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Pleuropulmonary pathology of vascular Ehlers–Danlos syndrome: spontaneous laceration,haematoma and fibrous nodules |
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| Authors: | Yoshinori Kawabata Akira Watanabe Shozaburo Yamaguchi Masahiro Aoshima Akira Shiraki Atushi Hatamochi Tetsuji Kawamura Takashi Uchiyama Atushi Watanabe Yuh Fukuda |
| Affiliation: | 1. Division of Pathology, Saitama Cardiovascular and Respiratory Centre, Saitama;2. Division of Control and Treatment of Infectious Diseases, Chiba University Hospital, Chiba;3. Department of Pulmonary Medicine, Saitama Cardiovascular and Respiratory Centre and;4. Department of Pulmonary Medicine, Sekisinkai Sayama Hospital, Saitama;5. Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya;6. Department of Dermatology, Dokkyo Medical University, Tochigi;7. Department of Pulmonary Medicine, National Hospital Organization Himeji Medical Centre, Himeji;8. Department of Pulmonary Medicine, Double Red Cross Hospital;9. Division of Clinical Genetics, Nippon Medical School Hospital and;10. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan |
| Abstract: | Kawabata Y, Watanabe A, Yamaguchi S, Aoshima M, Shiraki A, Hatamochi A, Kawamura T, Uchiyama T, Watanabe A & Fukuda Y (2010) Histopathology 56, 944–950 Pleuropulmonary pathology of vascular Ehlers–Danlos syndrome: spontaneous laceration, haematoma and fibrous nodules Aims: The aim was to clarify the pleuropulmonary pathological findings of vascular Ehlers–Danlos syndrome (vEDS). Methods and results: Nine patients with confirmed vEDS by means of cell culture and/or molecular biological studies who had undergone surgical lung biopsy (SLB), lobectomy or autopsy were studied. Six patients were male and three were female with a mean age of 23.2 years. Histological features were as follows: (i) the main pulmonary lesions related to fragility and spontaneous laceration, these being haematomas in seven, acute haemorrhage in nine, fibrous nodule in eight, with ossification or bone marrow formation in six; vascular disruption in five; intraluminal haemosiderosis in nine; interstitial haemosiderosis in seven, with iron deposition in the alveolar wall and/or vessel wall in five and foreign body reaction in two; emphysematous changes in eight; and bleb formation in two; (ii) secondary iatrogenic pleuropulmonary injuries during SLB or lobectomy comprised pleural laceration in seven of 10 and lung laceration in eight of 10 specimens. Conclusions: Spontaneous laceration of lung tissue is an essential feature and is followed by haematoma and possible fibrous nodule formation. |
| Keywords: | haematoma haemosiderosis laceration lung pseudotumour |