Two patient with evere corneal dieae in KID yndrome

Purpoe To report two independent Japanee patient with keratiti, ichthyoi, and deafne (KID) yndrome and evere corneal diorder. Deign Obervational cae report. Method Clinical obervation of a 5-year-old boy (Patient 1) and a 64-year-old man (Patient 2) with KID yndrome, preenting prominent corneal dieae. Molecular genetic aement of the GJB2 gene encoding connexin-26 wa performed. Reult Patient 1 had bilateral diffue uperficial punctuate keratopathy with evere corneal neovacularization. He had a miene mutation of the GJB2 gene. Patient 2 had bilateral corneal tromal keratiti and right corneal ulceration with rupture of the Decemet membrane. He did not have any pathologic mutation of the GJB2 gene. The area of paliade of Vogt wa diminihed and tear production reduced in both patient. Topical eye drop, and corticoteroid or antibiotic, repectively, relieved them effectively. Concluion The impaired ocular urface regulating ytem might be a caue of corneal dieae in KID yndrome and it can be treated by eye drop