آنژیوادم ارثی: تشخیص نامناسب،‌ درمان ناکافی و گزارش 7 مورد از یک خانواده

Hereditary angioedema is a rare disorder of complement system which is often seen with autosomal dominant hereditary. Clinical characteristics include non- pruritic and non-pitting mucocutaneous edema that could involve all parts of the body. This study reports seven cases of hereditary angioedema with classical manifestations accompanied by low function of C1INH (type 2). One death occurred due to laryngeal edema. This case study aimed at increasing the knowledge regarding hereditary angioedema, its early diagnosis and correct managements.