A rare disease; Erdheim-Chester disease presenting with spontaneous pneumothorax

COŞKUNER M. B., ÖZLÜ T., BÜLBÜL Y.

TUBERKULOZ VE TORAK-TUBERCULOSIS AND THORAX, vol.67, no.3, pp.225-230, 2019 (ESCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 67 Issue: 3
  • Publication Date: 2019
  • Doi Number: 10.5578/tt.68541
  • Journal Name: TUBERKULOZ VE TORAK-TUBERCULOSIS AND THORAX
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.225-230
  • Keywords: Histiocytosis, Erdheim-Chester disease, lung
  • Karadeniz Technical University Affiliated: Yes

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis of unknown etiology, it is characterized by organ infiltration of foamy histiocytes. Diagnosis of ECD involves the analysis of histiocytes in tissue biopsies: these are typically CD68+ CD1a-. These characteristic histiocytes may be found in almost any tissue in cases of ECD. We present an interesting case of ECD that was presented by spontaneous pneumothorax and neurological involvement.