Hyperimmunoglobulinaemia E and defective chemotaxis associated with recurrent bacterial infections and asthma

A new case of Hyperimmunoglobulinaemia E syndrome in a boy is reported. Allergic reactions started at 6 mth of age with an 'obstructive bronchitis', which was followed by five severe attacks of asthma in the course of four years. Recurrent bacterial infections started at 9 mth with an abscess of the scalp and continued with recurrent cutaneous infections, abscesses in several sites, adenitis and two pleuropneumonias with residual gross pneumatoceles and lung abscesses. The patient died at 4 yr of age after a bronchopneumonia. Immunological and granulocytic function studies were performed several times. They showed high levels of IgA, IgG and IgM, with normal complement factors and cellular immunity. IgE was highly increased in five determinations; RAST was positive with several antigens. Hypereosinophilia was almost constant. Granulocytes had defective chemotaxis of cellular origin. Phagocytosis, NBT reduction, superoxide formation, iodination and leukocyte enzyme activities were found normal. In this case, the probable hereditary base (mother with asthma and skin and respiratory infections), the respiratory type of the allergic reaction (asthma) and the severe evolution of the lung infections must be emphasized. The hypotheses about the physiopathology of this syndrome are discussed and references to the therapeutical approaches are made.