Hereditary angioedema
Hereditary angioedema Causes
Precipitating factors of attacks may include trauma (especially dental trauma), menstruation, anxiety, infection, exercise, alcohol consumption, and stress. Medications (examples like estrogen, ACE inhibitors, angiotensin II type 1 receptor antagonists) have also been shown to induce attacks.
Hereditary angioedema Definition
Angioedema, also known as Quincke's edema, is the rapid swelling (or edema) of the skin, mucosa and submucosal tissues. Aside from the common form, mediated by allergy, it has been reported as a side effect of some medications, specifically ACE inhibitors.
Hereditary angioedema Diagnosis
The diagnosis is done on the clinical picture. Routine blood tests (such as complete blood count, electrolytes, renal function, liver enzymes) are usually performed. Mast cell tryptase levels may be heightened if the attack was due to an acute allergic (anaphylactic) reaction.
Hereditary angioedema Symptoms and Signs
The skin of the face, typically around the mouth, and the mucosa of the mouth and/or throat, as well as the tongue, swell up over the period of minutes to several hours. The swelling can also arise elsewhere, typically in the hands. This swelling can be quite itchy. There may also be slightly reduced sensation in the affected areas due to compression of the nerves. Urticaria (or hives) may occur simultaneously.
Hereditary angioedema Treatment
The aim of acute treatment is to stop progression of the edema as quickly as possible, which can be life-saving, particularly if the swelling is in the larynx. In an emergency, fresh frozen blood plasma, which also contains C1-INH, can also be utilized. Fresh Frozen Plasma (FFP) can also be used as an alternative to C1-INH concentrate.
Drugs used for treatment of Hereditary angioedema
Danazol