Familial Nonallergic Angioneurotic Edema (C'1 Esterase Inhibitor Deficiency). Presented by DR. K. FRANK AUSTEN.
A 23-year-old white man is presented from the Massachusetts General Hospital with an intermittent dermatosis of 19 years' duration.
Beginning at 4 years of age, the patient has had episodes of localized angioedema of the trunk and extremities, usually associated with severe, crampy abdominal pain and vomiting. Attacks occur regularly at two to four week intervals and can be precipitated by emotion or trauma. The patient's mother and maternal grandmother have the same syndrome.
On Nov 30, 1963, there was pitting edema of the neck, face, and lips. On April 25, 1964, there was pitting edema of the right hand to the wrist, with a sharp, erythematous border (Fig 1 and 2).
A complete blood count and urine analysis were normal (no eosinophilia); serum C'2 was absent on November 30, and C'1-esterase inhibitor was absent in
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