Angioedema (Quincke's edema)

Quincke's edema is an acute, non-inflammatory swelling of the skin and subcutaneous tissue or mucous membranes and submucous tissues, that can lead to suffocation if it occurs in the larynx. náhled|Swollen hand in a patient with hereditary angioedema

Etiology
The cause of edema is a lack of serum inhibitor of the C1 component of complement (C1-INH). This leads to autoactivation of the C1 component, consumption of the C2 and C4 components and release of kinin mediators inducing edema.

We distinguish two forms:
 * hereditary – an autosomal dominantly inherited mutation in the C1-INH gene that leads to a decrease in the level of the inhibitor or its dysfunction;


 * acquired
 * increased degradation of C1-INH in malignant disease (type I),
 * the presence of autoantibodies against the inhibitor (type II),
 * a type I allergic reaction that takes place under the skin.

Clinical picture
The swelling is pale (non-inflammatory). It occurs mainly on the eyelids, lips, external genitalia, skin above the joints of the hands and feet. It is formed within minutes, and dissapears in a few days (1-3 days). It may be accompanied by vomiting, diarrhea and headaches. It mainly affects younger women and is often recurrent.

Diagnosis

 * Determination of C2, C4 complement and C1-INH levels;;
 * General examination - especially hematological

Diferential diagnosis
It is necessary to distinguish swellings of other etiology arising from various diseases - herpes zoster, lymphedema, erysipelas , trauma.

Therapy

 * C1-INH concentrate or frozen plasma;
 * prophylaxis: androgens, antifibrinolytics;
 * acquired forms: type I - necessary treatment of malignant disease, type II - immunosuppressive treatment.

Related articles

 * Hereditary angioedema
 * Immunopathological reaction type I

Source

 * BENEŠ, Jiří. Study materials [online]. © 2007. [feeling. 11.1.2011]. < http://jirben2.chytrak.cz/ >.

Used literature

 * VOKURKA, Martin and Jan HUGO, et al. Great medical dictionary. 9th edition. Prague: Maxdorf, 2009. 1159 pp.  ISBN 978-80-7345-202-5.
 * ŠTORK, Jiří, et al. Dermatovenerology. 1st edition. Prague: Galén, 2008. 502 pp.  ISBN 978-80-7262-371-6.