28 -year-old male with only minimal reported nicotine dependence presents with dyspnea and cough

CXR (PA and Lateral) shows no acute cardiopulmonary disease with mild interstitial prominence

CT scan shows multiple small cysts predominantly in the upper lobes. The cysts are round and air filled large and are between 5mm-8mm
These findings are consistent with Langerhans histiocytosis though the relatively minor smoking history was inconsistent with the diagnosis and thus the person was subjected to multicentric wedge biopsies.

 

CT UPPER LUNG ZONE PROMINENCE OF SMALL THIN WALLED CYSTS
LANGERHANS HISTIOCYTOSIS
Ashley Davidoff MD

Pathology revealed numerous stellate nodules which suggested “burnout pulmonary Langerhans cell histiocytosis though immunostains including Langerin, CD1a and S100 were negative thus failing to confirm the diagnosis Other findings of histiocytic aggregates raised the possibility of an inhalational hypersensitivity pneumonitis.

References and Links

• TCV

  • • Langerhans Cell Histiocytosis Introduction
      • Atlas
      • 015Lu Langerhans vs Inhalational Drug Cystic Disease 27M
      • 016Lu Langerhans Histiocytosis 53F
      • 062Lu Langerhans Histiocytosis
      • 80Lu Langerhans  Histiocytosis with Adenocarcinoma
      • 114Lu Pulmonary Langerhans Histiocytosis (PLHC)
      • 162Lu Pulmonary Langerhans Cell Histiocytosis and Treated TB