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Pulmonary Langerhans cell histiocytosis

ORPHA:687733· ICD-10 C96.5

Definition

A form of Langerhans cell histiocytosis characterized by interstitial changes in the lung tissue, manifesting as a variable combination of cellular inflammation, cystic, and fibrotic lesions. Patients can have symptoms like dyspnea, cough and fever but most of them are asymptomatic, and they may undergo spontaneous remission. It is predominantly observed in young smokers or ex-smokers.

Inheritance
Not applicable