Two months later, the patient returned with a 2-week history of worsening vision, pain, and corneal ulceration. The right eye had no light perception and the left eye had light perception with projection. Examination showed extensive progression of the cutaneous NXG with loss of preseptal tissue but sparing of pretarsal tissue; the tissues were secondarily infected with multiple microbes. All eyelids were immobile, resulting in exposure keratopathy. The right cornea was superinfected and perforated, and the left cornea was opacified inferiorly, presumably from infection (Figure 1). After beginning systemic and topical antibiotic treatment, the right globe was enucleated and the periorbital tissue débrided. The left eye required several procedures to cover the exposed cornea, including conjunctival flaps and amniotic membrane grafts; finally, a buccal mucosal graft was required because the conjunctival flaps became necrotic. Adjuvant systemic therapy throughout this course included 2 -chlorodeoxyadenosine (or cladribine) and hyperbaric oxygen, with significant improvement of the cutaneous disease. The left eye received a buccal mucosal graft with a Boston type II keratoprosthesis 10 months after her initial visit to us, resulting in best-corrected visual acuity of 20/60 (Figure 1).