Rapid corneal thinning and perforated ulcerative keratitis in a patient with relapsing polychondritis

BACKGROUND: To report rapid corneal thinning and perforation in a case with relapsing polychondritis. CASE PRESENTATION: A 43 year-old male diagnosed with relapsing polychondritis suffered from bilateral scleritis, bilateral swelling of pinna, saddle nose and tracheal stenosis. The patient presented with right eye pain and redness for one month. Slit lamp examination of the right eye showed 80% peripheral corneal thinning between 3 and 7 o’clock. The best-corrected visual acuity (BCVA) was 1.0 bilaterally. The degree of corneal thinning worsened to 90% after one week of oral corticosteroid use. Subsequently, topical cyclosporine 2% eye drops four times a day, oral doxycycline 100 mg twice a day and oral vitamin C 2 g daily were added. The corneal thinning gradually improved to about 60%. However, the patient rapidly tapered oral prednisolone against medical advice and returned with an acute drop in vision in his right eye. Slit lamp examination of the right eye showed peripheral corneal perforation with iris prolapse. An emergency repair with cyanoacrylate glue was performed. Intravenous methylprednisolone 1 mg/kg body weight was administered for three days and 1 g/day intravenous immunoglobulin was administered every four weeks. At 3 months postoperatively, BCVA in the right eye was 0.6. Slit lamp examination showed a well-formed anterior chamber with glue in situ. CONCLUSIONS: Relapsing polychondritis may be associated with rapid corneal thinning. The clinicians should be aware of the possibility of corneal perforation in these cases. Cyanoacrylate glue is a viable temporary management option in such scenarios.