Successful nonsurgical management of corneal perforation in a patient with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy

PURPOSE: To describe a patient with a history of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) who has experienced two episodes of noninfectious corneal ulceration with perforation that has been successfully managed with nonsurgical therapy. METHODS: Case report. RESULTS: An 18-year-old male with a history of APECED has been followed up with recurrent keratoconjunctivitis and episodes of microbial keratitis since 4 years of age. Ophthalmic complications have included two episodes of sterile corneal ulceration associated with corneal perforation. These episodes of stromal ulceration associated with a flat anterior chamber were managed with a bandage contact lens, topical antibiotics, and a topical cycloplegic agent. In one instance, addition of cyclosporine led to reformation of the anterior chamber and arrest of the ulcerative process. CONCLUSION: Sterile corneal ulceration and perforation associated with APECED may be managed medically to avoid surgical intervention, which has been reported to lead to a poor outcome.