Refractory corneal melting due to IgG4-related disease: Successful management via buccal mucosal graft transplantation

Abstract

Purpose

To present the first case of isolated corneal involvement in IgG4-RD, managed successfully with oral mucosal grafting due to the destructive course of the disease.

Case report

A 56-year-old female one-eyed patient was referred to our clinic with a diagnosis of unresponsive infectious corneal melting. The ophthalmologic examination revealed light perception visual acuity, intense conjunctival inflammation and an infected area of approximately 4 × 5 mm with accompanying melting. Her medical history includes a diagnosis of dry eye disease that has persisted for over a decade, multiple previous corneal transplant surgeries in the right eye with similar complaints, and subsequent evisceration surgery. Her complaints in the left eye had increased over the previous six months, and she had undergone eight instances of amniotic membrane transplantation at an external medical facility. Corneal scraping, conjunctival and corneal biopsy were performed. The histopathologic examination and elevated serum IgG4 level indicated the presence of IgG4-related disease. She was referred to the rheumatology department, where immunosuppression treatment was initiated. Oral mucosal grafting was performed to address the uncontrolled melting. At the follow-up examination, the melting was successfully controlled, the inflammation regressed, and the early hypertrophy of the oral mucosal epithelium flattened over time.

Conclusion

This case highlights the potential for IgG4-related disease to manifest as atypical ocular surface involvement. Dry eye secondary to the disease can exacerbate existing findings. In such instances, preserving the integrity of the globe is of the utmost importance. Therefore, oral mucosal grafting, which is known for its durability, should be considered a viable option.