Ehlers-Danlos Syndrome with Recurrent Spontaneous Pneumothoraces and Cavitary Lesion on Chest X-ray as the Initial Complications

• Ishiguro Takashi

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Takayanagi Noboru

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Kawabata Yoshinori

  Division of Pathology, Saitama Cardiovascular and Respiratory Center

• Matsushima Hidekazu

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Yoshii Yutaka

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Harasawa Keiji

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Yamaguchi Shozaburo

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Yoneda Koichiro

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Miyahara Yousuke

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Kagiyama Naho

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Tokunaga Daido

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Aoki Fumiaki

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Saito Hiroo

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Kurashima Kazuyoshi

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Ubukata Mikio

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Yanagisawa Tsutomu

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Sugita Yutaka

  Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center

• Okita Hiroshi

  Department of Dermatology, Dokkyo Medical University

• Hatamochi Atsushi

  Department of Dermatology, Dokkyo Medical University

Abstract

A 17-year-old-man developed left-sided pneumothorax in 1995. Chest computed tomography (CT) showed a thick-walled cavity in the left lower lobe. Video-assisted thoracic surgery was performed, and pathologic findings of the resected lung showed a cavity, organizing hematoma, and a fibrous nodule. Fragility of connective tissue was suspected, and biochemical and molecular analysis showed reduction of type III collagen production and point mutation of the COL3A1 gene. The patient was diagnosed as having vascular-type Ehlers-Danlos syndrome (EDS). From 2002, the patient developed hemoptysis and bloody sputum once a year. Chest CT detected several nodules and cavities, which were regarded as hematomas with or without excretion. Several vascular changes including aneurysmal formations have been found since 2002, and an aneurysm of the left ulnar artery was resected. The patient continues to be followed regularly on an outpatient basis. We report a rare case of vascular-type EDS who developed pulmonary symptoms as an initial complication.<br>

Journal

• Internal Medicine

  Internal Medicine 48 (9), 717-722, 2009

  The Japanese Society of Internal Medicine

Keywords

• Ehlers-Danlos syndrome
• vascular type
• lung
• pneumothorax

Details

• CRID: 1390282679850365824
• NII Article ID: 130000122489
• DOI: 10.2169/internalmedicine.48.1818
• ISSN: 13497235
• ISSN: 09182918
• Web Site: http://www.jstage.jst.go.jp/article/internalmedicine/48/9/48_9_717/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2010012028
• Text Lang: en
• Data Source:
  • JaLC
  • Crossref
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed