Respiratory complications of Ehlers-Danlos syndrome type IV

S. Bruce Dowton*, Stuart Plncott, Lurle Demmer

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

74 Citations (Scopus)


Pulmonary complications are described in a case of Ehlers-Danlos syndrome type IV, established by studies of collagen biosynthesis. At age 20.5 years the patient, who had previously suffered a spontaneous colonic perforation, developed intermittent recurrent hemoptysis and had a spontaneous hemopneumothorax. At presentation, imaging studies revealed multiple scattered cavitary lesions in both lungs. On separate occasions large parenchymal cysts ensued and subsequently regressed. Reviews of other reported patients indicate that pulmonary complications do occur in patients with Ehlers-Danlos syndrome type IV but have not resulted directly in patient mortality.

Original languageEnglish
Pages (from-to)510-514
Number of pages5
JournalClinical Genetics
Issue number6
Publication statusPublished - Dec 1996
Externally publishedYes


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