A syndrome involving the skin and subcutaneous submucosal tissues, occurring in a hereditary and a sporadic form. It presents with the sudden appearance of painless, circumscribed, nonpitting swellings of the face (around the eyes, chin, and lips), tongue, feet, genitalia, and trunk, which persist from a few hours to 2 or 3 days and then fade.
The heritable form, hereditary angioneurotic oedema, is transmitted as an autosomal dominant trait, involving the larynx and viscera. It is marked by abdominal pain, and has occasional severe and fatal respiratory complications. It is due to C1 esterase inhibitor deficiency. This inhibitor usually keeps the inflammatory reaction in check, but when it fails, a Quincke’s oedema may result. The term Quincke's oedema should be maintained, as the term angioneurotic oedema is less accurate. The sporadic form may be caused by allergy, infection, or emotional stress. Both sexes affected.
The first description of angioneurotic oedema was made by Marcello Donati (1538-1602) in 1586. It was subsequently described in 1876 as “giant urticaria” by the British surgeon and dermatologist John Laws Milton (1820-1898) in London. Further description in 1882 by Quincke, and by Henry Martyn Bannister (1844-1920) in 1894.
- Marcello Donati:
De medica historia mirabili. Mantuae, per Fr. Osanam, 1586.
- J. L. Milton:
On giant urticaria.
Edinburgh Medical Journal, 1876, 22: 513-526.
- H. I. Quincke.
Über akutes umschriebenes Hautödem.
Monatshefte für praktische Dermatologie, Hamburg, 1882, I: 129-131
English translation in Ralph Hermon Major (1884-1970): Classic Descriptions of Disease. Springfiled, C. C. Thomas, 1932.
- H. M. Bannister:
Acute angioneurotic oedema.
Journal of Nervous and Mental Diseases, Chicago, 1894, 21: 627-631.