網膜脂血症をみた脂肪萎縮性糖尿病（lipoatrophic diabetes）の1例

A case of lipoatrophic diabetes with retinal lipemia

　脂肪萎縮性糖尿病は特殊型糖尿病の代表的疾患とされ，全身の脂肪組織の欠如と肝脾腫，インスリン抵抗性糖尿病，高脂血症，代謝亢進を伴う症候群であり，その特異な病態にもかかわらず発症機序はいまだに明らかにされていない。筆者らは，脂肪萎縮性糖尿病を基礎疾患として有する17歳の女性に網膜脂血症をみた症例を経験した。

　本症例は，初診時，血中トリグリセライド値が2,500mg／dlを超え，リポ蛋白分画定量結果からは高脂血症に対するFredrickson分類のV型に相当した。眼底所見上，網膜面および網膜血管の色調は特異な性状を呈し，後極部網膜は黄白色の色調が強く，中間周辺部から周辺部にかけて動静脈の色調はほぼ同一の淡紅色で，その識別は困難であった。

　本症例は，Vingerらによる網膜脂血症の分類にしたがえばgrade Iに相当した。

A l7-year-old female was referred to us for ophthalmological examinations related to diabetes. She had manifested disappearance of systemic adipose tissue since 2 years of age showing signs of total lipodystrophy. Hyperlipemia, type V by Fre-drickson's classification, was detected at 12 years of age. She was diagnosed as insulin-resistant diabetes mellitus and has been treated by peroral antidiabetic medications and diet therapy since 5 years before. Her corrected visual acuity was 1.0 in either eye. Fundus examinations showed no signs of ordinary diabetic retinopathy. Instead, retinal arteries and veins were salmon pink in color, pre-sumably due to hyperglyceridemia. This appear-ance was more pronounced towards the periphery. The serum triglyceride level was 2500 mg/dl. Fun-duscopic features in this case corresponded to grade I of Vinger's classification of retinal lipemia.