Table A1.

The differential diagnosis of posterior polymorphous corneal dystrophy and iridocorneal endothelial syndrome.

		PPCD	ICE Syndrome
Etiology		PPCD1: VSX1 PPCD2: COL8A2 PPCD3: ZEB1	Unknown Probable viral etiology
Inheritance		Autosomal dominant	Sporadic
Presentation		More frequently bilateral	More frequently unilateral
Onset		Early childhood	Adulthood
Signs	Cornea	Endothelial lesions Vesicular Banded Diffuse	“Beaten metal” appearance Chandler: early marked edema APS: possible edema Cogan-Reese: possible edema
	Anterior chamber	Peripheral iridocorneal adhesions in 25%	Peripheral anterior synechiae
	Iris	Atrophy	Chandler: iris atrophy PIA: Full thickness holes Cogan-Reese: nodules + atrophy
	Pupil	Corectopia	Chandler: corectopia PIA: polycoria Cogan-Reese: uncommon changes
Cytokeratins		CK7 CK19	34BE12 Pkk1 KL1 CK19

Abbreviations: APS, antiphospholipid syndrome; PIA, progressive iris atrophy; ICE, iridocorneal endothelial syndrome; PPCD, posterior polymorphous corneal dystrophy.