Lasram L, Rais C, el Euch M, Ouertani A
Service d'Ophtalmologie, Hôpital Charles-Nicolle, Tunisie.
J Fr Ophtalmol. 1994;17(1):24-8.
Gelatinous drop-like corneal dystrophy is a rare disease, described for the first time by Nakaizumi (Japan) in 1914. We report 5 cases of this primary corneal amyloidosis. Three of our patients are brothers, the two other cases are isolated cases. Photophobia and progressive loss of vision are the first presenting signs. Corneal lesions are typically bilateral white nodular deposits beneath the epithelium. Patients underwent repeated keratoplasties with a mean interval of 5 years because of the recurrence of the disease on the corneal graft. Diagnosis of corneal amyloidosis was confirmed in all cases by histology. We compared our results with the data reported in the literature.
胶冻样滴状角膜营养不良是一种罕见疾病,由中泉(日本)于1914年首次描述。我们报告5例这种原发性角膜淀粉样变性病。我们的患者中有3例是兄弟,另外2例为散发病例。畏光和进行性视力丧失是最初出现的症状。角膜病变典型表现为上皮下双侧白色结节状沉积物。由于角膜移植后疾病复发,患者平均每隔5年接受一次重复角膜移植术。所有病例经组织学检查均确诊为角膜淀粉样变性病。我们将我们的结果与文献报道的数据进行了比较。