence of lymphocytes, plasma cells, eosinophils, or other white blood cell)