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Hereditary angio-oedema

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Complement in Health and Disease

Part of the book series: Immunology and Medicine ((IMME,volume 20))

Abstract

Hereditary angio-oedema is defined clinically by episodic attacks of painless non-pruritic swelling of the external surfaces of the body (Figure 8.1) and/or the gastrointestinal tract that affects multiple generations of a family1-4. In the American literature, credit for the original description is often given to William Osier who described a family with the disease affecting members of four generations5. The autosomal dominant nature of the inheritance pattern (Figure 8.2) was remarked upon in this early study. In fact, the initial observations of this disease are older; in Europe, the disease is often known as Quincke’s oedema6 and there are older descriptions. As originally noted, the disease is defined by episodic attacks of non-pruritic oedema that affect any external surface of the body as well as by attacks of severe abdominal pain1-4. The attacks usually have a duration of one to three days, occurring with no clear pattern. Although clinically the disease is characterized by the occurrence of episodes of oedema of the deeper tissues of the dermis leading to marked local swelling, other organs can be affected. The older literature suggests that many organs can be so affected and there are reported cases of angio-oedema of cerebral tissue leading to episodic hemiparesis as well as angio-oedema of many other organs leading to dysfunction3. The original description of Quincke includes such an attack6. In fact, those who have followed large numbers of patients with biochemically proven hereditary angio-oedema have not been struck by the frequent attacks of angio-oedema of internal organs with the single exception of the gastrointestinal tract.

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  1. M. M. Frank
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Editors and Affiliations

  1. Department of Immunology, University of Leicester, Leicester, UK

    K. Whaley

  2. Institut für Medizinische Mikrobiologie, University of Mainz, Mainz, Germany

    M. Loos

  3. University of Iowa, Iowa City, Iowa, USA

    J. M. Weiler

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Frank, M.M. (1993). Hereditary angio-oedema. In: Whaley, K., Loos, M., Weiler, J.M. (eds) Complement in Health and Disease. Immunology and Medicine, vol 20. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-2214-6_8

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  • DOI: https://doi.org/10.1007/978-94-011-2214-6_8

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