CTICasesBirt-Hogg-Dubé Syndrome (Severe Presentation)
Imaging findings
Chest CT demonstrates extremely numerous, bilateral, thin-walled pulmonary cysts of variable sizes, involving all lung zones but maintaining a lower lobe and subpleural gradient. Typical elongated cysts along the fissures are visible alongside more atypical, larger cysts.
Key takeaways
This severe genetic-proven case of Birt-Hogg-Dubé syndrome demonstrates how the number of cysts can sometimes be so overwhelming that they mimic lymphangiomyomatosis or Langerhans cell histiocytosis, obscuring the typical subpleural and septal sparing pattern usually seen in classic presentations.
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