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Vascular Ehlers-Danlos Syndrome — RACP Adult Medicine MCQ

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HardCardiologyVascular Ehlers-Danlos SyndromeRACP Adult Medicine

A 35-year-old man with hyperextensible skin, easy bruising, and hypermobile joints presents with acute abdominal pain. CT shows a spontaneous splenic artery rupture. He has a strong family history of sudden death from arterial rupture. What is the most likely inherited condition?

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Correct answer: C — Ehlers-Danlos syndrome (vascular type)

Vascular EDS (type IV, COL3A1 mutations) presents with thin translucent skin, easy bruising, and spontaneous arterial, intestinal, or uterine rupture. It is the most dangerous EDS subtype. Spontaneous splenic artery rupture with family history of vascular catastrophes is characteristic. Diagnosis is genetic testing. Surveillance and avoidance of invasive procedures is key.

Reference: Ehlers-Danlos Society – 2017 – EDS Classification; ESC 2024