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This is an inherited disorder affecting the immune system.
The abnormal level of a protein called C1 inhibitor causes the affected
individual to develop painless and non-itchy swelling of the skin, affecting
also the airway as well as the abdominal organs.
The skin swelling is most commonly seen in the face. It can also be seen in
other areas like the arms, hands, legs, feet, genitals, and buttocks. There
are no hives on the skin thus differentiating it from urticaria.
The presence also of swelling in the intestines and other abdominal organs
like the stomach causes the individual to present with abdominal pain
described either as colicky or crampy. This may be accompanied by vomiting
which may lead to dehydration or shock.
The condition is sometimes accompanied by laryngeal edema causing a sudden
hoarseness. There can be airway obstruction making the condition
life-threatening.
The attacks are usually precipitated by stress or anxiety, most often by
trauma and they are usually more severe during the second decade of life.
Antihistamines has limited role in the treatment of hereditary angioedema.
It is important to avoid the precipitating factors to prevent recurrence of
attack. Once an individual present with the signs and symptoms, bring him
immediately to the emergency room.
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