Familial bilateral perilimbal cystic benign melanosis with lipid keratopathy--a new entity?

We examined the clinicopathologic findings in two sisters with pigmented conjunctival lesions. Slit-lamp examination showed bilateral, circumferential cystic pigmented perilimbal lesion and prominent lipid keratopathy. The pigmented lesions were examined via biopsy, and histologic and ultrastructural examination of the biopsy specimens showed secondary melanosis of numerous conjunctival epithelial inclusion cysts. The two sisters have familial bilateral perilimbal cystic benign melanosis with lipid keratopathy, an idiopathic condition that requires no further surgical management.