A 15-year-old girl with abnormal findings detected on a medical check-up chest
x-ray film was admitted to our center. High-resolution computed tomography, performed upon hospitalization, demonstrated panlobular nodular darkening in left lung fields, and an expanding, blended, map-like darkening near the pleura. Since a Grocott
stain-positive
cyst was confirmed histopathologically by transbronchial lung biopsy, the patient was given a diagnosis of
Pneumocystis carinii pneumonia.
Drug therapy was initiated with sulfamethoxaxole
trimethoprim (Baktar), and on the 58th day, chest CT confirmed that the darkening observed at admission had virtually disappeared. Underlying diseases, such as
AIDS,
malignant lymphoma and secondary immunodeficiency caused by
immunosuppressive agents or adrenocorticosteroids, were excluded as the cause of P. carinii
pneumonia based on clinical/laboratory findings. Under the suspicion of the possibility of primary immunodeficiency, various immunological competence tests were performed. However, no abnormal findings indicating cell-mediated immunity, humoral immunity,
complement immune function, neutrophil phagocytic capacity, or bactericidal capacity were recognized. Since significant increase of serum
IgE suggested
hyper-IgE syndrome,
IgE antibody specific to
Staphylococcal enterotoxin A and B, and the
exotoxins of Staphylococcus aureus were measured with positive results. Since all three diagnostic criteria for
hyper-IgE syndrome (i.e., high serum
IgE values, positive
IgE antibody specific to
Staphylococcal enterotoxin and
recurrent infection) were fulfilled,
hyper-IgE syndrome was diagnosed. This is a rare case of
hyper-IgE syndrome as a result of P. carinii
pneumonia.