Hereditary angioedema (HAE) is a form of the kind of swelling discussed on this website. Its characteristic symptom is swelling that is localized in the hypodermis. Another name sometimes used is Quincke edema.
There are three types of HAE: I, II and III. In a sense, the first two are more similar to each other in that they are both brought on by a C1 inhibitor deficiency, while type III occurs due to particular F12 gene coding mutations. Information on this page might focus more on types I and II, and as with all details on this website, please contact a doctor or medical professional regarding particular advice for your case or that of someone you know. What you read here is only intended to be informational, and should not take the place of advice or assistance from someone who is qualified in the field.
When a doctor is checking for a possible diagnosis of hereditary angioedema, a blood test is taken. This is preferably administered while an episode of HAE is taking place. C2 and C4 are complementary to C1, and analyzing the levels of them in the individual may also be done.
When an episode is occurring, the point of treatment is to stop the continuation of the swelling. If the edema is located in the voice box (larynx), then this is particularly important. The method of treatment has the potential to vary in different cases. One option is giving the patient C1-INH via IV. Fresh frozen plasma, shortened as FFP, is also used in some cases. Other treatment methods are also possible.
In some individuals with hereditary angioedema, particular prevention methods should be used. This type method is known as prophylaxis, and there are both long-term and short-term versions. As in treatment for acute episodes, C1-INH concentrate and FFP are two options in the long-term form of prevention, although there are others. Long-term methods are used for patients who have an episode, or more, per month, and are also considered to have an elevated risk of developing this swelling in the voice box. The short-term form of prophylaxis is used before dental work or surgery.