Recurrence of macular corneal dystrophy after keratoplasty is rare. We repo
rt light microscopic, immunohistochemical, electron microscopic, and serolo
gic findings in a 78-year-old woman who underwent regrafting 49 years follo
wing the first penetrating keratoplasty. Examination of the corneal button
revealed deposits of glycosaminoglycans in the graft beneath the Bowman lay
er, throughout the stroma, and in the endothelium with positive staining fo
r antigenic keratan sulfate. By transmission electron microscopy, intracell
ular and extracellular deposits of a fibrillogranular material were detecte
d in the stroma, Descemet membrane, and endothelium. The serum level of ant
igenic keratan sulfate was normal. Our findings indicate that macular corne
al dystrophy type II may show late recurrence after penetrating keraloplast
y with intense deposition of antigenic keratan sulfate in all corneal layer
s.