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Atypical Pulmonary Langerhans Cell Histiocytosis

Imaging findings

Chest CT in a 30-year-old female smoker of marijuana and tobacco shows diffuse, thick-walled pulmonary cysts with atypical basal predominance, complicated by bilateral pneumothoraces. Biopsy confirmed Langerhans cell histiocytosis (PLCH).

Key takeaways

PLCH typically spares the lung bases. However, atypical presentations with basal predominance can occur and can be complicated by recurrent, bilateral pneumothoraces, requiring differentiation from lymphangioleiomyomatosis (LAM).

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