Clinicopathologic criteria for nephrogenic systemic fibrosis

Clinical features		Scoring	Assigned score
Major criteria	Patterned plaques	3 points for presence of any 1 major criterion 4 points for presence of more than 1 major criteria
	Joint contractures
	"Cobblestoning"
	Marked induration/peau d'orange
Minor criteria	Puckering/linear banding	1 point for 1 minor criterion 2 points for 2 or more minor criteria
	Superficial plaque/patch
	Dermal papules
	Scleral plaques (age <45 years)
Total clinical score:

Histologic features		Scoring	Assigned score
Increased dermal cellularity		1
CD341 cells with tram-tracking		1
Thick and thin collagen bundles		1
Preserved elastic fibers		–1
Septal involvement		1
Osseous metaplasia		3
Total histologic score:

Original figure modified for this publication. From: Girardi M, Kay J, Elston DM, et al. Nephrogenic systemic fibrosis: clinicopathological definition and workup recommendations. J Am Acad Dermatol 2011; 65:1095. Table used with the permission of Elsevier Inc. All rights reserved.

Graphic 64487 Version 4.0

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Clinicopathologic criteria for nephrogenic systemic fibrosis