A 25 year old women presents with a history of recurrent shortness of breath and severe wheezing. Laboratory studies demonstrate that she a has a deficiency of C1 inhibitor, an esterase inhibitor that regulates the activation of the classical complement pathway. What is the diagnosis?

Deficiency of C1 inhibitor, with excessive clearage of C4 and C2 by CLS, is associated with the syndrome of hereditory angioedema. This disease is characterised by episodic, painless, non pitting edema of soft tissue. It is the result of chronic complement activation, with the generation of a vasoactive peptide form C2 and may be life threatening because of the occurence of laryngeal edema. Chronic granulomatous disease is due to a hereditory deficiency of NADPH oxidase Myeloperoxidase deficiency increases susceptibility to infections with candida. Selective IgA deficiency and Wiskott Aldrich syndrome are congenital immunodeficiency disorders associated with defects in Lymphocyte Function.