This article reports on two Japanese cases of the gelatinous droplike
dystrophy; both are singletons. An 18-year-old man scheduled for anoth
er keratoplasty on his left eye, followed a lamellar keratoplasty twic
e on the right eye and once on the left eye. A 20-year-old man had fai
r left visual acuity, 20/400, after a lamellar keratoplasty on his lef
t eye, followed by a lamellar keratoplasty on his right eye and a pene
trating keratoplasty on his left eye. The prealbumin gene was analyzed
in these cases of gelatinous droplike dystrophy. Restriction enzyme a
nalysis and single strand conformation polymorphism (SSCP) analysis sh
owed no abnormal substitution in the prealbumin gene. The results sugg
ested that the pathogenesis of an accumulation of amyloid in gelatinou
s droplike dystrophy is different from that in familial amyloidotic po
lyneuropathy (FAP).