Ocular cicatricial pemphigoid: case report

Abstract

Background: The main characteristic of ocular cicatricial pemphigoid is subepithelial conjunctival fibrosis, which, if progressive, can lead to conjunctival shrinkage and symblepharon formation. Other characteristics include entropion, trichiasis, dry eye, cornea ulceration, neovascularization, and keratinization. Ultimately, blindness may occur. Cicatricial pemphigoid may also affect the skin and mucous membranes, including the nose, oral cavity, pharynx, esophagus, anus, and vagina. Treatment involves systemic immunosuppressants and corticosteroids to suppress active inflammation.

Case report: A 69-year-old man had symblephara, conjunctival shortening, and extraocular manifestations, and was diagnosed with ocular cicatricial pemphigoid on the basis of clinical appearance and immunofluorescent studies. Treatment included oral Dapsone, prednisone, and azathioprine to stabilize the condition.

Conclusion: Close monitoring of ocular cicatricial pemphigoid is imperative for ocular manifestations and for extraocular complications occurring with this progressive autoimmune disease and its treatment.