Hyper-IgE syndrome. Presentation of three cases.
Three cases of Hyper-IgE syndrome are herein described, corresponding to patients aged six, twelve and 4 years, who from infancy, had suffered a variety of recurrent pulmonary infections, staphylococcal cold abscesses and chronic dermatitis. IgE counts were superior to 2.000 u/ml and eosinophilia surpassed 0.6 x 10(9) cells/l in all three cases. Deficits in the mytogen and chemotaxis responses were also seen. These patients were treated with I.V. gammaglobulin, anti-biotherapy and ascorbic acid. The possible physiopathology of this syndrome and its association with different alterations to pulmonary immunity are discussed in this paper. Hyper-IgE Syndrome is characterized by recurrent dermal and pulmonary infections (pneumonia and abscesses), dominated by elevated IgE levels, and in 1974, this Syndrome was included within the "immunodeficiencies" group, and Hill and Quie described this as a process involving recurrent bacterial infections and eczemas accompanied by elevated IgE levels, together with a defect in the mobility of neutrophils. Previously, in 1966, Job described this Syndrome in fair-skinned, red-headed young girls, suffering from eczemas and recurrent cold abscesses of staphylococcal ethiology, found on the skin, in subcutaneous tissue cells and lymph nodes. Later, Dr. Buckley defined this Syndrome by an increase in serum IgE levels, chronic dermatitis and severe repetitive infections. This Syndrome today, is still an immunological mystery, defined by two clinically compatible criteria and an increase in IgE. Other analytical abnormalities, including neutrophilic mobility, the appearance of IgE antibodies anti-Candida or staphylococcus, are non-constant findings.
de la Torre Morín
F
F
Allergy & Immunology Service, Ntra. Sra. de la Candelaria Hospital, Tenerife.
García Robaina
J C
JC
Bonnet Moreno
C
C
Fonta
G L
GL
D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D004802:Eosinophilia / Q000150:complications / Q000175:diagnosis; D005260:Female; D006801:Humans; D007589:Job Syndrome / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D008297:Male