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.