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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Birt-Hogg-Dube syndrome


Other Names for this Disease

  • BHD
  • BHD syndrome
  • Birt Hogg Dube syndrome
  • Fibrofolliculomas with trichodiscomas and acrochordons
  • Hornstein-Knickenberg syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

What is the best way to treat the cysts that develop on the lungs as a result of this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might lung cysts associated with Birt-Hogg-Dube syndrome be treated?

At the time of diagnosis of Birt-Hogg-Dube (BHD) syndrome, a computed tomography (CT) scan, or high resolution CT scan if available, should be done to determine the number, location, and size of any cysts in the lungs.[1]  There is no recommended management of the lung cysts.  Lung cysts related to BHD have not been associated with long-term disability or fatality.[2]  The main concern is that the cysts may increase the chance of developing a collapsed lung (pneumothorax).

If an individual with BHD experiences any symptoms of a collapsed lung - such as chest pain, discomfort, or shortness of breath - they should immediately go to a physician for a chest x-ray or CT scan.[1]  Therapy of a collapsed lung depends on the symptoms, how long it has been present, and the extent of any underlying lung conditions.[2]  It is thought that collapsed lung can be prevented by avoiding scuba diving, piloting airplanes, and cigarette smoking.[2][3]

Individuals with BHD who have a history of multiple instances of collapsed lung or signs of lung disease are encouraged to see a lung specialist (pulmonologist).[3]
Last updated: 6/27/2014

References
Other Names for this Disease
  • BHD
  • BHD syndrome
  • Birt Hogg Dube syndrome
  • Fibrofolliculomas with trichodiscomas and acrochordons
  • Hornstein-Knickenberg syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.