Cicatricial pemphigoid: diagnosis and treatment

Cicatricial pemphigoid is a systemic autoimmune disease with both ocular and extraocular manifestations. Its effect on the eyes is characterized by chronic cicatrizing conjunctivitis and progressive conjunctival sub-epithelial fibrosis with resultant fornix foreshortening, symblepharon formation, trichiasis, meibomian, and lacrimal duct obstruction resulting in sicca syndrome, ocular surface keratinization, and eventually blindness. Extraocular manifestations include stricture formation of the esophagus, trachea, anus, or vagina. OCP is characterized by deposition of immunoreactants at the conjunctival basement membrane zone, initiating a type II hypersensitivity reaction. Demonstration of this deposition can be done using immunofluorescence or immunoperoxidase technique.