Eyeworld

EW RESIDENTS 60 April 2014 by Maxwell Elia, MD, resident, and John J. Huang, MD, residency program director, Department of Ophthalmology and Visual Science, Yale University School of Medicine Management of ocular cicatricial pemphigoid movements were full. Anterior exam was significant for bilateral symble- pharon and forniceal shortening as well as ankyloblepharon with corneal keratinization in the left eye (Figure 1). In an attempt to achieve disease control, the patient was started on prednisone 60 mg daily in addition to serum tears and topical prednisolone acetate 1% four times daily. One month later, the patient presented with worsening symble- pharon, ankyloblepharon and kera- tinization of both eyes. Her left eye also demonstrated worsening left upper eyelid cicatricial entropion. Mycophenolate mofetil 500 mg twice daily and tacrolimus ointment twice daily were recommended, in addition to her methotrexate, pred- nisone, and topical therapy. By the next visit, the patient's pain had improved, and her vision stabilized at counting fingers OD and hand motions OS. She complained of de- bilitating vision loss and wanted to discuss her options for visual recov- ery. Her anterior exam was notable for bilateral stage IV OCP (forniceal shortening, symblepharon, and corneal keratinization) as well as cicatricial entropion of the left upper eyelid (Figure 2). Discussion Cicatricial pemphigoid (CP) is a dev- astating systemic disease resulting from the deposition of autoantibod- ies at the epithelial-subepithelial junction of mucous membranes. The eyes are involved in 70% of the cases of CP. The condition can cause chronic conjunctivitis, subepithelial conjunctival fibrosis, fornix foreshortening, symblepharon and ankyloblepharon formation, meibomian gland obstruction, trichiasis, and eventual lacrimal gland obstruction. Vision lost is due Case presentation An 80-year-old woman was referred for bilateral eye pain. Her ocular history was significant for biopsy proven ocular cicatricial pemphigoid (OCP) for which she was receiving methotrexate 8 mg subcutaneous weekly injections. Aside from OCP, the patient was in good health. The patient previously underwent weekly epilations in the left eye, but she now complained of bilateral eye pain. On exam, she was 20/400 OD and counting fingers OS. Her IOP was normal and her extraocular Figure 1. Right eye (OD): extensive symblepharon with temporal and nasal conjunctival adhesion to the inferior conjunctival fornix; left eye (OS): corneal pannus with ankyloblepharon of the upper lid and conjunctiva to the superior cornea and limbus Figure 2. Right eye (OD): progression of the symblepharon and new ankyloblepharon of the temporal eyelid to the temporal cornea and limbus; left eye (OS): progression of the cornea pannus with keratinization of the corneal and conjunctival epithelium with extensive ankyloblepharon of the nasal and upper lid to the cornea Source (all): John Huang, MD John Huang, MD, residency program director, Department of Ophthalmology and Visual Science, Yale University School of Medicine Academic grand rounds

• Cover