Cavitary pulmonary lesions in type IV Ehlers-Danlos syndrome.
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.
Herman
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Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis Children's Hospital Department of Radiology, MO 63110.
McAlister
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D000293:Adolescent; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D006470:Hemorrhage / Q000000981:diagnostic imaging / Q000209:etiology; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D008297:Male; D011030:Pneumothorax / Q000000981:diagnostic imaging / Q000209:etiology*; D012008:Recurrence; D012422:Rupture, Spontaneous; D014057:Tomography, X-Ray Computed