Clinical presentation and management of ocular cicatricial pemphigoid in Mexico
Purpose: To analyze the clinical presentation and features, as well as the outcome of immunosuppressive chemotherapy in patients with ocular cicatricial pemphigoid (OCP) in Mexico, and to provide diagnostic evaluation and management recommendations. Methods: Retrospective, non-comparative, case series. We included 8 patients (16 eyes) diagnosed with OCP, confirmed by conjunctival biopsy immunohistochemical assay. All patients were managed with immunosuppressive drugs. Signs, symptoms, disease stage at diagnosis, extra-ocular manifestations, therapeutic response, and treatment-related adverse effects were analyzed. Results: 8 patients were included, 7 female (87.5%) and 1 male (12.5%), with mean age at presentation of 71.5 years. 50% of patients had extra-ocular lesions, the most frequent being in oropharynx (50%). 90% of patient-eyes presented with stage III/IV of the disease (Foster staging system), and all patient-eyes had symblepharon formation and fornix shortening. Superficial punctuate keratitis was the most common corneal finding (75%). After conjunctival biopsyproven diagnosis, immunosuppressive chemotherapy was applied: 5 patients were managed with azathioprine, and the 3 remaining were managed with cyclophosphamide. No treatment related complications were reported. Conclusions: The main goal of immunosuppressive therapy is to control chronic inflammation and halter its tissue destructive progression. The adequate, opportune and sufficient use of immunosuppressive chemotherapy achieves these objectives in the majority of cases.