Keratoconjunctivitis as a Single Entity in X-linked Agammaglobulinemia?

ABSTRACT

Purpose

To report a case of a male patient with a severe corneal and conjunctival immunopathy likely caused by an X-linked agammaglobulinemia.

Methods

A clinical case report with observation results from 2001-2021.

Results

A severe corneal immunopathy of both eyes is reported in a retrospective long-term observation of nearly twenty years in a 32-year-old male patient with X-linked agammaglobulinemia (XLA). A chronic progressive corneal scarring with a loss of visual acuity and typical symptoms of a phlyctenular keratoconjunctivitis were observed.

Conclusion

Whereas steroid eye drops like dexamethasone could control the symptoms and the corneal scarring progression as short time therapy options, ciclosporin A eye drops showed problems in therapy adherence in long-time use. Antibiotic eye drops supported the anti-inflammatory therapy effects, but no typical pathogen was detected. Antineovascular subconjunctival application did not show any relevant effect in one-time use. Artificial tears were needed as basic therapy.

KEYWORDS:

• Bruton syndrome
• corneal immunopathy
• corneal scarring
• phlyctal keratoconjunctivitis
• X-linked agammaglobulinemia

Acknowledgments

The authors are grateful to Kathy L. Brenner for her continuous help in improving the quality of the content and to the reviewers of this article for their valuable comments.

Disclosure statement

Stefan Mielke reports grants from Redwood Pharma AB, outside the submitted work. Bastian Grundel, Sebastian Schmidt, and Frank Tost declare that there is no conflict of interest.

Ethical committee approval

Because this report was only a clinical observation case, Ethical Committee approval was not necessary.

Informed consent

The patient signed informed consent to publish the clinical course, including images.

Additional information

Funding

The authors reported that there is no funding associated with the work featured in this article.