Erdheim-Chester disease with prominent pulmonary involvement associated with eosinophilic granuloma of mandibular bone

M. Kambouchner, T. V. Colby, C. Domenge, J. P. Battesti, P. Soler, A. Tazi

Research output: Contribution to journalArticle

57 Scopus citations

Abstract

We report a patient with eosinophilic granuloma localized to the left mandible who was subsequently shown to have Erdheim-Chester disease involving the lower extremities, omentum and lung. The diagnosis of eosinophilic granuloma was based on the presence of typical CD1a+ Langerhans' cell granulomas in a biopsy of mandible. The diagnosis of Erdheim-Chester disease was established on the basis of the pattern of radioisotopic uptake by long bones, seen on a technetium bone scan, and the presence of characteristic histopathological features in biopsies of lung and peritoneum. The pathological findings in lung were compatible with the abnormalities observed by tomodensitometry, but strikingly different from those seen in Langerhans' cell granulomatosis. The differences in the histological features of pulmonary involvement seen in the two diseases, and the possible relationship between Langerhans' cell granulomatosis and Erdheim-Chester disease, are discussed.

Original languageEnglish (US)
Pages (from-to)353-358
Number of pages6
JournalHistopathology
Volume30
Issue number4
DOIs
StatePublished - Jan 1 1997

Keywords

  • Bony sclerosis
  • Erdheim-Chester disease
  • Interstitial lung disease
  • Langerhans cells

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology

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