IMMUNOELECTRON LABELING OF MATRIX COMPONENTS IN CONGENITAL HEREDITARYENDOTHELIAL DYSTROPHY

Two corneal buttons were obtained from a patient with congenital hered itary endothelial dystrophy (CHED) at the ages of 2.5 years (right eye ) and 14 years (left eye) and were studied by light and electron micro scopy including immunogold labelling for collagen types I-V and lamini n. The posterior collagenous layer (PCL) of Descemet's membrane contai ned collagen types I, III-V, and laminin: the latter was also localise d to fine-banded and granular material in the posterior non-banded zon e (PNBZ). Comparison of the endothelium at 2.5 years and 14 years reve aled occasional dystrophic changes in the former and extensive dystrop hic changes in the latter. The distribution of collagen types I, III a nd V within the PCL supports previous morphological observations of fi broblast-like change of the endothelium in CHED. Persisting endothelia l properties were manifest as positive labelling of type IV collagen a nd laminin. An excessive amount of laminin found in PNBZ and PCL is an other stress-related endothelial reaction.