角膜の菲薄化と前方突出がみられ穿孔をきたした続発性角膜アミロイドーシスの1例

A case of corneal perforation owing to secondary corneal amyloidosis with local thinning and protrusion

要約　目的：病変部が角膜前後面ともに突出し，角膜穿孔に至った続発性角膜アミロイドーシス（SCA）の症例を報告する。

症例：26歳，女性。左眼の白色腫瘤と眼痛を主訴に受診し，睫毛乱生に続発したSCAと診断された。角膜病変は，局所的に角膜前後面ともに突出していた。経過中に角膜菲薄化が進行し，角膜穿孔に至ったため全層角膜移植術を施行した。病変部の病理所見では角膜全層がアミロイドに置換されており，正常な角膜構造は破壊されていた。

結論：本症例では病変部が角膜前後面ともに突出し，同部位は角膜全層にわたってアミロイドに置換されていた。全層にわたる病変を伴うSCAの症例は，菲薄化が急速に進行する可能性がある。

Abstract　Purpose：To report the case of a patient with secondary corneal amyloidosis（SCA）that showed corneal perforation owing to localized anterior protrusion from the posterior surface of the cornea.

Case：A 26-year-old female presented with a white protruded opacity and pain in her left eye. She was diagnosed with SCA associated with entropion. The corneal lesion protruded entirely from the posterior surface. The lesion was thinning and led to perforation, and she underwent penetrating keratoplasty. The pathological findings of the lesion showed that the entire corneal stroma had been replaced by amyloid deposit, and the normal corneal structure was destroyed.

Conclusion：In this case, the lesion protruded from the posterior surface of the cornea；it underwent thinning and was replaced by amyloid deposit in the entire corneal stroma. SCA patients with completely degenerated lesions should be followed up frequently because they may cause rapid progression of thinning and protrusion.