[Successful topical cyclosporin A in the therapy of progressive vascularising keratitis in keratitis-ichthyosis-deafness (KID) syndrome (Senter syndrome)].

Background: The keratitis-ichthyosis-deafness (KID) syndrome (Senter Syndrome) and its major criteria erythrokeratodermia, neuro-sensoric deafness and vascularising keratitis were defined in 1981. Several cases have been described since 1915, but up to now no causal therapy of this disease with sporadic occurrence has been found. Clinical experience using systemic Cyclosporin A (CsA) in the dermatological therapy of the KID syndrome differs. Up to now there has been no report on the use of topical CsA eye drops in the therapy of vascularising keratitis.

History And Signs: We report on a case of keratitis, ichthyosis and deafness (KID syndrome) in a 36-year old male. Both eyes were suffering from recurrent corneal ulcers (ulcus serpens corneae). No improvement had been observed on topical or systemic medication (including lubricants, antibiotics, steroids, etc.) during a history of more than two years.

Therapy And Outcome: Topical CsA 2 % (eye drops, 3 times daily) was administered in addition to lubricants. After 3 months the deep corneal neovascularisations were significantly reduced. The corneal ulcer was closed and visual acuity increased from 20/400 (OU) to 20/100 (OD) and 20/200 (OS), respectively.

Conclusion: A dose-dependent keratinocyte growth inhibition was found in vitro for cyclosporin A using ichthyosis skin samples. The reduction of activated T-lymphocytes and the resulting reduced HLA-Class-2 expression of conjunctiva and cornea as well as the inhibition of activated T-Cells in the lacrimal gland might be responsible. Nevertheless, the effect of topical CsA on the vascularizing keratitis in the KID syndrome is unknown.