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Abstract

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Article in Japanese

Case Report

A case of vascular Ehlers-Danlos syndrome presenting as spontaneous hemothorax and recurrent pneumothorax

Yoshiko Ishiokaa  Toshihiro Shiratorib  Kageaki Taimaa  Masamichi Itogaa  Shujiro Hayashic  Sadatomo Tasakaa 

aDepartment of Respiratory Medicine, Hirosaki University Graduate School of Medicine
bDepartment of Respiratory Medicine, Hachinohe City Hospital
cDepartment of Dermatology, Dokkyo Medical University School of Medicine

ABSTRACT

A 22-year-old man with a history of spontaneous pneumothorax visited a local hospital complaining of dyspnea. He was diagnosed with hemothorax by computed tomography (CT) with a consolidation and pleural effusion in the right lung. After 6 days, pleural effusion had increased, suggesting that bleeding continued. He was referred to our hospital for further examination. A thoracoscopic examination was performed, but no apparent bleeding was observed. Because his condition had improved, the patient was discharged. One month later, however, he developed left pneumothorax, requiring surgery. Since the surgical specimen was fragile and difficult to hold, we suspected vascular Ehlers-Danlos syndrome (vEDS) that was also suggested by his medical and family history. A genetic test revealed a COL3A1 mutation, which confirmed the diagnosis. vEDS should be considered in a case of repeated pneumothorax and hemothorax in a young non-smoking patient.

KEYWORDS

Vascular Ehlers-Danlos syndrome (vEDS)  Pneumothorax  Hemothorax  Type III collagen 

Received 15 Jun 2020 / Accepted 21 Jul 2020

AJRS, 9(6): 473-476, 2020

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