Pneumatocele complicating hyperimmunoglobulin E syndrome (Job's Syndrome)

Ann Thorac Surg. 1992 Dec;54(6):1206-8. doi: 10.1016/0003-4975(92)90100-i.

Abstract

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aspergillosis / diagnosis
  • Aspergillosis / etiology
  • Aspergillosis / surgery
  • Bronchial Fistula / etiology
  • Bronchial Fistula / surgery
  • Child
  • Cysts / complications
  • Cysts / diagnostic imaging*
  • Cysts / pathology
  • Humans
  • Inflammation
  • Job Syndrome / complications*
  • Job Syndrome / diagnosis
  • Lung Abscess / etiology
  • Lung Abscess / surgery
  • Lung Diseases / complications
  • Lung Diseases / diagnostic imaging*
  • Lung Diseases / pathology
  • Lung Diseases, Fungal / diagnosis
  • Lung Diseases, Fungal / etiology
  • Lung Diseases, Fungal / surgery
  • Male
  • Necrosis
  • Pleural Diseases / etiology
  • Pleural Diseases / surgery
  • Radiography