Lupus-Erythematosus-Like Disease in Three Unrelated Women with Hereditary Angioneurotic Edema

Excerpt

Inherited deficiencies of components of complement have been reported in association with connective tissue disorders, including lupus erythematosus. In hereditary angioneurotic edema serum the fourth (C4) and sometimes the second (C2) components of complement are readily depleted largely because of the activation of C1 that occurs in this hereditary deficiency of C1 inhibitor (1). Three patients with hereditary angioneurotic edema and lupus-erythematosus-like disease are described below.

Patient 1. A clinical diagnosis of hereditary angioneurotic edema made at age 23 was supported by virtual absence of serum C1 inhibitor function and antigens. She developed a "butterfly rash" on her face and