We describe the findings in nine Asian-Indian patients with gelatinous droplike corneal dystrophy (GDCD). Most patients showed initial signs of bilateral decreased vision and photophobia at an early age. The siblings were affected in four cases, and parental consanguinity was recorded in seven cases. We classified the disease into three forms based on clinicopathologic findings. Clinically, the mild form of the disease was evidenced by central subepithelial, whitish-yellow, nodular lesions that corresponded to the subepithelial nodular deposits seen histologically. In the moderate form, the lesions coalesced, forming central, diffuse subepithelial lesions with superficial vascularization. On histology, the amyloid deposits assumed a sheetlike distribution in the subepithelial regions and in the superficial stroma. Aggregates of chronic inflammatory cells and stromal neovascularization were seen in the adjacent stroma. The severe form presented as diffuse, whitish opacification of the cornea with extensive neovascularization and scarring. Histologically this form was characterized by a visible plaque of vascularized scar tissue that partly replaced the stroma and enveloped the amyloid deposits. Frequent, early recurrence of the disease was noted in the grafts. This study provides a detailed clinicopathologic description of GDCD from the Indian subcontinent. We also discuss previously unreported findings from the most advanced stage of the disease.
- Corneal amyloidosis
- Gelatinous droplike corneal dystrophy
ASJC Scopus subject areas