New diagnostic and therapeutic aspects of hyper-IgE syndrome. Report of 3 cases
Matamoros Florí, N.; Milá Llambí, J.; Hernández González, M.; Juliá Benique, M.R.
Medicina Clinica 101(4): 138-140
1993
ISSN/ISBN: 0025-7753 PMID: 8355543 Document Number: 406370
Three female patients, 46, 34, and 19 years old, diagnosed of hyper-IgE syndrome are reported. The most relevant clinical findings are recurrent 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 patients presented impaired antibody forming capacity to tetanus and pneumococcal antigens; one of these patients also had low serum IgG2 levels. After initiation of the intravenous gammaglobulin therapy, a marked improvement of infectious problems was observed. The controversial pathophysiology of this syndrome, the antibody deficiency present in some patients and the rationale for intravenous gammaglobulin therapy are discussed.