Cavitary pulmonary lesions in type IV Ehlers-Danlos syndrome

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Abstract

Type IV Ehlers-Danlos syndrome (EDS) is characterized by large vessel bleeding, visceral rupture and early death. Pulmonary complications are uncommon. We report an 18-year-old patient with EDS type IV who developed recurrent large, thick-walled lung cavitary lesions, probably a manifestation of focal lung rupture. Such hemorrhagic cavities are probably the basis of the bullous disase and recurrent pneumothoraces occasionally observed in type IV EDS.

Original languageEnglish
Pages (from-to)263-265
Number of pages3
JournalPediatric radiology
Volume24
Issue number4
DOIs
StatePublished - Aug 1 1994

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