CTICasesBirt-Hogg-Dubé Syndrome
Imaging findings
Chest radiograph and chest CT demonstrate extensive, bilateral, thin-walled pulmonary cysts with a strong basal and subpleural predominance. Many of these cysts run along pleural surfaces and interlobular septa or show a perivascular distribution, with some pulmonary vessels running directly through them.
Key takeaways
Birt-Hogg-Dubé syndrome is an autosomal dominant condition characterized by pulmonary cysts, recurrent spontaneous pneumothoraces, renal cell neoplasms, and fibrofolliculomas of the skin. While classic cysts are elongated, biconvex, and located in the subpleural lower lobes, the quantity and distribution of cysts can occasionally be much more extensive and atypical, raising a differential diagnosis that includes lymphoid interstitial pneumonia.
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