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

Note: table does not show the scores of the patient.