Birt-Hogg-Dubé syndrome: a rare cause of pulmonary cysts.

2Department of Surgery, Vancouver General Hospital and University of British Columbia, Vancouver, BC, V5Z 1M9 Canada. irt-Hogg-Dubé syndrome is a rare autosomal-dominant inherited disorder characterized by the presence of firm facial papules that on biopsy are shown to represent fibrofolliculomas [1–3]. Patients with Birt-HoggDubé syndrome have a high predisposition to malignant renal tumors, which are often bilateral and multifocal [2–4]. Several studies have shown that the prevalence of bullous emphysema, thin-walled cysts, and spontaneous pneumothorax is increased in these patients [2, 3, 5]. The description of the pulmonary complications has been limited to radiographs and published in the dermatologic literature [2, 3, 5]. We report the radiographic and high-resolution CT findings in a 54-year-old woman with Birt-Hogg-Dubé syndrome and history of renal cell carcinoma. The pulmonary abnormalities consisted of multiple thin-walled cysts measuring 1–5.5 cm in diameter and involving mainly the lower lobes.