Cavitary pulmonary lesions in type IV Ehlers-Danlos syndrome

Pediatr Radiol. 1994;24(4):263-5. doi: 10.1007/BF02015451.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Ehlers-Danlos Syndrome / complications*
  • Hemorrhage / diagnostic imaging
  • Hemorrhage / etiology
  • Humans
  • Lung Diseases / diagnostic imaging
  • Lung Diseases / etiology*
  • Male
  • Pneumothorax / diagnostic imaging
  • Pneumothorax / etiology*
  • Recurrence
  • Rupture, Spontaneous
  • Tomography, X-Ray Computed