Ocular Cicatricial Pemphigoid
An elderly patient with a past ocular history of biopsy-negative cicatrizing conjunctivitis with symblepharon in both eyes, dry eye syndrome, myopic degeneration, and bilateral pseudophakia presented with right eye pain and itching for 3 days. She reported no associated vision changes. Her exam was notable for visual acuity of 20/60 in the right eye and count fingers in the left eye. Intraocular pressure was 18 and 12. Slit lamp exam was most notable for symblepharon in both eyes, corneal haze in both eyes, circumferential pannus in the right eye, and punctate epithelial erosions in both eyes. There was a poor view to the posterior segment in both eyes. Prior to presentation, the patient had had a stable exam without active cicatrization for 4 years, treated with various topical therapies and mycophenolate mofetil. Over the next several months, she was treated conservatively with bandage contact lenses, lubricating ointment, and epilation, however she developed a persistent, enlarging corneal epithelial defect. She was started on acyclovir, systemic prednisone, and mycophenolate mofetil. She then underwent symblepharolysis with mucous membrane grafting and salivary gland implantation, first to the left eye, then to the right eye. Post-operatively, both eyes developed a ”wet” surface due to mucus production from the salivary gland grafts. A left keratoprosthesis was then placed. At post-operative month 2, vision had improved to 20/100 in the left eye. The patient is currently awaiting placement of a keratoprosthesis in the right eye.
Presentation Date: 04/22/2021
Issue Date: 05/07/2021
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