CTICasesEhlers-Danlos Syndrome Type IV
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[...]. Ehlers-Danlos Syndrome Type IV. CTICases. Published October 3, 2014. https://str-case-explorer.pages.dev/c/b4294
Imaging findings
Serial chest CT scans show emphysematous changes in the upper lungs, a large fluid-filled cavitary lesion in the right lower lobe, and bilateral pulmonary nodules that wax and wane over time.
Key takeaways
Vascular Ehlers-Danlos syndrome (Type IV) is an inherited connective tissue disorder caused by defects in type III procollagen. In addition to arterial and bowel rupture, it can manifest in the lungs with spontaneous pneumothoraces, emphysema, and cavitary or non-cavitary nodules caused by focal parenchymal hemorrhage and subsequent cyst formation.
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