Primary Corneal Keloid

To report the clinical presentation, histopathologic findings, and surgical treatment in a series of cases with primary corneal keloid. Six Iranian patients (a father and two sons, a mother and her daughter, and one isolated male patient) without any history of corneal trauma or systemic diseases were clinically and histologically evaluated for a slowly-growing, white opacity in the cornea. On slit-lamp examinations, corneal lesions appeared as a solitary, pearly white, well-circumscribed nodule with a smooth and glistening surface. Because of the deterioration of visual acuity, the nodule was surgically removed by deep anterior lamellar keratoplasty (DALK) in two patients. Histopathology of the excised nodules were consistent with the diagnosis of corneal keloid in terms of epithelial irregularities, Bowman’s layer disruption, thick and haphazardly-arranged stromal collagen fibers, and proliferation of fibroblastic cells that were immune-reactive to-smooth muscle actin antibody. Clinical outcomes of these 2 cases that underwent DALK were presented here. In this series, six cases of corneal keloids were reported, 5 of whom appeared to have an autosomal dominant inheritance pattern of variable penetrance. Surgical interventions are limited to selected patients with decreased visual acuity or cosmetic problems. Further genetic investigations are necessitated to verify the responsible defective genes.