pulmonary langerhans PLCH etiology, patient population
- related: pulmonary langerhans histiocytosis PLCH
- tags: #literature #boards
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Pulmonary Langerhans cell histiocytosis (previously histiocytosis X or eosinophilic granuloma) is a rare form of interstitial lung disease that primarily affects young adults.
90% of affected are cigarette smokers.1
PLCH is a rare sporadic cystic lung disease of unknown etiology, seen in young adult smokers, that is characterized by the infiltration and destruction of the wall of distal bronchioles by CD1a-positive Langerhans-like cells.2