Angioedema

Angioedema
Allergic angioedema: this child is unable to open his eyes due to the swelling.
Classification and external resources
Specialty	Allergy and immunology
ICD-10	D84.1 , T78.3
ICD-9-CM	277.6, 995.1
OMIM	606860 106100 610618
DiseasesDB	13606
MedlinePlus	000846
eMedicine	emerg/32 med/135 ped/101
Patient UK	Angioedema
MeSH	D000799
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Angioedema, also known as angiooedema, Quincke's edema, and angioneurotic edema, is the rapid swelling (edema) of the dermis, subcutaneous tissue,mucosa and submucosal tissues. It is very similar to urticaria, but urticaria, commonly known as hives, occurs in the upper dermis.

Cases where angioedema progresses rapidly should be treated as a medical emergency, as airway obstruction and suffocation can occur. Epinephrine may be life-saving when the cause of angioedema is allergic. In the case of hereditary angioedema, treatment with epinephrine has not been shown to be helpful.

Angioedema is classified as either hereditary or acquired.

Acquired angioedema (AAE) can be immunologic, nonimmunologic, or idiopathic. It is usually caused by allergy and occurs together with other allergic symptoms and urticaria. It can also occur as a side effect to certain medications, particularly ACE inhibitors. It is characterized by repetitive episodes of swelling, frequently of the face, lips, tongue, limbs, and genitals. Edema of the gastrointestinal mucosa typically leads to severe abdominal pain; in the upper respiratory tract, it can be life-threatening.

Hereditary angioedema (HAE) exists in three forms, all of which are caused by a genetic mutation inherited in an autosomal dominant form. They are distinguished by the underlying genetic abnormality. Types I and II are caused by mutations in the SERPING1 gene, which result in either diminished levels of the C1-inhibitor protein (type I HAE) or dysfunctional forms of the same protein (type II HAE). Type III HAE has been linked with mutations in the F12 gene, which encodes the coagulation protein factor XII. All forms of HAE lead to abnormal activation of the complement system, and all forms can cause swelling elsewhere in the body, such as the digestive tract. If HAE involves the larynx, it can cause life-threatening asphyxiation. The pathogenesis of this disorder is suspected to be related to unopposed activation of the contact pathway by the initial generation of kallikrein and/or clotting factor XII by damaged endothelial cells. The end product of this cascade, bradykinin, is produced in large amounts and is believed to be the predominant mediator leading to increased vascular permeability and vasodilation that induces typical angioedema "attacks".

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Wikipedia