Hyper-IgE, Pelger-Huet anomaly and chromosome 22s+ in an infant with skin abscesses

We describe a 10-month-old male who suffered from atopic eczema, lymphadenitis of the neck and staphylococcal deep skin abscesses which had required surgical treatment. Laboratory findings revealed peripheral blood eosiniphilia, extremely elevated serum IgE level, congenital Pelger-Huet (P-H) anomaly and chromosome karyotype 46, XY with enlarged satellite of chromosome 22 (22s⁺). Levels of other immunoglobulin classes and lymphocyte functions were within normal ranges as compared with normal subjects. Enzymatic and functional examinations of neutrophils also appeared normal. The familial study of this patient revealed that 22s⁺ was inherited from the maternal pedigree and seemed to be associated with P-H anomaly. Clinical and immunological features suggest that he suffered from atopic eczema as well as P-H anomaly rather than the kind of immunodeficiency disease known as 'hyperimmuno-globulinemia E syndrome.' 22s⁺ did not seem to exert an important effect on his clinical disorders.