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

COŞKUNER M. B., ÖZLÜ T., BÜLBÜL Y.

TUBERKULOZ VE TORAK-TUBERCULOSIS AND THORAX, cilt.67, sa.3, ss.225-230, 2019 (ESCI) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 67 Sayı: 3
  • Basım Tarihi: 2019
  • Doi Numarası: 10.5578/tt.68541
  • Dergi Adı: TUBERKULOZ VE TORAK-TUBERCULOSIS AND THORAX
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.225-230
  • Anahtar Kelimeler: Histiocytosis, Erdheim-Chester disease, lung
  • Karadeniz Teknik Üniversitesi Adresli: Evet

Özet

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.