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Radiology, Vol 132, 71-78, Copyright © 1979 by Radiological Society of North America

ARTICLES

Hyperimmunoglobulinemia E syndrome: radiographic observations

DF Merten, RH Buckley, PC Pratt, EL Effmann and H Grossman

Susceptibility to recurrent staphylococcal cutaneous and respiratory infections beginning in infancy associated with extreme hyperimmunoglobulinemia E is a recently described primary immunodeficiency syndrome. Other clinical features include depressed cellular immunity and deficient antibody formation. Recurrent pneumonia and cyst formation with variable persistence and expansion characterized the radiographic couse in 11 patients. Five cysts resolved with continuous antistaphylococcal therapy; 2 were resected without recurrence; and 4 persisted after surgery and/or antibiotics (2- -8 years). The cysts had dense, necrotic surfaces with fibrous walls, eosinophilic and other inflammatory cell infiltrates, and frequent, persistent, bronchial connections. Sinusitis (9/9) and mastoiditis (3/4) were also observed radiographically.


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Hyper-IgE Syndrome with Recurrent Infections -- An Autosomal Dominant Multisystem Disorder
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