Case Report
An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis
Table 1
The EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies 4.
| Variable | Score points | Without muscle biopsy | With muscle biopsy |
| Age of onset | Age of onset of first symptom assumed to be related to the disease ≥18 years and <40 years | 1.3 | 1.5 | Age of onset of first symptom assumed to be related to the disease ≥40 years | 2.1 | 2.2 | Muscle weakness | Objective symmetric weakness, usually progressive, of the proximal upper extremities | 0.7 | 0.7 | Objective symmetric weakness, usually progressive, of the proximal lower extremities | 0.8 | 0.5 | Neck flexors are relatively weaker than neck extensors | 1.9 | 1.6 | In the legs proximal muscles are relatively weaker than distal muscles | 0.9 | 1.2 | Skin manifestations | Heliotrope rash | 3.1 | 3.2 | Gottron’s papules | 2.1 | 2.7 | Gottron’s sign | 3.3 | 3.7 | Other clinical manifestations | Dysphagia or esophageal dysmotility | 0.7 | 0.6 | Laboratory measurements | Anti-Jo-1 (antihistidyl-tRNA synthetase) autoantibody present | 3.9 | 3.8 | Elevated serum levels of CK or LDH or aspartate aminotransferase (AST) or alanine aminotransferase (ALT) | 1.3 | 1.4 | Muscle biopsy features, presence of | Endomysial infiltration of mononuclear cells surrounding, but not invading, myofibres | | 1.7 | Perimysial and/or perivascular infiltration of mononuclear cells | | 1.2 | Perifascicular atrophy | | 1.9 | Rimmed vacuoles | | 3.1 |
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Note: table does not show the scores of the patient.
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