WISKOTT-ALDRICH SYNDROME PRESENTING AS POSSIBLE ALLERGY - UNUSUAL PRESENTATION FOR AN UNUSUAL DISEASE

A 10-year-old white boy was evaluated for potential allergic rhinitis but had no laboratory evidence of IgE-mediated allergy. He also had a history of eczema since birth. Subtle historical findings, including the presence of recurrent respiratory infections, protracted herpangina, paronychia, and previous leukopenia, suggested the need for an immunodeficiency evaluation. A constellation of immunologic aberrations was observed, including alterations in immunoglobulin levels, lack of humoral antibody production to polysaccharide antigens, and depressed cell-mediated immunity. Platelet numbers were normal or elevated, but the platelet volume was invariably low. The combination of defective antibody response to polysaccharide antigens and low platelet volumes in a male child with eczema since birth is most compatible with a variant of the Wiskott-Aldrich syndrome. The presentation was not classic, since affected patients are usually infants with bleeding, thrombocytopenia, or recurrent, severe infections. This case was unique because of the advanced age (10 years) and the unusual presenting complaint (potential allergic rhinitis). Therapeutic options for this patient are discussed.