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Topical cyclosporin A in the therapy of the KID-SyndromeM. Derse, E. Wannke, J. M. Rohrbach, M. Zierhut
Introduction: The keratitis, ichthyosis and deafness (KID) syndrome and its major criteria erythrokeratodermy, neuro-sensoric deafness and vascularizing keratitis has been defined in 1981. Several cases had been described since 1915, but no causal therapy of this sporadic disease had been found up to now. Clinical experience using systemic Cyclosporin A (CsA) in the dermatological therapy of the KID syndrome differs. Up to now there has been no report about the use of topical CsA eye drops in the therapy of vacularizing keratitis.
Case report: We report a case of keratitis, ichthyosis and deafness (KID-Syndrom) in a 36 years old male. Both eyes were suffering from recurrent corneal ulcers (ulcus serpens corneae). No reaction was observed on topical or systemic medication during a history of more than two years. Topical CsA 2 % (eye drops 3 times daily) was administered in addition with lubricants. After 3 months the deep corneal neovascularisations were significantly reduced. The corneal ulcer was closed and visual acuity increased from 20 / 400 (RE = LE) to 20 / 100 (RE) and 20 / 200 (LE), respectively.
Conclusion: Topical CsA in the therapy of vascularizing keratitis in KID syndrome was successfully used in this case. A dose dependent keratinocyte growth inhibition had been found in-vitro for cyclosporin A using ichthyosis skin samples. Similar effects might be responsible for corneal tissue. Nevertheless the effect of topical CsA on the vacularizing keratitis in the KID syndrome is unknown today.
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