Idiopathic angioedema is a broad class of angioedema, a type of swelling that can occur anywhere on the body, but most frequently appears around the lips and eyes. Less commonly, the tongue or the mucous membranes of the throat and the mouth can swell — which, if severe, can be life-threatening.
Idiopathic angioedema is a chronic condition where sudden attacks of swelling happen periodically.
Symptoms of idiopathic angioedema
Swelling in this form of angioedema is similar to an allergic reaction, and is characterized by smooth welts of raised or engorged skin accompanied by a rash or hives.
However, in this case, swelling does not happen as a sudden acute reaction to an allergy-triggering food item, object, or medicine. Instead, swelling is chronic, recurring regularly on a long-term basis.
While the swelling is very visible and may be uncomfortable, it is usually not dangerous unless it happens in the throat, mouth or neck, and is severe enough to block the airway and block normal breathing. In such cases, the condition can become life-threatening and requires immediate medical attention.
Causes of idiopathic angioedema
The cause of recurring chronic angioedema may be difficult to determine.
Individuals may have an allergy to something commonly found in low levels in their environment (such as penicillin in milk or pollutants in the air). It can also occur in people who may be stressed, recovering from an illness, or, in an estimated 30-50 percent of cases, may have an underlying autoimmune disease.
Treatment of idiopathic angioedema
Low levels of C1 inhibitors, an important immune system protein found in the blood, are associated with angioedema. Boosting this protein’s levels with the medications ecallantide and icatibant can help prevent angioedema attacks even if the trigger is unknown.
Cannabis, which has been shown to have anti-inflammatory properties, may also be an option to treat idiopathic angioedema. In a 2015 case study, a patient with chronic idiopathic angioedema achieved long-term relief from his symptoms with medical cannabis. However, more research is needed before cannabis can be used in the clinic as a treatment for idiopathic angioedema.
In a life-threatening situation, where swelling in the throat or mouth has blocked the airway, an Epi-pen (epinephrine) can be used to quickly treat the individual.
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