<p>Musculocontractural Ehlers-Danlos syndrome (mcEDS) is a&#xa0;rare autosomal recessive connective tissue disorder characterized by fragility of skin, vasculature and musculoskeletal structures. We report a&#xa0;case of a&#xa0;young male with CHST14(Carbohydrate sulfotransferase 14)-related mcEDS who developed a&#xa0;massive subcutaneous hematoma following minor trauma, necessitating surgical evacuation. This case highlights the potential for life-threatening bleeding complications in mcEDS and underscores the importance of early recognition and multidisciplinary management.</p>

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Hypovolemic shock due to massive subcutaneous hemorrhage in a patient with musculocontractural Ehlers-Danlos syndrome (mcEDS)

  • R. Laggner,
  • S. Payr,
  • L. Adam,
  • D. Baron,
  • L. Zak

摘要

Musculocontractural Ehlers-Danlos syndrome (mcEDS) is a rare autosomal recessive connective tissue disorder characterized by fragility of skin, vasculature and musculoskeletal structures. We report a case of a young male with CHST14(Carbohydrate sulfotransferase 14)-related mcEDS who developed a massive subcutaneous hematoma following minor trauma, necessitating surgical evacuation. This case highlights the potential for life-threatening bleeding complications in mcEDS and underscores the importance of early recognition and multidisciplinary management.