Detection of Hereditary Angioneurotic Edema by Demonstration of a Reduction in the Second Component of Human Complement

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Abstract
ACUTE circumscribed subepithelial edema of the skin was recognized as a distinct clinical entity by Milton1 and by Quincke,2 and shortly thereafter was termed angioneurotic edema by Strübing.3 In 1888 Osler4 called attention to a rare hereditary form of this syndrome, transmitted as a mendelian dominant5 6 7 with irregular penetrance.8 , 9 Some 503 cases in 61 families have been reported,7 , 10 11 12 13 but in only two thirds are there sufficient family data to permit a reasonably firm diagnosis of angioneurotic edema of the hereditary type.Hereditary angioneurotic edema is characterized by recurrent, acute, circumscribed and transient subepithelial edema of the skin, gastrointestinal mucosa . . .