Hereditary angioedema

Hereditary angioedema is a rare genetic disorder characterized by recurrent, self-limiting episodes of nonpitting, nonpruritic subcutaneous and submucosal swelling occurring without urticaria

Hereditary angioedema is a rare genetic disorder characterized by recurrent, self-limiting episodes of nonpitting, nonpruritic subcutaneous and submucosal swelling occurring without urticaria

The disease predominantly involves the skin of the face and extremities, the gastrointestinal tract—presenting with severe abdominal pain, nausea, and vomiting—and the upper respiratory tract, where laryngeal involvement can cause life-threatening airway obstruction

The disease predominantly involves the skin of the face and extremities, the gastrointestinal tract—presenting with severe abdominal pain, nausea, and vomiting—and the upper respiratory tract, where laryngeal involvement can cause life-threatening airway obstruction

Pathophysiologically, the condition is most commonly driven by deficient or dysfunctional C1 esterase inhibitor activity, which leads to unregulated contact-system activation, excessive bradykinin generation, and dramatically increased vascular permeability

Pathophysiologically, the condition is most commonly driven by deficient or dysfunctional C1 esterase inhibitor activity, which leads to unregulated contact-system activation, excessive bradykinin generation, and dramatically increased vascular permeability