Ocular cicatricial pemphigoid

Abstract

Ocular cicatricial pemphigoid (OCP) is an autoimmune ocular disease that causes severe dry eye syndrome, conjunctival scarring with inferior fornix shortening and entropion along with trichiasis. Corneal keratinization and corneal ulcers may lead to permanent vision loss. The therapeutic approach of OCP is a challenging one. Thus, the treatment consists of a systemic therapy that includes immunosuppressive as well as corticosteroid medication. Also, surgical procedures for modifications of eyelid position, symblepharon and cataract may aggravate the evolution of the disease. Dry eye syndrome, which is known to be a multifactorial disorder of the ocular surface secondary to qualitative or quantitative alteration of the tear film, is a severe and frequent complication of OCP. In this article, we presented 3 patients diagnosed with OCP, who developed severe dry eye syndrome, entropion, corneal erosions and ultimately, permanent vision loss.

Keywords: OCP; dry eye syndrome; entropion; symblepharon.

Fig. 1

Right eye symblepharon formation

Fig. 2

Left eye symblepharon formation

Fig. 3

Slit lamp: Corneal punctate epithelial erosions in the right eye