Characteristics of patients with ocular cicatricial pemphigoid referred to major tertiary hospital.

Objective: To evaluate the demographic and clinical characteristics of patients referred to a tertiary care hospital cornea clinic for ocular cicatricial pemphigoid (OCP) assessment.

Design: Retrospective, nonrandomized, consecutive case series.

Participants: Thirty three patients with OCP who were treated at the corneal clinic of Toronto Western Hospital from 2003 to 2012.

Methods: Database search of patients from 2003 to 2012 with a referral request or diagnosis of OCP was conducted at a tertiary care hospital cornea clinic. Charts of 33 patients (64 eyes) were reviewed. Outcome measures included patient demographics, methods of diagnosis, visual acuity, ocular features, and disease staging using Foster's staging system, systemic modes of treatment, disease progression, and presence of systemic involvement.

Results: Mean patient age at presentation was 69.8 years (range 40-91), and 81% (27/33) were female. At presentation, disease staging consisted of stage I (subepithelial fibrosis) 7.8% (5/64), stage II (shortened fornices) 21.8% (14/64), stage III (symblepharon formation) 65.6% (42/64), and stage IV (keratinization with or without globe immobility) 4.6% (3/64). At the final follow-up visit, the proportions of the involved eyes for stages I to IV were 1.5% (1/64), 10.9% (7/64), 76.5% (49/64), and 10.9% (7/64), respectively. Conjunctival biopsies were obtained from 81% (27/33) of patients and reported as positive in 30% (8/27), negative in 63% (17/27), and inconclusive in 7% (2/27) of patients. Mean duration of follow-up was 6.8 ± 5.6 years (range 0.5-22), and 66.6% (22/33) of patients had progressive disease. Systemic mucocutaneous involvement was noted in 36.3% (12/33) of patients.

Conclusions: The high rate of disease progression suggests the need for improved therapeutic options. Additional modalities are needed in addition to conjunctival biopsy to confirm a diagnosis of OCP in patients with clinical signs of the disease.