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Langerhans Cell Histiocytosis is a rare disease characterized by proliferation of abnormal cells resembling epidermal dendritic Langerhans cells. According to recent findings, LCH cells exhibit a unique transcription profile that separates them from plasmacytoid and myeloid dendritic cells, but also from epidermal Langerhans cells, indicating a distinct dendritic cell entity. Adults are likely to have isolated pulmonary LCH (PLCH), however extrapulmonary involvement is seen in up to 30% of cases. Adult PLCH occurs almost exclusively in smokers; it is now recognized as a form of smoking-related interstitial lung disease and can be complicated by pulmonary fibrosis and pulmonary hypertension.
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