ABSTRACT
In a typical clinical practice it is not uncommon to see patients with urticaria/angioedema who report relati ves wi th urticaria/angioedema, suggesting that there is a genetic intluence on these disorders. However, the diagnosis of true hereditary urticaria/angioedema is rare, because most diseases do not follow a classic hereditary pattern. In the first section of this chapter we will discuss the initial diagnostic evaluation and counseling of patients with a family history of these disorders. We will also discuss models of heredity and the methods used by investigators to identify the genetic bases of diseases. In the second section, we describe a group of rare inherited systemic intlammatory diseases that present with atypical urticaria as a prominent feature. Finally, we discuss several disorders with urticaria and/or angioedema that appear to be inherited.
