KID症候群の1例

A case of keratitis-ichthyosis-deafness（KID）syndrome

　目的：角膜炎，魚鱗癬，難聴をその内容とするKID症候群症例の生下時から3歳までの経過記述。症例：在胎32週，2,035gで出生した女児。生下時より無毛で紅角皮症を呈した。経過：生後3か月目に角膜実質炎，角膜浸潤，上皮欠損が左眼に生じた。抗菌薬とステロイド薬の点眼で角膜浸潤と上皮欠損が縮小し，発症から2か月後に角膜混濁と血管新入を残して症状が鎮静化した。感音性難聴があることが判明し，KID症候群と診断した。生後2年8か月目に右眼に同様な病変が生じ，同様な経過をとった。結論：KID症候群での角膜炎の機序は不明であるが，今回の症例で病変が鎮静化した後の所見は梅毒性角膜実質炎に酷似していた。

　Purpose：To describe a case of keratitis-ichthyosis-deafness（KID）syndrome up to the age of 3 years. Design：Observational case report. Method：Clinical observation of corneal involvement of a girl infant with KID syndrome. Results：Patient was born at 32 weeks weighting 2,035 g. She had alopecial,erythroeratoderma and reticulated palmoplanter hyperkeratosis at birth suggestive of KID syndrome. Her left eye developed severe interstitial keratitis with corneal infiltration and epithelial defect at the age of 3 months. Neurosensory deafness was detected,leading to the diagnosis of KID syndrome. The corneal lesion healed by topial antibiotics and corticosteroid leaving corneal opacity and vessel invasion. Her right eye developed essentially similar lesion at age 2 years 8 months. It healed similar to that in the left eye. Conclusion：Findings after cure of corneal lesions simulated those of syphilitic interstitial keratitis.