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Case Reports
. 1994;24(4):263-5.
doi: 10.1007/BF02015451.

Cavitary pulmonary lesions in type IV Ehlers-Danlos syndrome

T E Herman  1 , W H McAlister
Affiliations
Case Reports

Cavitary pulmonary lesions in type IV Ehlers-Danlos syndrome

T E Herman et al. Pediatr Radiol. 1994.

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 disease and recurrent pneumothoraces occasionally observed in type IV EDS.

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