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Systemic Langerhans Cell Histiocytosis

Imaging findings

Chest CT in a 24-year-old female smoker shows multiple bilateral pulmonary nodules with central cavitations, with significant involvement of the lower lobes. Brain MRI shows areas of enhancement, and biopsy of a chronic leg rash confirms Langerhans cell histiocytosis.

Key takeaways

Pulmonary Langerhans cell histiocytosis (PLCH) is typically a smoking-related disease with upper and mid-lung predominance. In contrast, systemic or malignant Langerhans cell histiocytosis is a blood-borne process that frequently involves the lower lung zones due to gravity-dependent blood flow and has multisystem involvement.

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