- Autosomal dominant
- cystic lung disease
- basal predominance
- often close to pleura and fissures
- Costophrenic anglesd
- PTX associated
- skin lesions kidney lesions (including RCC)

Imaging of Cystic Lung Disease Thomas Hartman MD Mayo Clinic Video on Imaging Cystic Lung Disease Society of Thoracic Radiology
Links and References
Lee JE et al Birt-Hogg-Dubé syndrome: characteristic CT findings differentiating it from other diffuse cystic lung diseases Diagn Interv Radiol. 2017 Sep; 23(5): 354?359.