1Werschnik C., 1Duncker G. I. W., 2Keyßer G., 1Hammer T., 1Wilhelm F.

1Department of Ophthalmology of the Martin-Luther-University Halle- Wittenberg, Magdeburger Str. 8, 06097 Halle (Saale), Germany; 2Department of Internal Medicine of the Martin-Luther-University Halle- Wittenberg, Ernst-Grube-Str. 40, 06120 Halle (Saale), Germany

Background: Relapsing polychondritis is a very rare systemic disease with intermittent inflammation of nasal and auricular cartilage, ocular tissues and of the cardiovascular system. Ocular involvement (60%) occurs as keratitis with corneal ulcerations, conjunctivitis, episkleritis, iritis, neuritis of the optic nerve or paralysis of external muscles. An autoimmunologic pathogenesis is supposed.
Case report: A 43-year-old male patient presented with recurrent conjunctivitis and marginal corneal ulcerations of his right eye in 1998. The best corrected visual acuity was 20/80 on the right eye (strabismic amblyopia) and 20/30 on the left eye. Slitlamp examination showed a deep marginal corneal ulcer of the nasal limbal area. A lamellar horseshoe-like keratoplasty was performed. The clinical follow-up of the patient, further investigations and the problems of therapy as well as the systemic involvement in presumed relapsing polychondritis are demostrated.
Conclusions: A severe corneal manifestation of relapsing polychondritis is not very common, but most challanging regarding to the therapy of the patients. Immunsuppressive treatment with steroids alone or in combination with azathioprine, cyclophosphamide, cyclosporine or methotrexate are recommended in the literature. Recurrences are not rare, so that long-term follow-up and care of the patients are very important.

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