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  • Title: Large mediastinal mass in a 15-year-old boy.
    Author: Gohar Ali M, Zubairi ABS, Qamar FN.
    Journal: BMJ Case Rep; 2018 Nov 08; 2018():. PubMed ID: 30413445.
    Abstract:
    Hyperimmunoglobulin E syndrome is a rare multisystem inherited disorder characterised by high serum IgE levels, skin disorder causing eczema, dermatitis, recurrent staphylococcal infections and pulmonary infections and various skeletal and connective tissue abnormalities. Common presentation is with recurrent skin and sinopulmonary infections. Several features unrelated to immune system such as characteristic facial features, hyperextensibility of joints, multiple bone fractures and craniosynostosis have been described in the literature. We describe a rare presentation of this disease with invasive aspergillosis presenting as mediastinal mass with extension to mediastinal structures and pulmonary vasculature.
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