CTICasesBirt-Hogg-Dubé Syndrome
Imaging findings
CT shows multiple bilateral, basal-predominant, subpleural floppy lung cysts, some with adjacent pulmonary vessels. The patient has multiple facial papules (fibrofolliculomas) previously misdiagnosed as contact dermatitis, confirmed by skin biopsy.
Key takeaways
Birt-Hogg-Dubé syndrome is a rare autosomal dominant genetic disorder characterized by fibrofolliculomas, renal tumors, and lung cysts. The cysts have a characteristic basal-predominant, floppy, subpleural appearance that allows for diagnostic confidence.
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