A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome

Abstract Birt-Hogg-Dubé syndrome, initially described in 1977, is an autosomal dominant inherited condition characterised by basal pulmonary cysts often resulting in pneumothorax, renal tumours and cutaneous involvement. Lung cysts have been described in up to 90% of patients with a corresponding ri...

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Bibliographic Details
Published in:Respiratory medicine case reports 2016-01, Vol.18, p.90-92
Main Authors: Minnis, P, Riddell, P, Keane, M.P
Format: Article
Language:English
Subjects:
Birt-Hogg-Dubé syndrome
Case Report
Cutaneous lesions
Internal Medicine
Pneumothorax
Pulmonary cysts
Pulmonary/Respiratory
Renal tumors
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Summary:Abstract Birt-Hogg-Dubé syndrome, initially described in 1977, is an autosomal dominant inherited condition characterised by basal pulmonary cysts often resulting in pneumothorax, renal tumours and cutaneous involvement. Lung cysts have been described in up to 90% of patients with a corresponding risk of pneumothorax of 50 times greater than the normal population. We describe here a case of Birt-Hogg-Dubé diagnosed in the 9th decade of life and discuss the radiological findings and clinical implications.
ISSN:2213-0071
2213-0071
DOI:10.1016/j.rmcr.2016.05.007