全身皮膚色素異常を伴ったSclerocorneaの症例

A case of bilateral total sclerocornea associated with café-au-lait spot like pigmentation of the general skin

　両側のTotal sclerocorneaに全身の皮膚色素異常を伴った生後1ヵ月の男児の症例を報告した。生後2歳時に左眼に対し表層角膜移植を行い良好な結果を得た。光学顕微鏡による組織検査にて，角膜上皮の浮腫，Bowman層の欠如，実質内への血管侵入および膠原線維の不規則な配列等の特徴が見られた。眼合併症，全身合併症との関連性に於て，発生学的見地よりsclerocorneaの病因の文献学的考察を行った。

Bilateral total sclerocornea was diagnosed in a male infant one month after normal birth and growth. The whole skin was affected by pigmented patches like café-au-lait in color. Lamellar kerato-plasty was performed on the left eye at the age of two. The postoperative course was uneventful and the grafted cornea remained transparent one year after surgery. The removed corneal tissue showed epithelial edema, absence of Bowman's membrane, presence of irregularly arranged wavy collagen bundles and vascularization into the stroma.