iridocorneal endothelial syndrome
Definition A condition (abbreviated ICES) which is clinically and pathologically similar–if not identical–to posterior polymorphous membranous dystrophy (PPCD, OMIM:122000) and thus discussed together in this work.
Clinical findings ICES is usually unilateral, symptomatic, progressive, and presents at middle age, more commonly in women. It is attributed to an insult—e.g., herpes simplex infection, occurring in post-embryonic corneal development.
PPMD is bilateral, usually asymptomatic, nonprogressive, occurs at all ages, in all sexes (male, female, etc) and has been attributed to an insult during embryogenesis.
Histopathology Corneal oedema, glaucoma, and iris changes are more common in ICES.
In PPCD, endothelial cells are more likely to display epithelial-like characteristics.