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ANGIOEDEMAS can be classified as IgE-mediated, hypocomplementemic, or idiosyncratic.1 More specific classifications include angioedema related to physical stimuli, such as pressure, and idiopathic varieties.2 Eosinophilia in peripheral blood or tissue is not usually associated with angioedema.3, 4 An exception is the case described by Miller and Pepper,5 in which the patient experienced attacks of convulsions and angioedema involving the face, arms, and neck. At the peak of an attack, this patient's weight increased by 1.8 to 3.6 kg. During attacks, the leukocyte count increased to 44,000 per microliter, with 85 per cent eosinophils.
We report on four cases of episodic angioedema associated with eosinophilia, all in white patients. The attacks were accompanied by urticaria, fever, marked eosinophilia, and 10 to 18 per cent increases in weight. Eosinophils degranulated in the dermis, and the granule major basic protein6 caused a wheal-and-flare reaction, indicating its ability to activate dermal mast cells.
Case Reports
Patient 1
In 1947, when he was 16 years old, Patient 1 had begun to have recurrent attacks of periorbital angioedema and hives that started with itching and whealing followed by swelling. They occurred monthly and were so regular each time that the patient made no plans for that week. He had leukocytosis (26,900 leukocytes per microliter, 60 per cent eosinophils); the sedimentation rate was 62 mm in one hour. A muscle-biopsy specimen was negative for Trichinella spiralis larvae. The bone marrow was densely packed with immature eosinophils. In 1949 he was evaluated at the Mayo Clinic during an attack. Physical examination revealed swelling of the hands and feet and excoriated areas on the legs. The leukocyte count was 14,100 per microliter (62.5 per cent eosinophils).
The attacks continued regularly until age 28 and then stopped. At age 46 they recurred at monthly intervals, with weight gains of 4.5 to 6.8 kg. Treatment with hydroxyzine and cyproheptadine was not helpful. The leukocyte count was 9200 per microliter (30 per cent eosinophils). The alkaline phosphatase level was elevated (267 U per liter; normal value, 90 to 239), as were the levels of total serum protein (9 g per deciliter; normal, 6.3 to 7.9) and gamma globulin (3.25 g per deciliter; normal, 0.7 to 1.6). Other laboratory data are listed in Table 1. The patient...