HIPER-IGE SYNDROME - NEW DIAGNOSTIC AND THERAPEUTIC ASPECTS - REPORT OF 3 CASES

Three female patients, 46, 34, and 19 years old, diagnosed of hyper-Ig E syndrome are reported. The most relevant clinical findings are recur rent sinopulmonary tract infections, cold staphylococcal abscesses and chronic dermatitis. All patients presented elevated serum IgE levels (> 3,000 U/ml) and blood eosinophilia (> 0.6 X 10(9) cel/l). Two patie nts presented impaired antibody forming capacity to tetanus and pneumo coccal antigens; one of these patients also had low serum IgG2 levels. After initiation of the intravenous gammaglobulin therapy, a marked i mprovement of infectious problems was observed. The controversial path ophysiology of this syndrome, the antibody deficiency present in some patients and the rationale for intravenous gammaglobulin therapy are d iscussed.