Sammanfattning
Hereditary angioedema is a rare, life-threatening disease characterized by episodic self-limiting swellings of the skin, mucous membranes and underlying tissues due to a deficiency of functional complement C1 esterase inhibitor. This article describes the clinical features, pathophysiology, differential diagnoses and treatment possibilities of hereditary angioedema. In patients with recurrent angioedema without urticaria and/or abdominal attacks without explanation, it is important to rule out complement C1 esterase inhibitor deficiency. Treatment of hereditary angioedema is different from that for other types of angioedema, as these patients do not respond satisfactorily to antihistamine agents, steroids or epinephrine. The multifaceted symptoms bring these patients into contact with many different medical and surgical specialties, which is why close collaboration among hospitals, general practitioners and dentists is essential.