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Ann Thorac Surg 1992;54:1206-1208
© 1992 The Society of Thoracic Surgeons

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Articles

Pneumatocele complicating hyperimmunoglobulin E syndrome (Job's syndrome)

Departments of Surgery, Pediatrics (Division of Pulmonary Medicine), and Pathology, Children's Hospital and Harvard Medical School, Boston, Massachusetts, USA

Accepted for publication February 28, 1992.

^_* Address reprint requests to Dr Shamberger, Department of Surgery, Children's Hospital, 300 Longwood Ave, Boston, MA 02115.

The case of a boy with hyperimmunoglobulin E syndrome or Job's syndrome is presented to demonstrate the occurrence of pneumatoceles in this syndrome as well as their unusual natural history and failure to spontaneously resolve. Surgical resection was required for two complications in this patient, persistent bronchopleural fistula and a pulmonary abscess that destroyed one lung and required pneumonectomy. Pathologic examination of the specimens demonstrated the wall of the cysts consisted of granulation tissue with chronic active inflammation surrounded by infarcted pulmonary parenchyma with coagulative necrosis. The mechanism responsible for increased immunoglobulin E production in this syndrome is unknown, as is the manner in which elevated immunoglobulin E levels impair normal immune function.

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