Birt-Hogg-Dubé syndrome: a little known cause of pulmonary cysts.
نویسندگان
چکیده
Dermatologists are more familiar with Birt-Hogg-Dubé syndrome than radiologists are, since the cutaneous symptoms are the dominant feature of this rare syndrome. It is an autosomal dominant genodermatosis classically composed of multiple cutaneous hamartomas, renal tumours, and pulmonary cysts, which are often forerunners of spontaneous pneumothorax. It is rarely considered in the differential diagnosis of pulmonary cysts.
منابع مشابه
An inherited cause of pneumothorax--the Birt-Hogg-Dubé syndrome.
A 38-year-old woman presented in 2004 a spontaneous pneumothorax (Figure 1a). Outcome was favorable with treatment with chest tube drainage. Eight years later, she complained of dyspnea. A computed tomography scan of the chest revealed lung cysts (Figure 1b). She presented 2–4mm, white, dome-shaped papules involving the neck, which appeared during the last 2 years. Her brother also presented mu...
متن کاملA rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome
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-Du...
متن کاملBirt-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, w...
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Birt-Hogg-Dubé syndrome is characterized by clinical manifestations such as hamartomas of the skin, renal tumors and lung cysts with spontaneous pneumothoraces. Patients with Birt-Hogg-Dubé syndrome may present with only multiple lung cysts. We report the chest computerized tomography (CT) features of three patients with Birt-Hogg-Dubé syndrome. Each patient had multiple lung cysts of various s...
متن کاملBirt-Hogg-Dubé Syndrome: Answering Questions Raised by a Case Report Published in 1962
In 1962, J.J. Collins from the United States Naval Medical Research Laboratory published an unusual case of air embolism precipitated by decompression in The New England Journal of Medicine [1962;266:595-598]. The case was unusual because it was the first where multiple pulmonary cysts were discovered after a successful recompression treatment. Although various hypotheses were put forward by th...
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عنوان ژورنال:
- Diagnostic and interventional imaging
دوره 96 1 شماره
صفحات -
تاریخ انتشار 2015