Angioedema acquired T78.3

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 27.09.2022

Dieser Artikel auf Deutsch


Acquired quinckeedema; angioneurotic edema; Bannister's disease; Quincke edema acquired

This section has been translated automatically.

Differently defined group of diseases. Originally, the adjective "acquired" was used to refer to "acquired C1-esterase inhibitor deficiency" and thus was used in opposition to hereditary angioedema (see below complement system). However, "acquired" forms of angioedema in the broader sense also include:

This section has been translated automatically.

Type I (acquired angioedema, AAE I): partly with lymphoproliferative malignancies, e.g. malignant lymphoma.

Type II (AAE II): like AAE I, detectable antibodies against C1-INH.

This section has been translated automatically.

Quantitative and/or functionalC1 esterase inhibitor deficiency. Pronounced C1q deficiency.

Drug-induced, e.g., by ACE inhibitors, ciclosporin A, or other drugs (e.g., hawthorn extracts [Crataegutt] and others).

Furthermore, the formation of antibodies against the active site of theC1 inhibitor as well as the activation of the complement cascade by anti-idiotypic antibodies with excessive consumption (AAE type II) are described.

This section has been translated automatically.

3.8% ofC1-esterase inhibitor deficiencies(angioedema) are acquired First manifestation in middle or old age (in contrast to hereditary angioedema), negative family history.

Clinical features
This section has been translated automatically.

Recurrent occurrence of cortisone- and antihistamine-insensitive angioedema over 48-72 hours. Abdominal symptoms and lethal course may occur. Associations with neoplasms such as lymphomas are also possible. Common occurrence of livedo racemosa and paraproteinemia without tumor detection and persistence during angioedema therapy are described in individual case reports. Regular check-ups to rule out lymphoma.

This section has been translated automatically.

DecreasedC1q (typical of acquired forms); in addition to reduced C1 esterase inhibitor deficiency (protein concentration and enzymatic activity),C2,C4 and CH-50 are also reduced. During therapy normalization or increase to subnormal values of complement fractions.C4 can be seen as an indicator for a successful therapy.

This section has been translated automatically.

This section has been translated automatically.

  1. Kirkup ME et al (2003) Experience with tranexamic acid for nonheriditary angio-oedema and urticaria. Br J Dermatol 149 (Suppl 64): 25-26
  2. Nashan D et al (1995) Life-threatening angioedema due to acquired C1-inhibitor deficiency associated with paraproteinemia and livedo racemosa. Dermatologist 46: 339-342
  3. Nettis E (2003) Clinical and aetiological aspects in urticaria and angio-oedema. Br J Dermatol 148: 501-506
  4. Netzwerk aktuell (2010) Angioedema caused by hawthorn extract (Crataegutt Novo). Medication telegram 41: 54


Please ask your physician for a reliable diagnosis. This website is only meant as a reference.


Last updated on: 27.09.2022