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Start free with EleplanPulmonary Langerhans cell histiocytosis
ORPHA:687733Clinical subtype
Also called Single-system pulmonary Langerhans cell histiocytosis · Single-system pulmonary histiocytosis X · PLCH · Single-system Langerhans cell granulomatosis
What it is
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.
Key facts
- Inheritance
- Not applicable
- Classified as
- Clinical subtype
Recorded for the broader condition
- Prevalence
- 1-9 / 100 000 (Europe)Langerhans cell histiocytosis
- Age of onset
- All agesLangerhans cell histiocytosis
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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