Background Angioedema is a highly heterogeneous group of conditions and is characterized by sudden, pronounced swelling of the lower dermis and subcutaneous. Because of its frequent coexistence with urticaria, it is often classified in the same manner as urticaria. However, it also includes categories not associated with urticaria. Angioedema without urticaria is characterized by hereditary and...

BACKGROUND Hereditary angioedema with C1 inhibitor deficiency is characterized by recurrent, unpredictable swelling episodes caused by uncontrolled plasma kallikrein generation and excessive bradykinin release resulting from cleavage of high-molecular-weight kininogen. Lanadelumab (DX-2930) is a new kallikrein inhibitor with the potential for prophylactic treatment of hereditary angioedema with...

BACKGROUND The importance of acquiring comprehensive epidemiological and clinical data on hereditary angioedema has increasingly caught the attention of physicians and scientists around the world. The development of networks and creation of comprehensive policies to improve care of people suffering from rare diseases, such as hereditary angioedema, is a stated top priority of the European Union...

Angioedema is a potentially life-threatening disease cate-gorised into several forms such as allergic, hereditary, acquired autoimmune and idiopathic. Allergic angioedema is dependent on histamine release, whereas mutations in the C1-inhibitor and factor XII genes have been described in hereditary angioedema (1, 2). Bradykinin-induced acquired angioedema is often caused by antibodies to C1-inhi...

INTRODUCTION — Acquired deficiency of C1 inhibitor (C1-INH), also called acquired angioedema (AAE), is a rare syndrome of recurrent episodes of angioedema, without urticaria, which is associated with B cell lymphoproliferative disorders in some patients [1,2]. Angioedema typically affects the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. The swelling is self-limi...