Pneumocystis carinii Pneumonia in an Infant with the Hyperimmunoglobulinemia E Syndrome

A 4-month-old female presented with "cold" groin abscesses, otitis, and interstitial pneumonia. Open lung biopsy showed extensive Pneumocystis carinii pneumonitis. An immunologic workup revealed exaggerated blood levels of immunoglobulin E (over 2000 IU/ml by 10 months of age) and abnormal polymorphonuclear chemotaxis, both characteristic of the hyperimmunoglobulinemia E (HIE) syndrome. No well-defined lymphocytic defect known to predispose to opportunistic infection was discovered in this patient. We believe that our patient represents the first reported association of P. carinii pneumonia in a patient with HIE syndrome. This finding has implications for the spectrum of immunologic aberrations that may predispose to P. carinii pneumonia and also should raise consideration for P. carinii as a potential etiologic agent of pneumonitis in patients with HIE syndrome.