Characteristics of the Patients

Patient 1Patient 2
Age at onset of contractures/sex8 y/male5 y/male
Joints involvedMild limitations of PIP, DIP, wrists, elbows, hips, knees, anklesModerate limitations of PIP, DIP, wrists, hips, knees, ankles, cervical spine
Time from onset of polyarthritis and onset of morphea7 mo13 mo
Type of morphea3 superficial plaques (pubis, back, leg)5 superficial plaques (neck, axilla, pubis, groin)
Eosinophilia/elevated CPKNo/noNo/no
AutoantibodiesANA, anti-SSA, anti-SSB, anti-RNP, anti-Scl70: negativeANA and RF: negative
Wrist MRI with gadolinium infusionMild synovial thickening with slight enhancement of synovial membrane after gadolinium infusionNo synovial thickening, slight enhancement of synovial membrane after gadolinium infusion
No joint effusion, sparse intraosseous edema without bone erosionNo joint effusion, no bone abnormalities
Skin biopsyDermal fibrosis, mild lymphocytic infiltrate, horizontalization of elastic fibers and adnexa rarefactionDermal fibrosis, sparse inflammatory infiltrate
Synovial biopsyDense fibrosis with very sparse inflammatory infiltratesNot done
  • ANA, antinuclear antibodies; CPK, creatinine phosphokinase; CRP, C reactive protein; DIP, distal interphalangeal joint; ESR, erythrocyte sedimentation rate; NSAID, nonsteroidal antiinflammatory drug; PIP, proximal interphalangeal joint; RF, rheumatoid factor; RNP, ribonucleoprotein; SSA and SSB, Sj√∂gren syndrome A and B.