Background: Association of keratitis, ichthyosis, and deafness is known as KID syndrome. Only four cases have been reported in Japan by dermatologists.
Case: A male infant manifested absence of hair and generalized keratosis of the skin since birth. He had been diagnosed as having chronic mucocutaneous candidiasis (CMCC).
Findings: The patient manifested punctuate superficial keratopathy in the right eye and corneal opacity with peripheral neovascularization in the eye when initially seen at the age of 1 year and 4 months. The corneal lesion later developed into stromal keratitis with neovascularization in the stroma. Abnormalities simulating Hutchinson teeth were detected at the age of 4 years. Neurosensory deafness in the high-frequency region was detected at the age of 6 years. Serous tests for syphilis were consistently negative.
Conclusion: This child is the first reported case in Japan manifesting KID syndrome with Hutchinson teeth. The associated CMCC was interpreted as due to liability to infection in patients with KID syndrome.