Bullous lung disease or bullous emphysema?

Parenchymal emphysema measured by CT lung density correlates with lung function in patients with bullous disease.

In subjects with chronic obstructive pulmonary disease (COPD) computed tomographic (CT) lung density correlates with direct pathological measurements of the size of the distal airspaces, as well as with measurements of airflow limitation and impairment of the diffusing capacity. Thus, CT lung density can be used to quantify emphysema in life. We wanted to assess the use of CT scanning to detect...

Giant bullous emphysema in the right middle lobe.

Giant bullous emphysema, or vanishing lung syndrome, typically occurs in young, thin male smokers with large bullae in one or more upper lobes occupying at least one-third of the hemithorax. We present here a rare case of giant bullous emphysema in a mid-age nonsmoking female who was seen for progressive shortness of breath and cough. Chest computed tomography found a giant bulla in the middle ...

Bullous Emphysema of the Lung

Recurrent spontaneous pneumothoraces and bullous emphysema. A novel mutation causing Birt-Hogg-Dube syndrome

Birt-Hogg-Dube syndrome (BHDS) is a rare form of classically cystic lung disease that may present with spontaneous pneumothorax. The associated skin manifestations (fibrofolliculomas) are not always present. This article describes a case of spontaneous pneumothorax secondary to bullous emphysema in an otherwise healthy gentleman caused by a novel mutation in the folliculin (FLCN) gene.

Bullous emphysema as first presentation of Ehlers-Danlos syndrome in monozygotic twins

Ehlers-Danlos syndrome, characterized by hyperextensible skin, hypermobile joints, and fragile vessels, is the most common heritable disorder of connective tissue and has an estimated prevalence of 1 in 5000. Pulmonary involvement with signs of lung destruction (bullous emphysema) as first presentation is unusual. We report a case of monozygotic twins 37 years old men with occasional evidence o...