Cunnane S C, Kent E T, McAdoo K R, Caldwell D, Lin A N, Carter D M
Efamol Research Institute, Kentville, Nova Scotia, Canada.
J Invest Dermatol. 1987 Oct;89(4):395-9. doi: 10.1111/1523-1747.ep12471769.
The fatty acid composition of plasma and erythrocyte phospholipids was determined in children with various subtypes of epidermolysis bullosa (EB) and in their parents. Patients with recessive dystrophic, dominant dystrophic, simplex, or junctional forms of EB had a higher percentage composition of arachidonic acid in plasma and/or erythrocyte phospholipids compared to age-matched controls. Epidermolysis bullosa patients treated with diphenylhydantoin had lower levels of arachidonic acid in plasma and erythrocyte phospholipids than did untreated EB patients. Parents of children with the recessive dystrophic or junctional EB subtypes had higher linoleic and arachidonic acids in plasma and erythrocyte phospholipids than did controls. Plasma and erythrocyte total lipids were within the normal range in children with EB. Plasma zinc was also normal but plasma copper was elevated in children with recessive dystrophic EB. We conclude that higher arachidonic acid in plasma and erythrocytes may be related to the pathology of EB.
对患有不同亚型大疱性表皮松解症（EB）的儿童及其父母的血浆和红细胞磷脂的脂肪酸组成进行了测定。与年龄匹配的对照组相比，患有隐性营养不良型、显性营养不良型、单纯型或交界型EB的患者血浆和/或红细胞磷脂中花生四烯酸的百分比组成更高。接受苯妥英治疗的大疱性表皮松解症患者血浆和红细胞磷脂中的花生四烯酸水平低于未治疗的EB患者。患有隐性营养不良型或交界型EB亚型的儿童的父母血浆和红细胞磷脂中的亚油酸和花生四烯酸含量高于对照组。EB患儿的血浆和红细胞总脂质在正常范围内。血浆锌也正常，但隐性营养不良型EB患儿的血浆铜升高。我们得出结论，血浆和红细胞中较高的花生四烯酸可能与EB的病理有关。
