Chronic Early-Stage Ocular Cicatricial Pemphigoid

Abstract

Ocular cicatricial pemphigoid (OCP) represents an insidious, autoimmune-mediated disease of the conjunctiva, initially presenting as chronic conjunctivitis and progressing to fibrosis, cicatrization, and eventually blindness secondary to corneal keratinization. This series reports 3 cases presenting with chronic conjunctivitis lasting an average of 10 years without cicatrix formation, ultimately diagnosed as OCP based on direct immunofluorescence of conjunctival biopsy samples. This chronic conjunctivitis without fibrosis suggests the possibility of an OCP subtype with a prolonged early stage or prodrome prior to cicatrization, which may benefit from early diagnosis and treatment to prevent complications of this disease.